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Table of Contents   
Year : 2010  |  Volume : 3  |  Issue : 2  |  Page : 202-229
Abstracts presented at the Annual Meeting of the Pediatric Cardiac Society of India, 2010

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Date of Web Publication16-Dec-2010

How to cite this article:
. Abstracts presented at the Annual Meeting of the Pediatric Cardiac Society of India, 2010. Ann Pediatr Card 2010;3:202-29

How to cite this URL:
. Abstracts presented at the Annual Meeting of the Pediatric Cardiac Society of India, 2010. Ann Pediatr Card [serial online] 2010 [cited 2019 Apr 18];3:202-29. Available from:

Acute and short-term hemodynamic effects of beta blockers in Eisenmenger syndrome

Ramakrishnan S, Vyas C, Kukreti BB, Kothari SS, Bhargava B, Juneja R, Seth S, Saxena A, Bahl VK

All India Institute of Medical Sciences (AIIMS), New Delhi, India

INTRODUCTION: Sudden cardiac death and progressive heart failure are the commonest causes of death in Eisenmenger syndrome. Beta blockers reduce mortality and improve functional capacity in heart failure due to systolic dysfunction. Betablockers may be useful in Eisenmenger syndrome also, but the safety is not proven.

MATERIALS AND METHODS: Fifteen patients [10 females] of ES with a mean age of 22.6±8.6 years were included. Hemodynamic parameters were measured at baseline, after 15 mg of intravenous metoprolol and 6-weeks after oral metoprolol.

RESULTS : Following intravenous metoprolol there was an acute decrease in venous and arterial saturation due to significant reduction in pulmonary and systemic blood flows. There was an acute increase in pulmonary vascular resistance (PVRI) (23.3±8.3 Wood units to 27.4±10.3 Wood units; P =0.02) and systemic vascular resistance (SVRI) (37.6±8.6 wood units to 46±12 wood units; p =0.008). After 6 weeks of oral metoprolol, PVRI and SVRI returned towards the baseline values (23±10 WU and 40.8±11.5 WU respectively). The 6 minute walk distance (466.46±135.56 meters to 536.53±128.48 meters; p =0.04) improved significantly. None of the patients reported any adverse events.

CONCLUSION : Preliminary observations suggest that metoprolol is safe and may improve exercise capacity in selected patients of ES. Hemodynamic parameters worsen acutely, but recover over the short term.

Lethal arteriopathy syndrome of infants associated with a novel mutation of FBLN4 gene - identification of a new syndrome and understanding the critical role of fibulin 4 in human vascular elastogenesis

Mahesh K, Nampoothiri S 1 , Paepe AD 2 , Kannan R 3 , Malfait F 2 , Laer LV 2 , Renard M 2 , Loeys B 2 , Sunitha V, Kumar S, S Menon 4 , Hiran KR 5 , Faiyaz-Ul-Haque M 6 , Vaidyanathan B, Francis E, Katewa A 7 , Kurup R 8 , Remadevi KS 8 , Krishna Kumar R 9

Departments of Pediatric Cardiology, 1 Pediatric Genetics, 3 Radiology; 5 Pathology - Amrita Institute of Medical Sciences and Research Centre, Kochi; 2 Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium; 4 Pulmonary Vascular Research Institute; 6 Department of Cardiology, King Faizal Hospital, Riyadh, Saudi Arabia; 7 Formerly at Department of Cardiovascular and Thoracic Surgery, Amrita Institute of Medical Sciences and Research Centre, Kochi; 8 Malabar Institute of Medical Sciences, Calicut, 9 Formerly at Department of Pediatric Cardiology, Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala, India

OBJECTIVES : We describe a new syndrome of lethal arteriopathy associated with a novel mutation in fibulin 4 gene (FBLN4) in a unique cohort of sixteen infants from Kerala.

BACKGROUND : Connective tissue disorders producing significant vascular manifestations are exceedingly rare. Molecular mechanisms involved in human vasculogenesis are incompletely understood.

MATERIALS AND METHODS : Sixteen consecutive patients presenting with characteristic arterial dilatation, elongation and tortuosity were prospectively studied with clinical characterization, cardiovascular imaging, and molecular genetic studies.

RESULTS: The patients (9 males, 7 females) presented at median age of 1.5 months, belonging to unrelated families from identical ethno-geographical background; seven had history of consanguinity. Cardiovascular features included elongation, dilatation, tortuosity and narrowing of aorta, pulmonary artery and their branches (median Z-scores at presentation: Aortic Root +4.6, Ascending Aorta +7.6, isthmus -2, Main Pulmonary Artery +4.1). Phenotype included a variable combination of cutis laxa-(53%), long philtrum-thin upper lip-(87%), micrognathia-(47%), hypertelorism-(53%), prominent eyes-(53%), sagging cheeks-(47%), long slender digits-(40%), and visible arterial pulsations-(40%). Genetic studies revealed identical c.608A>C (p.Asp203Ala) mutation in exon 7 of FBLN4 gene in all 16 patients, homozygous in 15, and compound heterozygous in one patient. Homozygosity was lethal (13/15 died, median age 2.5 months), terminal event being cardio-respiratory failure. Isthmic hypoplasia (n=8) correlated with early death (≤4 months). Mutation studies enabled fetal diagnosis in one case.

CONCLUSIONs: This is the first description of a lethal arteriopathy syndrome affecting a unique population, linked to novel mutations in the FBLN4 gene. The study provides new insight into the molecular mechanisms of human vascular elastogenesis.

Pediatric cardiac surgery: Is the girl child neglected?

Ramakrishnan S, Khera R, Jain S, Saxena A, Suparna K, Mani K, Juneja R, Bhargava B, Kothari SS, Bahl VK

All India Institute of Medical Sciences (AIIMS), New Delhi, India

INTRODUCTION: Several socio-economic factors govern the compliance to surgical treatment in patients with congenital heart disease (CHD). These factors have not been studied systematically.

MATERIALS AND METHODS: Parents or guardians of patients (aged up to 12 years) who had been recommended a surgical procedure for CHD were prospectively interviewed using a predesigned proforma. After a year, the surgery status of the patients was noted and the factors associated with non-compliance were analyzed. Additionally, twenty parents of children with maximum overall delays were involved in a qualitative study with a semi-structured interview format.

RESULTS : Of the total 405 patients studied, 271 were male and 134 were female with a mean age of 3.43±3.44 years. After a year, 70% of male patients had undergone surgery, as opposed to only 44% of the female patients (P=<0.00001). The predictors of noncompliance to surgery included socio-economic class by modified Kuppuswamy's scale ( p =<0.00001), educational status of the head ( p =0.006), cost of surgery ( p <0.05) and the number of siblings ( p =0.005). On multivariate analysis, female sex, lower socio-economic status and higher cost of surgery were independently associated with the lower rates of opting for surgery. Qualitatively, parents mentioned issues such as matrimonial prospects and lack of family support exclusively in cases of girl child.

CONCLUSION : Female sex emerges as an important factor governing the delay in opting for surgery and the difference between sexes persist even within individual socio-economic classes.

Dedicated pediatric cardiac intensive care unit, Does it improve outcome?

Balachandran R, Mahesh K, Francis E, Vaidyanathan B, Krishnakumar R, Sunil GS, Shivaprakasha K, Rao SG, Nair SG

Departments of Pediatric Cardiac Intensive Care, Cardiac Anesthesia, Pediatric Cardiology, Pediatric Cardiac Surgery, Amrita Institute of Medical Sciences, Kochi

INTRODUCTIONand Aim : Focussed Pediatric cardiac intensive care has translated to better outcomes in developed nations. We have evaluated the benefits of a dedicated pediatric cardiac intensive care unit in the early post operative outcomes of patients.

MATERIALS AND METHODS : Prospectively collected data of 634 consecutive patients who underwent congenital heart surgery from September 2008 - September 2009 were analysed. A dedicated Pediatric Cardiac ICU was started after 6 months. The patients who were treated in this pediatric cardiac intensive care unit formed the study group {Group B, n=318}. The patients who were treated in a adult cardiac surgery ICU formed the control group {Group A, n=316}. Early post operative outcomes between the two groups of patients were compared.

DATA ANALYSIS AND RESULTS: The two groups were comparable with respect to age, weight, use of CPB and Aortic cross clamp. The ICU stay, duration of ventilation, invasive lines and use of inotropes showed improvement as detailed below. In addition blood stream infections also decreased significantly after the dedicated ICU was started.

CONCLUSION : Establishment of a dedicated pediatric intensive care unit has shown better outcomes in terms of earlier extubation, de-intensification and discharge from ICU. Blood stream infections were also reduced.

Natural history of neonatal ductal stenting for pulmonary circulation in real world

Bhagyavathy A, Ramyashri C, Sreeja P, Satish C, Hariprakash S, Sanjay B, Coelho R, Sivakumar K

MIOT Hospital, Chennai, India

BACKGROUND: Ductal Stenting (DS) is less morbid compared to neonatal Blalock Taussig shunt as it avoids thoracotomy, lung injury and pulmonary overcirculation is better tolerated. However the longevity of palliation is unknown.

MATERIALS AND METHODS: We analysed the followup of all neonates after DS till their next palliation. The cohort was divided into three groups; A: concomitant pulmonary valvotomy, B: Biventricular second stage palliation and C: Univentricular second stage palliation. Interstage mortality was studied.

RESULTS : 19 neonates underwent successful neonatal DS for pulmonary circulation. 6 patients in group A were followed for 1-6 years (median 3.5 years). Echocardiographic right ventricular systolic pressure was below 50 mmHg in all. Tricuspid valve, pulmonary annulus and right ventricle grew with age, but Z scores remained same. Pulse oximeter saturations ranged from S90-98%. Antiplatelets were discontinued after 1 year and 3 stents were patent at last follow up. Conduit repair was done in 8 patients in group B after 3-13 months (median 6 months). Bidirectional Glenn shunt and confluence repair was done in 4 patients after 5-13 months (median 6 months). Even though the Glenn pressures were marginally elevated, hospital stay (7-11 days median 8 days), ventilation (12-36 hours median 28 hours) were not long. Three patients in group C had death at home of febrile illnesses.

CONCLUSIONS : Duration of palliation offered by DS is sufficient to allow adequate somatic growth before second stage surgery. After pulmonary valve perforation and valvotomy, prolonged ductal patency is not needed to maintain pulmonary circulation. There is an interstage mortality of 15% presumably of stent thrombosis though unproven.

Ductal stenting for late presenters with TGA intact ventricular septum

Kothari SS, Ramakrishnan S, Boopathy N, Bisoi AK

All India Institute of Medical Sciences, New Delhi, India

INTRODUCTION: The ideal management strategy for patients presenting late with Transposition Of The Great Arteries (TGA), Intact Ventricular Septum (IVS) and regressed left ventricle is not clear. Late switch, 2-stage switch and Senning are the options. Left ventricular retraining by ductal stenting may retrain the left ventricle with less morbidity, but the experience is very limited.

MATERIALS AND METHODS: Five children aged 3 - 9 months with TGA-IVS with regressed left ventricle underwent recanalization and transcatheter stenting of ductus arteriosus. The ductal stent was removed during one-stage arterial switch surgery.

RESULTS : All the patients had totally occluded ductuses and needed recanalisation with coronary CTO hardware. The ductus was dilated and stented with coronary stents. In all the patients there was significant luminal narrowing despite adequate stent placement and deployment. Two patients needed reintervention for abrupt closure of ductal stent. Ductal stenting resulted in improvement of left ventricular mass, left ventricular free wall thickness and left ventricular volumes within a few days. Four patients underwent successful uneventful arterial switch surgery. During surgery it was observed that the mucosal folds of duct were protruding through the struts of the stent. One patient with prior sepsis died of progressive sepsis after 14 days of stenting, even though the LV was prepared.

CONCLUSIONS : Ductal stenting is a good alternative strategy for left ventricular retraining in transposition of the great arteries with regressed left ventricle.

Focus category: Cardiac catheterization and interventions

#1 ASD device closure in elderly (>50 Years), our experience in last 10 years

Bobhate P, Khatri S, Awasthy N, Tomar M, Radhakrishnan S, Shrivastava S

Department of Pediatrics and Congenital Heart Disease, Escorts Heart Institute and Research Centre, New Delhi, India

INTRODUCTION: ASD device closure in the elderly requires careful evaluation for complicating factors.

MATERIALS AND METHODS: We retrospectively reviewed all patient>50 yrs (n=47) who underwent ASD device closure between March 2001 to May 2009. All patients underwent baseline clinical and echocardiographic evaluation including shunt calculation and coronary angiography. Postprocedural follow up included clinical and echocardiographic evaluation from 1-8 years.

RESULTS : Immediate complications included arrhythmia (n=5), hematoma at puncture site (n=1); LA perforation requiring surgery (n=1). One patient developed pulmonary edema. Forty-six patients were followed up for 1-8 years (mean 4.5 years). Thirty patients (66%) in class II prior to the procedure reported improvement in their exercise capacity. Two patients had NYHA class III symptoms, one of whom had improvement to class II. Five patients had atrial fibrillation prior to the procedure, 3 remained well on medical treatment however 2 had recurrence of atrial fibrillation on follow up. Significant coronary artery disease was found in 1 patient. Septal motion normalized in all while mildly dilated RV persisted in 24 (48%). The systolic pulmonary artery pressure dropped from 41+ 14 to 24+9 mm Hg. Forty-five patients had normal biventricular function on follow up. One patient who had RV dysfunction prior to the procedure continued to have RV dysfunction on follow up. Five patients developed mild LV systolic dysfunction after the procedure that normalized over 3 months.

CONCLUSION : ASD device closure in elderly is a safe and effective method of ASD closure. There was significant improvement in symptoms, reduction in RV end diastolic volume and PA pressures following ASD device closure.

#2 Balloon aortic valvoplasty in paediatric patients: how common is progressive aortic regurgitation?

Awasthy N, Bobhate P, Khatri S, Tomar M, Radhakrishnan S, Shrivastava S

Department of Pediatrics and Congenital Heart Disease, Escorts Heart Institute and Research Centre, New Delhi, India

OBJECTIVE : To evaluate immediate and midterm results after balloon valvoplasty in a paediatric population with congenital aortic stenosis, giving special consideration to aortic regurgitation.

DESIGN: Retrospective study. Setting : Tertiary referral centres for paediatric cardiology. Patients: 108 consecutive patients, with an age range of 0-64 years. Group A infants, (n=24). Group B>1 year old (n=84). Median follow up time was 55 months, range 0-144 months. Intervention: All patients underwent balloon aortic valvoplasty. The balloon to annulus ratio was selected at a mean of 0.90 (range 0.67-1.0). Main outcome measures: Doppler gradients and degree of aortic regurgitation.

RESULTS: The pressure gradient dropped significantly with the intervention and increased mildly at follow up in both the groups although the group A required intervention much earlier. Freedom from significant aortic regurgitation (moderate and severe) was similar in both groups throughout the study period.

CONCLUSION: Aortic balloon valvoplasty is safe and effective but has a high rate of early reintervention in infants with critical aortic stenosis. The considered problem of progressive aortic regurgitation is not to the magnitude widely projected. It does not seem to be prevented by the use of smaller balloons.

#3 Balloon pulmonary valvotomy as interim palliation for symptomatic cyanotic congenital heart disease beyond infancy

Agarwal J, Francis E, Vaidyanathan B, Kumar RK

Division of Pediatric Cardiology, Amrita Institute Of Medical Sciences and Research Center, Kochi, India

BACKGROUND : Corrective surgery is not a realistic option for all patients with Congenital Cyanotic Heart Disease (CCHD) who present beyond infancy. Traditionally, the Blalock-Taussig (BT) shunt is used to palliate these patients.

AIMS AND OBJECTIVES : To describe case selection, techniques, and immediate and short term results of Balloon Pulmonary Valvotomy (BPV) as a palliation in selected patients with CCHD over 2 years age who were not candidates for immediate surgical correction.

METHODS AND RESULTS : Retrospective study. Symptomatic patients of CCHD with a significant valvar component to the pulmonary stenosis and O2 saturation<85% were offered BPV. Thirty eight patients of complex cyanotic congenital heart disease (varying from TOF with hypoplastic branch PAs; TOF with AV canal defect ; DORV VSD,PS ; cTGA, VSD, PS ; Single ventricle, PS) with median age 13.59±8.9 years, weight range 6.7 to 55 kg (27.85 +/- 13.45 kg) underwent palliative BPV from June 2004 to January 2010. Of 38 patients, 14 were males and 24 were females. In 6 patients (single ventricle with malposed great arteries) the pulmonary valve was crossed via a catheter passed from the aorta into the ventricle. The right ventricular outflow tract was crossed with a Terumo wire and dilated with a Tyshak balloon valvuloplasty catheter. Mean O2 saturation increased significantly from 73.42 +/- 6.6% to 89.11 +/- 5.45%. Improvement in saturations occurred in all but 2 patients. There was no procedural mortality. Minor complications included ventricular tachycardia in one patient requiring cardiversion and transient pulse loss in 2 patients. Period of follow up was 15±10 months and saturation on follow up was 85±5%. During follow-up, 10 patients underwent ICR, 2 patients underwent BDGS, one underwent infundibular resection.

CONCLUSION : Balloon pulmonary valvotomy is a safe and effective palliative procedure for complex cyanotic congenital heart disease patients .

#4 Coronary dependent pulmonary circulation in pulmonary atresia ventricular septal defect

Nathani S, Sateesh, Anamika, Shah S, Maheshwari S, Suresh PV

Department of Pediatric Cardiology, Narayana Hrudayalaya, Bangalore, India

In pulmonary atresia and ventricular septal defect, pulmonary circulation depends on systemic aortopulmonary collaterals or patent ductus arteriosus. Along with systemic aortopulmonary collaterals, coronary to pulmonary collaterals are also described. We describe the case of a 15 year boy who presented with cyanosis since early infancy and had recent onset of worsening of symptoms. This boy was investigated and found to pulmonary atresia and ventricular septal defect. Echocardiography was unable to define the pulmonary confluence. He underwent catheterization which showed a major left coronary to pulmonary artery collateral which was sole supply of pulmonary circulation. This variety of pulmonary circulation is rarely found in absence of other major systemic to pulmonary collaterals. Since most of the lung fields do not have dual blood supply, treatment was even more challenging. We reviewed the embryologic explanation of such condition and possible management options.

#5 Closure of large PDA with ASD occluder: Three case series

Agarwal M, Pallavi M, Chattopadhyay A, Bandyopadhyay B

Rabindranath Tagore International Institute of Cardiac Sciences, Kolkata, India

Catheter Intervention closure of PDA has now become routine in most pediatric cardiac care centers and can be offered to most of the infants and children with an isolated ductus. We present three patients with large PDAs and severe PAH aged 12, 14 and 23 years. They were first subjected to reversibility study with oxygen that showed reversible pulmonary hypertension in each case. Then balloon occlusion of the ductus was performed and this too showed no hemodynamic derangement. Subsequently PDA device occlusion was planned. Initially duct occlusion was attempted with the largest (16 mmΧ14 mm size) available device in the lab, but the device slipped through the duct before release in each case. Then closure was attempted with the ASD occluder. After securing the device in place and release of the RA disk in left pulmonary artery, a trans-thoracic echocardiogram was performe to confirm the normal flow pattern in the left pulmonary artery and descending aorta. The device was then released and aortic angiogram was done to confirm the final outcome. Follow up of the patients showed significant decrease in pulmonary artery pressure with normal flow pattern in left pulmonary artery and descending aorta. The utilization of ASD occluder for PDA closure here can be justified as the largest available duct occluder failed to retain a stable position. The suitability of the ASD occluder for the purpose was adequately assessed in terms of adequate size of aortic ampullae and branch pulmonary artery.

#6 Device closure of isolated secundum atrial septal defects in symptomatic children weighing less than 10 Kgs

Srivastava A, Trehan M, Anil S

Apollo Hospital, Hyderabad, India

Atrial Septal Defect (ASD) though usually asymptomatic in young children, can sometimes present with congestive cardiac failure, lower respiratory tract infections and failure to thrive, warranting early correction. There are many reports of successful surgical correction in young children but the experience and data with transcatheter closure is little. This study is a single centre experience evaluating efficacy, safety and outcome of device closure of ASDs in children weighing less than 10 kgs. Between January 2009 and May 2010, ASD closure with septal occluders (Amplatzer, Lifetech, Cardi-O-Fix) was attempted in 19 children weighing less than 10 kgs at our institution. The patients ranged in age from 8 months to 2 years. Their weights ranged from 6 to 10 kg. The size of the defect as measured by transthoracic echocardiography ranged from 10 to 20 mm. The indications for ASD closure were failure to thrive, significant chamber enlargement and hemodynamically significant shunts. The pulmonary/systemic flow ratio was 2.5±0.7 and mean PA pressure was 28±12 mmHg. The size of the device ranged from 10 to 22 mm and sheath size 7 to 10 mm. The device was successfully placed in 18 of 19 children. Either RUPV approach or balloon assisted technique were used in patients with deficient rims. No deaths or major complications occurred. Patients showed improvement on follow up in terms of clinical performance, growth and development. No mid-term complications were noted.

CONCLUSION : Device closure of ASDs is an effective and safe alternative to surgery in young children.

#7: Exclusion of infected pseudoaneurysm of modified BT shunt using a covered stent

Srivastava A, Trehan M, Anil SR

Apollo Hospital, Hyderabad, India

INTRODUCTION: A pseudoaneurysm due to infection after a modified Blalock-Taussig shunt (BTS) is a rare but potentially fatal complication which can rupture, compress mediastinal structures, produce shunt occlusion and bacteremia. We outline the use of a covered stent to exclude such pseudoaneurysm.

MATERIALS AND METHODS: This 6 year old girl presented to us with recurrent fever. She had undergone left modified BTS at age 5years. She was febrile, cyanotic (66% in room air) and had ascites, bilateral pedal edema and mild icterus. An echocardiogram revealed Dextroposition, Double Outlet RV, multiple VSDs, severe infundibular stenosis and a 2 cm mobile vegetation on the pulmonary valve with a patent BTS, dilated proximal subclavian artery, confluent pulmonary arteries, global pericardial effusion and normal ventricular function. She had anemia, leucocytosis and bacteremia. She was treated with intravenous Crystalline Penicillin, Gentamicin and Rifampicin but the fever and bacteremia persisted even after 3 weeks of antibiotics. She was taken for cardiac catheterization to delineate the infected pseudoaneurysm and possibly exclude it from circulation and to assess the suitability for BDG. Angiography showed moth eaten appearance of BTS with pseudoaneurysm at the origin of left subclavian artery. Two covered stents were deployed successively to exclude the pseudoaneurysm. Antibiotics were continued for two more weeks.

RESULTS : Patient showed clinical improvement and then was taken for bilateral BDG, MPA interruption and excision of the pulmonary valve with vegetation. She recovered well and was discharged.

CONCLUSION : This case highlights an alternate use of covered stent in the treatment of infected pseudoaneurysm.

#8 Device occlusion of pseudoaneurysm of ascending aorta: 2 case reports

Agarwal M, Pallavi M, Chattopadhyay A, Bandyopadhyay B

Rabindranath Tagore International Institute Of Cardiac Sciences, Kolkata, India

Pseudoaneurysm of the ascending aorta is an infrequent but well recognized and potentially fatal complication after cardiac surgeries. The complication has a variable time of onset and mode of presentation. We present two cases of ascending aortic aneurysms that developed post cardiac surgery and were managed successfully with an ASD Occluder device.

The first case was a 57 year old man, who had undergone elective coronary artery bypass grafting for triple vessel coronary artery disease. He developed a large trilobed pseudoaneurysm from the ascending aorta with a narrow neck (9.0mm). Poor general condition and associated comorbid conditions made him a surgically inoperable candidate. He was taken to the cath lab and the neck of the aneurysm was closed with a 12mm ASD Occluder device.

The second case was a 35 year old male who had undergone a double valve replacement surgery. He was diagnosed to have a pseudoaneurysm seen arising from the ascending aorta, measuring 45Χ50mm with a narrow neck. After assessing the anatomical suitability, the neck of pseudoaneurysm was occluded with a 10mm ASD occluder device. The immediate post-procedure angiogram showed no residual flow into the sac. A six month follow up of both cases also showed excellent results.

Device occlusion of rhe neck of pseudoaneurysm is an effective and safe option of management. The suitability of the device has to be assessed minutely in order to achieve success.

#9 Interatrial septal stenting to decompress left atrium in left-sided obstructive lesions

Singh M, Padhi S, Bakshi K, Tumkur A, Kanchi V, Kumar S, Rao NK

Department of Pediatric Cardiology, CARE Hospital, Hyderabad, India

We describe two children who underwent stenting of Interatrial Septum (IAS) to decompress the Left Atrium (LA) in left-sided obstructive lesions. Case 1 was a 9-month old infant with Congestive Heart Failure (CHF) and cyanosis, diagnosed as aortic atresia, mildly hypoplastic left ventricle and a small Patent Foramen Ovale (PFO). Under general anaesthesia, after several unsuccessful attempts to cross the PFO, septal puncture was done using Brockenbrough needle. IAS was predilated with a short 3Χ15mm balloon after positioning an 0.014" Galeo extrasupport wire in the LA. A 4.0Χ15mm ProLink stent was deployed under fluoroscopic guidance. The child improved and heart failure subsided. The child developed symptoms again after 3 months. Evaluation showed stent obstruction. A repeat transcatheter procedure was performed to remove the obstructed stent. The patient underwent emergency atrial septectomy, removal of the stent with the entangled snare, and bilateral Pulmonary Artery Banding (PAB). The baby died on day 6 due to ventricular arrhythmias.

Case 2 was a 14-month old girl with CHF and pulmonary hypertension. Echocardiography showed a single ventricle with left atrioventricular valve atresia, D-malposed great arteries and unprotected pulmonary blood flow with restrictive interatrial communication. The atrial septal defect was crossed with a 4F JR catheter using 0.035" angulated Glidewire. A 7.0 x 12mm renal stent (Biotronik, Germany) was deployed under fluoroscopic and transesophageal echocardiography guidance. Saturation improved from 85% to 95%. The child was discharged on day 3. PAB is planned to be performed after 4 weeks.

These two cases illustrate the feasibility and effectiveness of IAS stenting in left-sided obstructive lesions.

#10 Is the cath lab really required for balloon atrial septostomy?

Merchant S, Khan Z, Prabhu A, Patil S, Tailor K, Salvi P, Mhatre A, Kumar N, Joshi S

Pediatric and Congenital Heart Center, Fortis Hospitals, Mumbai, India

INTRODUCTION: Balloon atrial septostomy (BAS) can be performed more rapidly under echocardiographic guidance without transporting high-risk neonates to the cath lab. No such studies have been reported from India aimed at assessing the efficacy and safety of BAS monitored with echocardiography alone.

MATERIALS AND METHODS: From June 2009 to May 2010, 5 infants underwent the procedure under exclusive echocardiographic monitoring. Success was defined as creation of an atrial septal defect with liberal mobility of its margins and a wide colour Doppler flow jet across it with concomitant improved arterial blood gas as well as clinical parameters.

RESULTS : Median age was 20 days (3-90), and the median weight was 3200gram (2700-3700). Four patients had transposition of the great arteries while one had mitral valve atresia. The mean arterial oxygen saturation increased from 30.9±17.4% to 61.8±9.7% ( P <0.001) whereas the mean pO2 improved significantly from 14.88+2.39 mmHg to 31.75+9.5 mmHg ( P <0.001). Mean pH improved from 7.12+0.15 to 7.41+0.06 ( p <0.05). Mean urine output improved from 2.1+1.1ml/kg/hr to 4.2+1.8ml/kg/hr ( p <0.01). There were no major complications except transient bradycardia in one neonate. One baby presenting with hypoxic seizures, was on mechanical ventilation, inotropic support and phenobarbitone pre-procedure improved and inotropic support was stopped. Feeding was reinstituted at a mean of 4 hours after the procedure in all. Two babies were on prostaglandin infusion pre-procedure. A baby with mitral atresia underwent BAS under trans-esophageal echocardiography along with a concomitant pulmonary artery banding. All the other babies underwent arterial switch operation at a later date. Patients were discharged from the ICU after a mean stay of 22 days.

CONCLUSION : Echocardiographic monitoring of BAS avoids the transportation of severely sick neonates to the cath lab, helps determining position of the balloon catheter, immediate assessment of the result of every traction of the balloon without using radiation thus making the procedure effective and safe.

#11 Largest ASD device closure - case report

Agarwal M, Sugaonkar P, Chattopadhyay A, Bandyopadhyay B

Rabindranath Tagore International Institute Of Cardiac Sciences, Kolkata, India

ASD device usage has increased up significantly because of cosmetic benefits, safety and short learning curve.

A 35 yr old male, weighing 65 kg presented with c/o shortness of breath on exertion and palpitations. Upon investigation, he had a 44 mm OS ASD with R-L shunt with adequate rims of septal tissue. Rght femoral artery (6F) and venous(8F) access was obtained. A 10F delivery system on an exchange length Amplatzer wire was pre-positioned into the RUPV. The sheath was completely de-aired. A 46mm Searcare ASD occluder device was positioned. The device was deployed under fluroscopic and TEE guidance. Post procedure ECHO showed the device to be perfectly in place, no LV inflow and outflow obstruction, no obstruction to PV/SVC/IVC. In this procedure no other special techniques (balloon assisted or modified sheath) were used. Post procedure ECG showed normal sinus rhythm. 6 Months f/u showed the device in position without any obstruction to adjacent structures, absent pericardial effusion and a normal sinus rhythm.

In carefully selected cases, a very large ASD device can be used safely.

#12 Palliative stenting of patent ductus arteriosus in older children and adults with congenital cyanotic heart disease

Francis E, Kumar S, Kumar RK

Amrita Institute of Medical Sciences and Research Center, Kochi, India

OBJECTIVE : To describe the indications, technique and results of palliative patent ductus arteriosus (PDA) stenting in selected patients (>2 years) with congenital cyanotic heart disease with reduced pulmonary blood flow who were not candidates for definitive surgery in the immediate future.

MATERIALS AND METHODS: Hospital records of patients (>2yrs) undergoing PDA stenting between January 2007 - September 2009 were reviewed. The access (femoral vein or artery, axillary or radial artery) and approach was dictated by the anatomy of the PDA. A coronary guiding catheter (5-7 F) or a long sheath (4-7 F) was used to access the PDA. Coronary or peripheral vascular balloon expandable stents were used for stenting.

RESULTS : Fifteen patients with median age 14 years (range 2-18 years); median weight 17.5 kg (range 7-57 Kg) were included. Indication for intervention was hypoxia (mean saturation 69 8.9%, hemoglobin 19.8±2.69 gm/dl) with no immediate prospect of definitive surgical correction because of immediate morbidity, unsuitable anatomy or economic considerations. Stents of 3.5 - 8 mm diameter and 12 - 38 mm length were used. Successful stenting was accomplished in all (Flouro time- 24.6±16.7 minutes) with no complications and the saturations improved to 88±2.3%. The oxygen saturations on follow-up, median period of 13 months (range 1 - 21 months) were 82±2.8%. One patient underwent corrective repair.

CONCLUSION : PDA stenting can provide effective palliation in selected older patients with cyanotic congenital heart disease.

#13 Percutaneous balloon valvoplasty of congenital aortic stenosis: Independent predictors of outcome

Awasthy N, Bobhate P, Khatri S, Tomar M, S Radhakrishnan, Shrivastava S

Department of Pediatrics and Congenital Heart Disease, Escorts Heart Institute and Research Centre, New Delhi, India

OBJECTIVE : To evaluate independent predictors of outcome of aortic valvoplasty.

MATERIALS AND METHODS: Retrospective follow up study in a tertiary care centre. Independent predictors of outcome identified by multiple logistic regressions. The study group consisted of 108 consecutive patients treated at the median age of 13 years (0-64 years) of whom 24 (18%) were under 1year age. The follow up period was up to 12 years (median 5.3). Interventions: Percutaneous balloon valvoplasty with mean (SD) balloon to annulus ratio 0.97 (0.08). Main outcome measures: Restenosis was defined as a mean gradient>40 mmHg on echocardiography, grade 3 aortic regurgitation, surgery, death, and valvoplasty failure (significant restenosis or regurgitation or surgery or death).

RESULTS : There were 84% males with weight ranging from 2.3 to 76 kg (mean=21 kg). The restenosis rate was 26%; significant regurgitation developed in 8%. Surgery was needed in 1patient. ''Valvoplasty failure'' occurred in none of the patients. LV dysfunction was present at the time of presentation in 42% of the patients (n=45); this persisted during follow up in 6 (4%) of the patients. There was no procedural mortality but 2 neonates died on day 2 of procedure probably due to arrhythmia. Mean (SEM) survival probability 12 years after the procedure was 0.89 (0.02). The independent predictors were as follows: for restenosis: small aortic annulus; for insufficiency: bicuspid aortic valve and a low left ventricular ejection fraction. Persistent LV dysfunction was more likely at a younger age at presentation, pulmonary artery hypertension at initial presentation and AR.

CONCLUSION : Independent predictors of unfavorable outcome are a small aortic annulus, bicuspid aortic valve, poor function of left ventricle.

#14 Percutaneous closure of nonseptal cardiac defects/malformations with amplatzer devices

Tomar M, Awasthy N, Khatri S, S Radhakrishnan, Shrivastava S

Department of Pediatrics and Congenital Heart Disease, Escorts Heart Institute and Research Centre, New Delhi, India

INTRODUCTION: The Amplatzer devices are the most commonly used throughout the world for septal defects. In this report, we are reporting the efficacy of closure of nonseptal defects with the Amplatzer group of devices although they have not been prototyped for use in such conditions.

MATERIAL AND METHODS: Retrospective study in a tertiary referral center. Twenty-three patients, in the age group 2-67 yrs, were treated percutaneously with Amplatzer devices for the following conditions: coronary arteriovenous (AV) fistula (Amplatzer duct occluder, n=6), pulmonary AV fistula (Amplatzer duct occluder, n=2), closure of ascending aorta perforation (Amplatzer septal occluder, n=1), ruptured sinus of Valsalva (Amplatzer duct occluder, n=5), Fontan fenestration closure (Amplatzer PFO occluder and Amplatzer septal occluder n=4), closure of large decompressing venous channel from Glenn (Amplatzer vascular plug, n=4) and closure of mitral paravalvular leak (Amplatzer septal occluder, n=1) and aortic paravalvular leak (Amplatzer duct occluder, n=1).

RESULTS: Successful closure was achieved in all coronary AV fistulae (immediately n=4,at 3 months all), ruptured sinus of Valsalva (immediate in all), fenestrated Fontan (immediately in all), decompressing vein post Glenn (immediate) and ascending aorta perforation (immediate). The aortic paravalvular leak closed at 3 months follow-up. Continued residual flow persisted in mitral paravalvular leak at 6 months follow up. Complication: Intravascular hemolysis and residual mitral regurgitation persisted in the patient with mitral paravalvular leak requiring surgical exploration and closure. On follow up ranging from 2 months-6 years, complete closure of the defects persisted with no adverse effect.

CONCLUSION: Although devices have not been recommended for closure of non septal defects, it is possible, in selected cases, to treat these conditions non surgically with the use of non prototype Amplatzer device without significant complications at short to intermediate term follow up. Long term follow up will however be necessary before we can conclude that it can be a safe alternative to surgery.

#15 Reliability of transthoracic echocardiography in guiding ASD device closure

Srivastava K, Shetty K, Satheesh, Maheshwari S, Suresh PV

Department of pediatric cardiology, Narayana Hrudayalaya Institute of Cardiac Sciences, Bangalore, India

INTRODUCTION : In recent times, transcatheter closure of secundum ASD if suitable is the preferred choice rather than surgery which has disadvantages like increased morbidity, patient discomfort and thoracotomy scar. The aim of the present study was to assess the reliability of transthoracic Echocardiography in guiding ASD device closure.

MATERIAL AND METHODS: Patients admitted in Narayana Hrudayalaya from March 2005-March 2009 for ASD device closure formed the study group. TTE guidance for device closure was used in patients who had good echo windows and TEE guidance was used in those cases with poor echo windows and in cases of suspicion of additional lesions.

RESULTS : Appropriate statistical methods were used for data analysis. 400 patients were admitted for ASD Device Closure in the study period. It was a retrospective analysis studying ASDs planned for device closure during the study period. TTE was performed in 379 (94.75%) patients and TEE in 21(5.25%) patients. Out of 400 patients, 243 were females and 157 were males. Of the 379 patients who underwent TTE, 372(98.2%). Patients were subjected to an attempt of ASD device closure. The remaining 7(1.8%) patients were found not suitable for device closure and were directly referred for surgery. Among the 372 patients, 324 patients had successful device deployment. Among 21 patients monitored with TEE, Successful deployment could be done in 19(90.5%). Comparison of patients who underwent TTE guidance with TEE guidance in the present study showed statistically similar results in mean age, weight, defect, size, device size, procedure time, fluoroscopic time, procedure attempt success and immediate post procedure success.

CONCLUSION : The present study showed that TTE along with fluoroscopy was reliable in guiding ASD device closure. The inconvenience associated with TEE can be avoided using TTE alone in patients who have good transthoracic window.

#16 Renal artery aneurysm management by coil embolisation

Patil P, Changela V, Sateesh S, Mahala BK

Narayana Hrudayalaya, Bangalore, India

We report a case of renal artery aneurysm successfully by transcatheter embolisation with microcoils. A thirteen old was referred to us for emergency treatment after presenting with persistent hematuria. Patient presented with persistent hematuria one month after undergoing percutaneous nephrolithotomy for right sided nephrocalcinosis. Computed tomography showed a right renal artery aneurysm along with a perirenal hematoma. The aneurysm was thought to be due to traumatic injury to renal artery during surgery. Emergency angiography demonstrated a saccular aneurysm in the lower aspect of renal hilum. A total of three micro-coils (1mm) were placed in the feeding branch of the aneurysm. The first coil was partially placed in the feeding branch over which other coils were mounted. Postprocedure angiography showed a favourable result. The patient had an uneventful course in hospital.

CONCLUSION : Endovascular treatments, including coil embolisation, appear to effective, safe and less invasive than surgery. The methods should be considered as a treatment of choice for renal artery aneurysm.

#17 Refractory renovascular hypertension and acute hemiplegia: The missing clue!!

Chaudhuri M, M Jayranganath, Rao S, V Subash Chandra

Saint Philomena's Hospital, Bangalore, India

INTRODUCTION : Pediatric CVA with renovascular hypertension has multiple etiologies. We present a child with Moyamoya disease and simultaneous renal artery stenosis. This combination is unreported in literature.

MATERIAL AND METHODS: A 5 year old apparently asymptomatic girl presented with acute left sided hemiplegia and severe hypertension (BP=170/100mmHg) for two weeks prior. Physical examination revealed Grade IV/V motor power in left leg, no radiofemoral delay and a distinct abdominal bruit. Baseline investigations were normal. CT Brain showed acute left parieto-temporal infarct. Selective renal angiography revealed bilateral, multifocal, peripheral renal artery stenosis. An aortogram and common carotid arteriograms were normal. However, in view of cerebral ischemia, selective right internal carotid arteriogram was performed. There was complete occlusion of terminal right internal carotid artery with hypoperfusion of anterior and middle cerebral territories with pathognomonic appearance of puff of smoke or 'Moyamoya disease'. She successfully underwent cerebral revascularization by Encephalo-duro-arteriosynangiosis operation and has fully recovered motor power at one year follow-up. Bilateral multifocal renal involvement was not amenable to either angioplasty or stenting and perioperative BP control was achieved pharmacologically.

CONCLUSIONs : Moyamoya syndrome with extra-cranial involvement like renal arterial lesion is extremely rare and never before reported in pediatric age group. This rare differential should be remembered by the cardiologist while evaluating idiopathic pediatric hypertension. The diagnosis is missed unless selective internal carotid arteriogram is performed.

#18 Senning's baffle obstruction-balloon dilatation… is it effective?

Mahindrakar P, Ganguly D, Sugaonkar P, Bandyopadhyay B

Rabindranath Tagore International Institute Of Cardiac Sciences, Kolkata, India

INTRODUCTION: Baffle obstruction after Senning procedure is a serious complication. Percutaneous balloon dilation was attempted in three patients with pulmonary venous baffle obstruction.

MATERIAL AND METHODS : From April 2005 to May 2010 three patients underwent baffle dilatation.

RESULTS : Ages of these patients were 2.5, 6 and 3.5 years respectively. They had symptoms of baffle obstruction after 18, 36 and 25 months respectively. Baffle dilatation was done with a Tyshak balloon with gradual increments. Two patients (2.5, 6 years) had immediate relief of obstruction and the gradient came down from 20 to 7 mmHg and 32 to 4 mmHg respectively. Both of them did well for 5-6 months but had recurrence of baffle stenosis. The third patient's (3.5 years old boy) peak gradient came down from 42 to 38 mmHg post procedure. He then underwent PA band followed by arterial switch but expired post operatively.

CONCLUSION : Though short term results after balloon dilatation are promising, long term prognosis is guarded. Baffle obstruction needs definitive management like reoperation, arterial switch or cardiac transplantation.

#19 Safety and feasibility of neonatal transfemoral ductal stenting for pulmonary circulation

Ramyashri C, Bhagyavathy A, Sreeja P, Satish C, Hariprakash S, Sanjay B, Robert Coelho, Sivakumar K

MIOT Hospital, Chennai, India

BACKGROUND: Ductal stenting (DS) is a nonsurgical alternative in neonates with duct dependent pulmonary circulation. Femoral, axillary and carotid arterial access has been proposed for varying ductal anatomy making the procedure non uniform and complex. We present our results with a uniform transfemoral strategy.

MATERIAL AND METHODS: After discontinuing Prostaglandin E1 0-60 minutes before access, a lateral aortogram was obtained with a 4 french cut pigtail cathter to delineate the aortic origin of the duct. One or two 0.014" guidewires were used and tip parked in the distal pulmonary bed (the second as buddy wire). A 5 French right Judkins guide catheter was exchanged and a 3.5 or 4 mm coronary stent deployed (former if patient weighed under 2.5 kg). Stent length was chosen based on echocardiography parameters. Heparinisation for 48 hours, dopamine to maintain systemic pressures and dual antiplatelet agents were routine .

RESULTS: 22 neonates aged 3-28 days (median 12 days), weighing 2.2-4 kg (median 2.8 kg) had DS. Intracardiac anatomy was univentricular in 8 and biventricular in 14 patients. Pulmonary valve perforation with valvotomy was done in six patients. Ducts were vertical in 19, contralateral from innominate artery in 2 and bilateral to non-confluent pulmonary arteries in one. 7 of 22 patients required a second stent to cover the entire length of the duct. There were two procedural failures a stable guidewire position was not achieved in one and there was acute stent thrombosis in another. One patient with bilateral DS with 3.5 mm stents developed low cardiac output state and pulmonary over-circulation and died after 36 hours. 19 neonates were discharged from hospital without femoral arterial access complications.

CONCLUSIONs: Transfemoral stenting of vertical and tortuous neonatal ducts was a success in 90% of patients.

#20 Transcatheter atrial septal defect closure in infants and young children weighing ≤ 10 Kg

Kumar S, Jain S, Kumar RK, Francis E

Amrita Institute of Medical Sciences, Cochin, India

BACKGROUND : Although a small percentage of patients with ASD are known to present in early childhood with symptoms, there is limited information on feasibility and safety of transcatheter closure in them.

OBJECTIVE: To describe our institutional experience on transcatheter closure of atrial septal defect in infants and children weighing ≤10 kg.

METHODS AND RESULTS : Records of 32 patients weighing ≤10 kg (age: 27±10 months, range 4-42 months; weight: 8.7±1.6 kg, range 4.4-10 Kg) who underwent catheter closure of ASD in our Institute (2007- 2010) were reviewed. This represented 4.8% of our total experience in this period. Indications for closure included failure to thrive (83%), recurrent respiratory infections (54%), and heart failure(8%). Case selection was through trans-thoracic echocardiography. The ASD size was 14.5±3.2 cm and PA systolic pressure was 35±8 mm Hg. Closure was achieved in all (fluoroscopy time: 10±7.3 minutes; median device size 16 mm, range 9 - 20 mm). General Anesthesia and transesophageal echo guidance was utilized in 13. Complete closure was achieved in all but one (small additional defect). Transient ECG abnormalities included first degree AV block (2) and junctional rhythm (2). All had normal sinus rhythm at 24 hrs. On follow up (12±8 months) all patients were symptom free with improved weight Z scores (from -2.5±0.6 to -1.7±0.7; p <0.001), normal ECGs and satisfactory device positions on echocardiograms.

CONCLUSION : Transcatheter device closure of ASD is feasible and safe in selected infants and small children.

#21 Trans-catheter closure of ruptured sinus of Valsalva aneurysm: A single centre experience

Mohanty SH, Mahesh K, Francis E, Kumar RK, Vaidyanathan B

Department of Paediatric Cardiology, Amrita Institute of Medical Sciences and research centre, Kochi, India

INTRODUCTION: Ruptured Sinus of Valsalva aneurysm (RSOVA) is an uncommon clinical condition with a varying clinical spectrum ranging from cardiogenic shock to asymptomatic status. Recently, trans-catheter closure has been reported as an alternative to surgery in RSOVA. We report our institutional experience in the trans-catheter closure of RSOVA.

METHODS AND RESULTS: Trans-catheter closure was attempted in a total of 6 patients (5 female) during the study period 2006-10. The mean age was 31.5±14.6 years. The RSOVA was localised to right atrium in 4 patients and RV outflow in two. Half of the patients were in congestive heart failure; one was in cardiogenic shock and another was asymptomatic. The defect from crossed from the aortic side and the wire from snared out from the venous side forming an arterio-venous loop. All defects were occluded using a PDA device introduced from the venous side (12-10 device in 4 patients; 10-8 in the other two). Successful occlusion of the defect was achieved in all patients. Mean flouroscopic time was 17.3±10.2 minutes. There was one death; this was a 6-year old child who presented in a state of cardiac arrest due to an acute onset RSOVA to right atrium in whom rescue device closure was attempted. One patient had an asymptomatic dissection of the right coronary artery which required stenting. On follow-up (median 11 months), all survivors remained in NYHA class I with no residual shunt on echo.

CONCLUSION : Trans-catheter closure is a safe and effective alternative to surgery for most patients with RSOVA.

#22 Unilateral and bilateral transcatheter pulmonary artery stenting - MIOT hospital experience

Ramyashri C, Bhagyavathy A, Sreeja P, Satish C, Hariprakash S, Sanjay B, Robert Coelho, Sivakumar K

MIOT Hospital, Chennai, India

BACKGROUND : Pulmonary artery (PA) stenosis are difficult surgical substrates due to high incidence of restenosis. Since balloon angioplasty leads to recoil, PA stenting provides a better alternative.

MATERIAL AND METHODS : We reviewed our experience of PA stenting with Cordis Genesis XD, EV3, Cheatham platinum and Atrium V12 stents that can be dilated later to a larger diameter.

RESULTS : Thirteen patients aged 2-12 years, weighing 10-34 kg received 17 stents. Bilateral stents were placed in 4 patients aged 2-7 years. Two of the 4 were in conduits after truncus and Rastelli repair; 2 others had bilateral PA hypoplasia (William syndrome and post Fallot repair). The right ventricular systolic pressure in this group dropped from 110-140 mmHg to 35-60 mmHg post procedure. Stents were deployed simultaneously in 3 and sequentially in one patient.

Two patients with univentricular hearts and either a post stage II Norwood (aged 3 years) or post Glenn shunt (aged 4 years) had left PA narrowing and were stented from 3-4 mm (stenosis diameter) to 10-12 mm.

Unilateral PA stents were placed in 7 patients. The indications were native left PA stenosis (n=1), truncus conduit narrowing (n=1) and post Fallot repair with PA plasty (n=5). The stenosis diameter of 3-6 mm (associated with nuclear scan perfusion discrepancy) was stented to 8-14 mm. There was no major procedural complications, stent fractures or migration. On a follow up of 6-36 months, none of them needed a stent redilatation.

CONCLUSIONs : PA stenting provides safe, effective and lasting palliation in native and post operative PA stenosis. Stents that can be post dilated make transfemoral PA stenting feasible even in smaller patients.

Focus category: Non invasive pediatric cardiology including echocardiography, CT scan and MRI

#23: An unusual case of left main coronary artery aneurysm with right ventricle fistula

Nathani S, Kiran, Shah S, Maheshwari S, Reddy C, Sures

Narayana Hrudayalaya, Bangalore, India

INTRODUCTION: Left main coronary artery (LMCA) aneurysm is a rare finding (0.1%). It is more often seen in adults due to post atherosclerotic changes. A 4 year old boy presented with complaints of repeated respiratory tract infections since 3 months of age and failure to thrive. Echocardiography showed dilation of the left main coronary artery with flow into the right ventricular outflow tract (RVOT). An initial diagnosis of a left coronary cameral fistula was made. However the LMCA was significantly dilated, out of proportion to the flow into the RVOT. A cardiac catheterization was performed to delineate the coronary artery anatomy and to measures the pulmonary artery pressures. It showed a giant (14mm) left coronary artery aneurysm with a fistulous connection to the right ventricle outflow tract. The giant aneurysmal coronary was obstructing the opening of the left main coronary artery and the LMCA was retrogradely filling up from collaterals arising from the right coronary artery. A surgical repair of LMCA aneurysm by 2 patch technique and plication of the aneurysmal wall was performed. Recovery was uneventful. This is the largest congenital left main coronary artery aneurysm in pediatric age group to be reported in literature.

#24 Atrial septal defect closure-why is infective endocarditis not recommended?

Patil P, Mehta A, Shah S, P.V. Suresh, Alva P, Shekhar Rao

Department of pediatric cardiology, Narayana Hrudhayalaya, Banglore, India

Infective endocarditis is rare in post operative patients with ASDs. There are very few case reports describing infective endocarditis after surgical or device closure of atrial septal defect. We report of a case of seven month old female child with infective endocarditis of atrial septum following surgical closure. The patient presented to us with a history of breathing difficulty. Echocardiography showed unobstructed cardiac total anamolous pulmonary venous drainage to coronary sinus with small restrictive atrial septal defect. Patient underwent rerouting of pulmonary veins to left atrium with pericardial patch closure of atrial septal defect. Postoperative course in intensive care unit was uneventful. Patient presented with fever on the ninth postoperative day. Investigation revealed raised leukocyte count with neutrophilic predominance. Markers of inflammation (CRP, ESR, Procalcitonin) were positive. Blood culture did not grow any organism. Post operative echocardiogram showed thickened sphear shaped atrial septum 12x14mm in size with a vegetation on tricuspid valve. The patient was treated with intravenous antibiotics. Patient's general condition improved and a repeat echocardiogram showed a decrease in thickness of the atrial septum with disappearance of the tricuspid valve vegetation. Infective endocarditis prophylaxis is not recommended for postoperative atrial septal defect closure. But in certain scenarios, it is beneficial to recommend infective endocarditis prophylaxis for postoperative patients.

#25 Fetal echocardiography - spectrum of congenital heart disease and outcome

Venkatesh S, Prabhu S, Kulkarni S, Singh P, Sharma P

Divison of Pediatric Cardiology, B. J. Wadia Hospital For Children, Mumbai, India

OBJECTIVE : To document the spectrum of CHD and its impact on antenatal diagnosis of congenital heart disease by fetal echocardiography.

MATERIAL AND METHODS : The records of 1044 mothers referred for fetal echocardiography were analyzed. Fetal echo scan was done after 16 weeks of gestation. In complex CHD, a guarded prognosis was explained to the parents. Repeat echocardiograms were done in all neonates to correlate the antenatal diagnosis. Cardiac autopsy was carried out in case of terminated pregnancies. Intrauterine transfer to higher centers was advised in cases of complex CHD.

RESULTS : Expectant mothers age ranged from 18-47 yrs (mean 24yrs). The gestational age was between 16 to 36 weeks (mean 22.3 weeks). The indications for referral were abnormal level 1 scan (28.5%), previous child with cardiac anomalies in 230 cases (22%), associated congenital anomalies in 68 (6.5%), gestational diabetes (20.49%), bad obstetric history (12.9%). One hundred and seventy of 1044scans (16.3%) were abnormal. Acyanotic heart diseases (ASD, VSD) were noted in 5.8%. Functional abnormality (significant valvular regurgitation) was noted in 9 and Complex CHD was seen in 5.1%. Bradyarrhythmia and tachyarrhythmia was seen in 3 and 5 cases respectively. Two pregnancies were advised termination before 20 weeks of gestation and autopsy finding correlated with the antenatal diagnosis of Hypoplastic left heart and hypoplastic right heart syndrome respectively. Five mothers received pharmacotherapy for fetal tachyarrhythmia with successful pharmacological cardioversion in three. Sixty-two mothers were advised institutional delivery. Twelve neonates needed PGE 1 infusion at birth. Eight underwent a neonatal procedure (septostomy/valvuloplasty). Pacemaker implantation was done in 2 children with Complete Heart Block born to mothers with SLE. Ninety-three percent of antenatal echoes correlated well with neonatal scan.

CONCLUSION : Fetal echocardiography positively impacts early diagnosis and management of children with congenital heart disease.

# 26 Bronchogenic cyst

Alva P, Reddy C, Suresh PV, Patil P, Mehta A, Shah S

Narayana Hrudayalaya, Bangalore, India

An eight month old female infant was referred to the intensive care unit with a diagnosis of TAPVC. There was a history of rapid breathing and repeated respiratory infections for the 4 months prior to presentation. Examination revealed tachycardia, tachypnea and normal pulse volume. Blood pressure was normal for age. SpO2 in room air was 82%. With oxygen it rose to 96%. Respiratory system examination revealed retractions and rhonchi bilaterally. Cardiovascular examination revealed a soft ejection systolic murmur in the left upper sternal border. Echocardiography showed a structurally normal intra-cardiac and vascular anatomy with a PFO with left to right shunt. However the left atrial free wall appeared flattened. The left atrium was compressed by an external hyperechogenic, well circumscribed mass. A collapsed left lung adjacent to the left ventricle was also well delineated. A CT scan showed a solitary posterior mediastinal mass measuring 4.2cm x 3cm x 2.8cm which was compressing the left bronchus and the left atrium. Left lung collapse was also confirmed.

RESULT : The mass was surgically excised following which the patient improved and was discharged home. Histopathological examination of the mass showed the mass to be a Bronchogenic cyst.

CONCLUSION: This case underlines the utility of ECHO in the diagnosis of extracardiac malformations which many a time present to the pediatric cardiologist. This case also emphasizes the importance to clues like improvement of saturation with O2 which points to the non-cardiac origin of the respiratory distress.

#27 Comparative roles of intraoperative epicardial and early postoperative transthoracic echocardiography in the assessment of surgical repair of congenital heart defects

Awasthy N, Tomar M, S.Radhakrishnan, Shrivastava S, Kaushal S, Iyer KS

Department of Pediatrics and Congenital Heart Disease. Escorts Heart Institute and Research Centre, New Delhi, India

AIM : To study the efficacy of intraoperative epicardial imaging.

MATERIAL AND METHODS : In 104 consecutive patients undergoing surgical repair of congenital heart defects intraoperative (after cardiopulmonary bypass) epicardial two-dimensional and Doppler color flow imaging was performed by a single observer. The results were compared with those of sequential transthoracic echocardiography performed within 24 hrs of surgery and repeated prior to hospital discharge.

RESULTS : Epicardial Spectral Doppler imaging underestimated or did not identify a residual outflow tract gradient in 6 patients. Left atrioventricular (AV) valve regurgitation after repair of complete AV septal defect was underestimated in three patients. A minor degree of shunting around the patch was a common finding on epicardial and early postoperative imaging and persisted at the time of hospital discharge in 17 of 46 patients who had undergone patch closure of a ventricular septal defect as part of the surgical procedure. Additional trabecular septal defects were missed on color flow imaging after cardiopulmonary bypass in three patients, but none of them required subsequent reoperation. Epicardial echo was able to delineate the cause as dynamic RVOT obstruction in 8/20 cases of Tetralogy operated without transannular patch (out of 31 cases of TOF). These resolved to acceptable limits in postoperative period.

CONCLUSION : Although intraoperative two-dimensional and color flow imaging permitted the recognition of the majority of residual defects requiring immediate revision, AV valve regurgitation was usually underestimated and dynamic RVOT gradients were very well profiled.

#28 Isolation of the left subclavian artery in a patient with absent pulmonary valve syndrome

Jadhav M, Garekar S, Kulkarni S, Radhakrishnan B, Mohanty SR, Rao SG

Children's Heart Centre, Kokilaben Dhirubhai Ambani Hospital, Mumbai, India

A 2 week old infant presented to us as a case of multiple VSDs with a murmur. He had mild respiratory distress, hyperdyanamic precordium, normal heart sounds, peripheral pulses and to and fro murmur at the left upper sterna border. The saturation and blood pressure in both upper and lower extremities were within normal limits. The X ray showed borderline cardiomegaly with normal lung fields. ECG had RVH. Echocardiography showed an absent pulmonary valve with moderate restriction and regurgitation; severe hypertrophy of the right ventricle and multiple tiny VSDs in the apical muscular septum. The aortic arch was right sided and large PDA was present. The neonate was taken up for PDA ligation. The LSCA was noted to be arising from the PDA. The LSCA was sacrificed and PDA was ligated. A post operative CT scan confirmed the origin of the LSCA from the PDA and also showed collaterals filling up the distal LSCA.

An isolated LSA is a rare congenital anomaly. Embryologically it is explained by regression at two levels in Edward's hypothetical arch plan. The most frequently encountered CHD is Tetralogy of Fallot. CT scan or MRI are best for diagnosis. Surgical repair of the accompanying heart disease and PDA ligation is required.

#29 Non surgical management of a congenital aortocaval fistula from right subclavian artery to the superior vena cava with SVC obstruction

Awasthy N, Tomar M, S Radhakrishnan

Department of Pediatric Cardiology, Escorts Heart Institute and Research Center, New Delhi, India

Congenital Aortocaval fistula is a rare cause of left-to-right shunt. While 4 cases of the communication from Subclavian artery to pulmonary circulation were found, no such case to SVC has been reported in the literature. We present a case of a congenital aortocaval fistula from right subclavian artery to the superior vena cava in a 4 month old boy. The infant was suspected to have heart disease in view of tachypnea and feeding difficulty. A continuous murmur was audible over the right infraclavicular region. Echocardiogram showed continuous color and Doppler flow from right subclavian artery to SVC with SVC stenosis (SVC mean PG of 24 mm Hg) and a tiny patent ductus arteriosus (shunting left to right). CT angiography further delineated the anatomy of the atriovenous fistula from the right SCA to mid SVC. The patient was taken for cardiac catherization. Pressures tracing showed pull back gradient of 17 mm Hg across the SVC. Right and left ventricular filling pressures and pulmonary artery pressures were normal. An Amplatzer Vascular plug 6 mm was deployed from the right subclavian artery occluding the abnormal fistulous connection. The post device angiogram revealed completely occluded lumen of the fistulous tract with sluggish flow across the tract. A Genesis (Cordis) 9x 29 mm stent was deployed across the stenosed end of SVC. The post stent angiogram revealed a laminar flow pattern in SVC. The predischarge echocardiogram showed no residual flow in the fistulous communication The patient had an uneventful course and was healthy at the 6 month follow up visit.

#30 Lipoma

Nathani S, Mehta A, Shah S, Maheshwari S, Reddy C, PV Suresh

Narayana Hrudayalaya, Bangalore, India

Mediastinal tumors may have varied clinical presentations. We present a 12 year old boy who presented with class IV dyspnea on exertion. He had signs and symptoms of congestive cardiac failure. The chest X-ray showed massive cardiomegaly and a large radio opaque shadow near the right cardiac silhouette. Echocardiography showed severe biventricular dysfunction, grade III mitral regurgitation, massive pericardial effusion and a large echogenic mass compressing the atria. In view of biventricular dysfunction child was started with decongestants and inotropic support and he improved symptomatically. For complete evaluation of mass, the patient underwent cardiac CT scan which confirmed large mass as lipoma of 12x10 cm in size. Due to compression on the heart and alteration of mitral valve geometry child had severe mitral regurgitation and congestive heart failure. Intra-operatively it was found that mass was well encapsulated but had multiple pedicles encroaching on surrounding mediastinal structures (vena cava and great vessels). Due to huge size of mass, inaccessible location and encroachment, it was difficult to remove it in total. Hence the patient underwent cardiopulmonary bypass surgery and mass was removed in piece by piece. Histopathology confirmed the mass to be a myolipoma. Post operatively there was an improved ventricular function and decrease in mitral regurgitation.

This is first report in literature of a huge lipoma presenting as congestive cardiac failure in achild. The method of removal of lipoma was unique. It also proves the usefulness of echocardiography beyond evaluation of cardiac structures.

#31 Obstructed total anomalous pulmonary venous drainage with coarctation-a rarity

Patil P, Shah S, Kiran VS, PV Suresh, Reddy C

Narayana Hrudayalaya, Bangalore, India

Total anomalous pulmonary venous drainage is a common lesion requiring correction early in infancy. There are numerous case reports in literature describing various associations between total anomalous pulmonary venous drainage and other types of congenital heart disease but its association with coarctation is rarely described. A eight day old neonate was referred to us with history of breathing difficulty. On examination, the baby had cyanosis with saturations of 70% in all four limbs. Cardiac examination revealed loud second heart sound with continuous murmur in left second intercoastal area. Echocardiography showed infradiaphargmatic total anamolous pulmonary venous connection with obstruction at junction of vertical vein to hepatic veins. There was preductal coarctation of aorta with peak systolic gradient of 20 mm Hg (underestimated) with a restrictive duct supplying descending aorta. CT and intraoperative finding confirmed the echocardiography findings. The patient underwent successful coarctation and TAPVC repair.

This association is rare with respect to embryology and diagnosis. Occurrence of both could not be explained by a single interfering event in cardiac development. Absence of differential cyanosis in this patient with patent ductus arteriosus supplying descending aorta can be explained by obstructed pulmonary venous drainage. The complexity of the lesion necessitates a collaborative effort from from cardiologists and surgeons about the best way to deal with such a challenge.

#32 Outcome of fetal cardiac evaluation at tertiary referral centre

Rao NK, Kanchi V, Begum S, Bakshi K, Geetha 1 , Susheela 1 , Seelam S

Department of Pediatric cardiology, Care hospital, Banjara hills and 1 Department of Fetal Medicine, Fernandez hospital, Hyderabad, India

INTRODUCTION : Fetal echocardiography is an established method to diagnose and manage congenital heart defects in the developed world. However it has not evolved to this level in our country.

MATERIALS AND METHODS : Seven hundred and ten fetal echocardiograms referred to two tertiary care centres and performed between January 2004 to May 2010 were analyzed.

RESULTS : The mean age of the patients were 25.9±4.1 years. The mean gestational age (GA) at which fetal echocardiogram performed was 25.6±4.6 weeks. The indication for fetal echocardiograms were high risk pregnancies in 44.7%, suspected heart disease in 22.7%, previous sibling with congenital heart disease in 17.5%, fetal arrhythmias in 6.3% and intracardiac echogenic focus(IEF) in 5.4%. Thirty seven percent were found to be abnormal of which 27.3% were CHDs, 5% were IEF and 4.4% were fetal arrhythmias. Among CHDs; 12.9% were HLHS, 10% HRHS, 7.3% DORV and 6.2% d- TGAs. Tricuspid atresia, single ventricle, AVSD and TOF were 5.7% each. There were 4.1% aortic arch abnormalities, 2.6% truncus arteriosus and 18.6% were in the miscellaneous category. Thirty one cases were fetal arrhythmias of which 54% were benign arrhythmias; 32.3% complete heart block and 16% were SVT. A total of 20 procedures were done within 6 weeks after birth (arterial switch in 6, balloon valvotomy in 6, coarctation repair in 2, systemic to pulmonary shunt in 2, ducus arteriosus stenting in one, permanent pacemaker in 2, and pericardiocentesis in one). Six of 20 neonates expired post procedure.

CONCLUSION : Antenatal diagnosis of CHD prepares the family for furthur management. Majority of the antenatally diagnosed CHDs are either terminated or lost to follow up.

#33 Percutaneous closure of patent ductus arteriosus in children: Immediate and short term changes in left ventricular systolic and diastolic function

Gupta SK, Krishnamoorthy KM, Tharakan JA, Sivasankaran S, Sanjay G, Bijulal S, Anees T

Sree Chitra Institute for Medical Sciences and Technology, Thiruvanthapuram, India

OBJECTIVES : To evaluate the effect of percutaneous closure of patent ductus arteriosus (PDA) in children on left ventricular (LV) systolic and diastolic function.

BACKGROUND : Limited studies are available on alteration in LV hemodynamics, especially diastolic function, after PDA closure.

MATERIALS AND METHODS: Thirty two consecutive children with isolated PDA underwent trans-catheter closure using Amplatzer type ductal occluder. LV systolic and diastolic function was assessed by two- dimensional (2D) echocardiography and tissue Doppler imaging (TDI) one day before PDA closure, on day 1 and at follow up.

RESULTS : At baseline, none of the patients had LV systolic dysfunction. Four (12.5%) had pulmonary hypertension (PH). On day 1 post PDA closure 8 (25%) children developed LV systolic dysfunction. The baseline LV ejection fraction (LVEF), LV end systolic dimension (LVESD) and PDA diastolic gradient predicted post closure LVEF. Baseline LVEF ≥ 63.5% predicted normal post closure LV systolic function with sensitivity of 83% and specificity of 87%. Irrespective of extent of LV systolic dysfunction, all children were asymptomatic and had normal LVEF at follow up. The LV diastolic function improved after PDA closure and trend of improvement continued at follow up. Subjects who developed LV systolic dysfunction post closure had poor LV diastolic function compared to those who did not. LV diastolic properties improved after PDA closure; however the improvement in LV diastolic properties lagged behind the improvement in LV systolic function.

CONCLUSIONs : Percutaneous closure of PDA is associated with reversible LV systolic dysfunction. Improvement in LV diastolic function lags behind improvement in LV systolic function.

#34 Tetralogy of Fallot and congenital mitral stenosis

Manvi V, Pawar R, Dixit M

KLES Heart Foundation, Dr P Kore Hospital and MRC, Belgaum, India

INTRODUCTION: The association of Tetralogy of Fallot (TOF) and mitral stenosis (MS) is extremely rare. However, their recognition is imperative since these potentially lethal anomalies are surgically correctable. The clinical and hemodynamic presentation is similar to that of cyanotic heart disease with pulmonary stenosis and post-capillary pulmonary hypertension. Associations of rheumatic MS with TOF have been described, but congenital MS with TOF is extremely rare.

A 4 yrs old male with a cyanotic congenital heart defect palliated with a left modified Blalock-Taussig (BT) shunt (8 months of age) at another centre, presented to us with NYHA class II symptoms. He was a moderately nourished child with central cyanosis and grade IV clubbing. He had a single second heart sound, with an apical ejection click. Pulse oximetry showed a saturation of 78% at room air. Chest X-ray revealed normal heart size, pulmonary oligemia and no evidence of pulmonary venous hypertension. Echocardiogram revealed TOF with large malaligned perimembranous ventricular septal defect, with severe infundibular and valvar pulmonary stenosis. There was severe congenital mitral valve stenosis with mean pressure gradient (PG) of 11 mmHg across the mitral valve. The mitral chordae tendinae were short and there was a single papillary muscle (parachute mitral valve). The mitral valve area by planimetry on echocardiography was 0.9cm 2 . There was no echocardiographic evidence of rheumatic mitral or aortic valve pathology. There was no mitral regurgitation. The serum antistreptolysin O titers, C-reactive protein and erythrocyte sedimentation rate was normal. In view of the risk and morbidity involved of doing intracardiac repair (ICR) along with possible mitral valve replacement (MVR) at this age, it was decided to medically follow up the patient till he is found suitable for ICR with MVR.

CONCLUSION: Despite the rare association of TOF with MS, the importance of its clinical recognition is mainly related to the surgical correction of both lesions.

#35 Rhabdomyoma with transposition of the great arteries, pulmonary atresia, and ventricular septal defects in a case of tuberous sclerosis-a rare association

Patil P, Mehta A, Shah S, PV Suresh, Rao

Department of Pediatric Cardiology, Narayana Hrudhayalaya, Bangalore, India

Cardiac rhabdomyoma is the most common primary cardiac tumor in infancy and childhood. Cardiac rhabdomyomas frequently occur in association with tuberous sclerosis, an autosomal dominant inherited or sporadically occurring disorder. Very few cardiac malformations such as tetalogy of Fallot or a double outlet right ventricle have been described with tuberous sclerosis and rhabdomyoma. We report a two month old male infant who presented with history of cyanosis and feeding difficulty. There was no family history of any neurological disease. On examination, patient was dysmorphic and had cyanosis. He had cafι au lait spots on his back and ash leaf spots were seen on abdomen. Cardiac auscultation revealed single second heart sound with continuous murmur. Echocardiogram showed multiple rhabdomyomas associated with dextrotransposition of the great arteries, pulmonary atresia, patent ductus arteriosus, multiple ventricular septal defects. Computed tomography brain didn't reveal tuberculomas.

Rhabdomyoma itself can lead to right or left ventricular tract obstruction but associated congenital heart malformations are rare. Presence of both malformations can not be explained on basis of a single embryological insult. Presence of rhabdomyomas can complicate surgical management of primary cardiac condition.

#36 Rare case of isolated interrupted aortic arch

Bobhate P, Tomar M, S.Radhakrishnan, Iyer KS

Department of Pediatric and congenital Heart Disease, Escorts Heart Institute and Research Centre, New Delhi, India

Interrupted aortic arch is defined as absence of continuity between the ascending and descending aorta. It is a very rare disorder with incidence of 3/million live birth. IAA is usually manifests in early infancy and is associated with a large PDA which supplies the descending aorta. We present a rare case of isolated interrupted aortic arch in an 11 year old boy. An 11 year old boy presented to our institution in a very sick state, with feeble pulses of all 4 limbs. Echocardiography revealed a severely obstructive subaortic membrane and interrupted aortic arch type B, severe LV dysfunction and flow reversal in vertebral arteries. The interventricular septum was intact. The patient was stabilized and a CT angiogram was performed. This revealed an isolated interrupted aortic arch type B, aberrant right subclavian, No PDA. The descending aorta was supplied by collaterals from the vertebrobasillar system. The patient underwent successful resection of subaortic membrane and repair of interrupted aortic arch with gortex graft. He had a relatively smooth post operative course with improvement in LV function to 40% at the time of discharge. Surgical treatment of this rare anomaly is relatively simple and produces distinct improvement in cardiovascular status.

#37 Single stage arterial switch in D-TGA with intact IVS At 1-4 months, factors related to prepared LV

Khatri S, Awasthy N, Bobhate P, Tomar M, S Radhakrishnan, Shrivastava S

Department of Pediatric and Congenital Heart Disease, Escorts Heart Institute and Research Centre, New Delhi, India

AIMS : Arterial Switch Operation for TGA-intact IVS beyond the first month of life has been considered at high risk because of a concern over a deconditioned left ventricle. We present interim data from an ongoing study of patients aged 1-4 months with d-TGA with intact IVS who have undergone single stage arterial switch (ASO) in our institute since February 2007. Our focus is on patients whose LV appeared prepared and our aim was to identify the factors associated with this.

MATERIAL AND METHODS : 12 patients having d-TGA with intact IVS underwent single stage ASO in our institute since January 2007. Age range was 30-130 days (median 54 days) and wt range was 3-4.6 Kg (median 3.4 Kg). Male Female ratio was 9:2. The peripheral oximetry ranged from 40-55%. Patients who had large PDA or ASD or LVOT obstruction were excluded. Echocardiographic parameters of 'prepared' LV were LV mass 30-36 gm/m 2 , LVPW thickness 3.2mm-3.4mm and RV free wall 3.6 - 3.9 mm.

RESULT : There was no operative mortality. All patient except one had sternal closure at 2 -7 days (median 4) post operatively. Ventilation duration was 3 -8 days (median 5) and median ICU stay was 6 days (5 - 12 days). Factor for trained LV was identified in them: presence of MAPCAs, branch pulmonary artery stenosis and severe hypoxia and polycythemia. MAPCAs were found in 5 patients; coiled in 4 patients and ligated in one). Unusual origin of pulmonary artery leading to physiological branch pulmonary artery stenosis was found in 4 patients. Severe hypoxia and polycythemia were factors in 2 patients.

CONCLUSION : The left ventricle maintains the potentional for systemic work well beyond the 1 st month of life. Infants can benefit from delayed arterial switch after carefully screening the LV for preparedness and factors responsible for it.

#38 Serial changes in myocardial tissue velocities and longitudinal strain in normal neonates

S Ramakrishnan, Dabral H, Gupta S, Saxena A, Kothari SS, Juneja R, Bahl VK

All India Institute of Medical Sciences, New Delhi, India

INTRODUCTION: Significant hemodynamic changes occur during neonatal period and also the neonatal myocardium is not fully mature. We conducted this study in normal neonates using tissue Doppler imaging to evaluate how the myocardium responds to changing hemodynamics..

MATERIAL AND METHODS : We prospectively studied 16 normal neonates over 4 weeks. Myocardial tissue velocities, time to peak systolic contraction, strain and strain rates were measured at 3-5 days, 2-weeks and 4- weeks. Measurements were done both for LV and RV at the basal and mid wall level.

RESULTS : Systolic and diastolic myocardial tissues velocities increased significantly with each visit. Time to peak systolic S1 and S2 waves i.e., Q-S1 and Q-S2 intervals, decreased significantly over time. However, there was no significant dyssynchrony; time differences between Q-S1 or Q-S2 were not significant when opposing walls of LV, or RV and LV were compared. Myocardial longitudinal systolic strain significantly increased with each visit at TV annulus, MV annulus and mid wall levels across the segments, but the strain rates showed no significant change.

CONCLUSIONs : During the neonatal period, myocardium matures over time with increase in the tissue velocities and reduction in time to peak systolic contraction. There is no dyssynchrony in ventricular contraction. Longitudinal strain increases during this period; however, this is not explained by change in the loading condition alone.

#39 The utility of 3D echocardiography in predicting immediate outcome of percutaneous transvenous mitral commissurotomy

Ramakrishnan S, Kukreti BB, Saxena A, Boopathy N, Goswami KC, Seth S, Bhargava B, Kothari SS, Bahl VK

All India Institute of Medical Sciences (AIIMS), New Delhi, India

INTRODUCTION: We assessed the utility of 3D echo parameters in predicting the immediate outcome after PTMC.

MATERIALS AND METHODS: In a series of 26 patients with severe mitral valve stenosis selected for percutaneous transvenous mitral commissurotomy (PTMC), 3D transthoracic echocardiography was performed before and after PTMC. The mitral valve area (MVA) was assessed pre- and post-PTMC by planimetry, pressure half time (PHT) and 3D methods. The results were compared with those obtained invasively. Additionally, mitral valve volume, SVD (subvalvular deformity) volume, SVD length, Mitral valve excursion, and 3D annular diameter were measured.

RESULTS: The mean MVA at baseline, measured by 3D TTE was 0.67±0.13 cm 2 . The MVA by Gorlin formula increased from 0.71±0.22 to 1.76±0.37 cm 2 after PTMC. The pre-PTMC MVA derived by 3D TTE, PHT, planimetry and Gorlins method were all in close agreement with each other. Post PTMC Gorlin's valve area was significantly related to PHT valve area ( p =0.011), but not to planimetry and 3D valve areas. There were 3 failures (2 grade 3 MR, and 1 MVA<1.5 cm 2 ). Mitral valve volume (1.39±0.82 mL Vs 0.89 mL±0.09 mL in successful vs unsuccessful cases respectively; p =0.032) and mitral valve excursion (1.75±0.2 Vs 1.46±0.65 cm, p =0.032) predicted success of PTMC.

CONCLUSION : Three-dimensional echocardiography is a feasible technique for assessing mitral valve structure in mitral stenosis and may be useful in predicting the immediate outcome of PTMC.

#40 Spectrum of abnormal sites of drainage of pulmonary veins in TAPVC: profile by echocardiography- review from a tertiary care center

Awasthy N, Bobhate P, Khatri S, Tomar M, S Radhakrishnan, Shrivastava S

Department of Pediatric and congenital Heart Disease, Escorts Heart Institute and Research Centre, New Delhi, India

BACKGROUND : Total anomalous pulmonary venous drainage (TAPVC) is essentially an echocardiographic diagnosis characterized by failure of the pulmonary venous confluence to be absorbed into the posterior wall of the left atrium. As a result, a variety of anomalous pathways for drainage of the confluence develop. These sites of abnormal drainage are particularly important before a case is taken to surgical table.

MATERIAL AND METHODS : We are presenting echocardiographic spectrum of abnormal sites of drainage of TAPVC seen by us over the last 5 years (n=87) presenting from 2005 to 2009. Two cases were diagnosed antenatally. Pulmonary venous connection was Supracardiac in 52% cases (46/87), Cardiac via Coronary sinus in 13/87 (15%), infracardiac in 11/87 (12.6%,) and mixed in 17/87 (19.5%). Of the supracardiac type of TAPVC drainage site was to innominate vein, most common in 52%(46/87 cases), to azygous in 0.04% (4/87), to left SVC in 6.5%(6/87) cases, to right SVC in 17%, 15/87 cases, to SVC -RA junction 14% (9/87 cases). Pulmonary venous obstruction (PVO) was present in 48% at presentation, most frequently with infracardiac connection type (P < 0.001), while it was observed in 24% of the supracardiac type, 23% of the cardiac type and 37% of the mixed type of TAPVC.

CONCLUSION : Multiple sites of systemic venous drainage need to be evaluated in a case of TAPVC. Infracardiac type is consistently associated with obstruction.

#41: Unruptured left sinus of Valsalva aneurysm: Case report

Jadhav M, Garekar S, Kulkarni S, Radhakrishan B, Ranganathan A, Mohanty SR, Rao SG

Children's Heart Centre, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Indi a

A 21 year male previously asymptomatic presented with palpitations and dyspnea (NYHA grade II) for 6 months prior to presentation. On examination he had normal vital signs, a hyperdyanamic precordium, normal heart sounds and continuous grade 3/6 to and fro murmur over the right upper parasternal area. The Chest X ray showed borderline cardiomegaly with a localized convex radiological prominence below the pulmonary trunk. ECG showed intermittent atrial ectopics. The 2 D Echocardiogram showed that there was dilatation and aneurysm formation of the left sinus of Valsalva. Diagnostic cardiac catheterization revealed that the aneurysm had a proximal windsock portion and distal sac with a narrow communication and the LCA was arising from the windsock. Surgical repair of sinus of Valsalva was done. At three months follow up he was asymptomatic with decreased atrial ectopics. SVA is a rare cardiac anomaly either congenital or acquired. It is seen most commonly in males with commonest involvement of right followed by noncoronary sinus. The left coronary sinus is least commonly involved. Most unruptured sinus of Valsalva aneurysms are silent. Other modes of presentation includes arrhythmias, heart failure, RVOTO. Suden death due to coronary artery obstruction are reported.

# 42 A neonate with spontaneous pulmonary artery thrombosis - A rare entity

Sapre A, Jadhav M, Garekar S, Kulkarni S, Kadam S, Kurien J, Utpat V, Ranganathan A, Mohanty SR, Rao SG, Hamdullay

Children's Heart Centre, Kokilaben Dhirubhai Ambani Hospital, Mumbai, India

Among children, newborn infants are most vulnerable to development of thrombosis and serious thromboembolic complications. But spontaneous pulmonary artery thrombosis is a rare condition in neonates. We report case of a full term, AGA 15 post natal day neonate, born by cesarian delivery. The antenatal period was uneventful except for oligohydramnios detected in last trimester. The baby had evidence of perinatal-asphyxia but was discharged home on day 3 of life. He was breast fed at home and was apparently healthy until the day of admission. The neonate presented with cyanosis and acute respiratory distress. Echocardiography showed a structurally normal heart, a patent foramen ovale shunting right to left, a PDA shunting left to right, moderate pulmonary hypertension and absent flow in the RPA. A CT scan of the thorax confirmed the suspicion of thrombus in the RPA and also showed thrombi in the distal LPA. After a prothrombotic lab panel was sent, the neonate was successfully treated with thrombolytic and anticoagulant therapy. Possibility of pulmonary artery thrombosis should be kept in mind in cyanotic neonates with a structurally normal heart. Prompt diagnosis and management with thrombolytic and antithrombotic therapy can be life saving.

#43 Wardenburg syndrome with CHD - first ever reported case

Nayak HK, Shetty DP, Suresh PV, Shah SS, Patil SJ

Department of Pediatric Cardiology, Narayana Hrudayalaya Institute of Medical Sciences, India

Extensive literature search shows that CHD is not described as an association of Waardenburg syndrome. A 24 day old, first order, term, male baby born of consanguineous marriage presented with fast breathing and feeding difficulty. Mother had a white forelock as did the maternal uncle and aunt.

On clinical examination baby had tachypnea, tachycardia, cyanosis with saturation of 75% in room air, white forelock of hairs, normal iris, telecanthus, small nose and mandible, white patch over left knee and right abdomen, single transverse palmar crease and right fifth finger clinodactyly. Cadiac auscultation revealed normal first heart sound, widely splitting and loud second sound, grade 2/6 ESM at LUSB. Other systems revealed no abnormality. He was clinically diagnosed as Waardenburg Syndrome with cyanotic CHD. Echocardiography showed mesocrdia and infracardiac TAPVC. Surgical rerouting of TAPVC and closure of ASD was performed. The neonate was discharged in a stable condition.

#44 Cardiac arrhythmias: can they be predicted in postoperative congenital heart

Awasthy N, Bobhate P, Khatri S, Tomar M, Radhakrishnan S, Shrivastava S

Department of Pediatric and congenital Heart Disease, Escorts Heart Institute and Research Centre,New Delhi, India

BACKGROUND : Arrhythmias were a major cause of mortality and morbidity after cardiac surgery for congenital heart disease.

OBJECTIVE :To analyze the incidence, characterize the type of arrhythmias and evaluate the etiological cause in early postoperative period.

MATERIAL AND METHODS : Diagnosis and treatment of early postoperative arrhythmias were prospectively analyzed in consecutive patients who underwent cardiac surgery between November 2007 and August 2008. All children were admitted to ICU, and continuous electrocardiogram monitoring was performed. Risk factors, such as age, weight, type of surgery, cardiopulmonary bypass time, aortic crossclamp time, metabolic parameters, inotropic score, use of deep hypothermia and circulatory arrest were compared. Statistical analysis using the SPSS software (version 13).

RESULTS : Arrhythmias occurred in 35% (n=178/511) patients. Arrhythmias were commonly seen after Fontan surgery, complex CHDs and VSD closure. Most common types of arrhythmia were premature ventricular ectopics (45%), junctional ectopic tachycardia (26%), supraventricular tachycardia (18%), and atrioventicular block (9%). Risk factors for arrhythmias, on univariate analysis were lower age, late presenters, longer cardiopulmonary bypass time(>75 minutes), aortic crossclamp time and use of deep hypothermia and circulatory arrest ( p value<0.05). In the multivariate logistic regression, aortic cross clamp time was statistically significant compared with CPB time (P<</i>.9), age (P<</i>.34). Another significant correlation was high association of the postoperative arrhythmias with the hypokalemia (K<3.3mEq/l) observed within 4 hours of the event in association with high pCO2 levels (CO 2 >40 mm Hg) ( p value- 0.002).

CONCLUSION : Lower age, longer aortic crossclamp time and use of deep hypothermia and circulatory arrest are the risk factors for postoperative arrhythmias. Premature ventricular ectopic followed by junctional ectopic tachycardia and supraventricular tachycardia were the commonest arrhythmias observed in our subgroup. High carbon di oxide (CO 2 levels) in association with hypokalemia were found to have setting for arrhythmia, a setting reported for the first time.

# 45 Clinical genetic evaluation and molecular cytogenetics in tetralogy of Fallot

Kotecha M, Kasar P, Changlan D, La Pl, Pavithran S, Vimala J, Suresh Kumar R

Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India

BACKGROUND : 22q11 microdeletion has been reported in 15% of patients with tetralogy of Fallot (TOF). All cases of TOF require clinical genetic evaluation with Fluorescence in situ hybridization (FISH) study in selected cases.

OBJECTIVE : Clinical genetic evaluation and FISH testing of 51 consecutive patients undergoing corrective surgery for TOF.

METHODS AND RESULTS : 51 consecutive patients with TOF (M : F - 37:14, age 1.5 - 27 years, median age 4 years) were studied by detailed clinical genetic evaluation and FISH testing for 22q11 microdeletion. FISH was positive in 1 of the 51 patients tested. This patient had dysmorphic features in form of hooded eyelids, narrow palpebral fissures, small ears, long narrow face, accessory nipple with delayed development and scoliosis. 21 FISH negative patients had long narrow face, flat malar bones, small chin, long slender fingers, high arched palate, small mouth and abnormal low set ears. Pre auricular tag (n=1), mid facial hypoplasia (n=1), global development delay (n=5), gross delay (n=5) and dysmorphic features in parents (n=4) was observed. Cardiac anomalies in form of DCRV (n=3), aortic arch abnormalities (n=2) and near pulmonary atresia (n=1) were observed in FISH negative patients with TOF.

CONCLUSION : In current series we observed FISH positivity for 22 q 11 deletion in 1 patient even though dysmorphism was seen in 41% patients. This underlines the need for further molecular genetics evaluation to detect more subtle abnormalities in TOF.

#46 Predictors of adverse clinical outcome in Eisenmenger syndrome

Ramakrishnan S, Kukreti BB, Juneja R, Kothari SS, Saxena A, Bahl VK

All India Institute of Medical Sciences (AIIMS), New Delhi, India

INTRODUCTION: Clinical course and predictors of adverse events in Eisenmenger syndrome are not well characterized.

MATERIALS AND METHODS : In this interim analysis, the data of 50 patients of Eisenmenger syndrome followed up for a mean duration of 18.2 months (range 2 to 84 months) is presented. Routine clinical examination and six minute walk test were done at enrolment and follow up visits.

RESULTS : Fifty patients of Eisenmenger syndrome of mean age 24±10 years were followed up for mean duration of 18 months (range 2 to 84 months). Mean six minute walk distance was 475.23±101.4 meters at enrolment and 486.2±115.5 meters at follow up. Adverse events (worsening heart failure and death) were seen in 7 patients. Adverse events were significantly correlated with follow up six minute walk distance 509±90 vs. 348±161 meters ( p =.011) and post 6-min walk heart rate.

CONCLUSION : Worsening of six minute walk distance is a useful and powerful predictor of worsening in Eisenmenger syndrome patients.

#47 Surveillance for atrioventricular nodal conduction disturbances after device closure of membranous ventricular septal defects

Shrinivas C, Ramyashri C, Sreeja P, Bhagyavathy A, Sanjay B, Satish C, Hariprakash S, Sivakumar K

MIOT Hospital, Chennai, India

BACKGROUND: Optimism of transcatheter membranous ventricular septal defects (VSD) closure has dwindled due to occurrence of permanent atrioventricular nodal block (AVB) in 1% of patients.

MATERIALS AND METHODS Patients with membranous VSD with left ventricular dilatation and significant shunt who opted for device closure after informed consent were included. Device size was 0-2 mm more than echocardiographic diameter. Asymmetric devices were used if there is no aortic margin; duct occluders if aortic margin is over 2 mm. Electrocardiograms were done at 0,7,14,30 days, 2,4,6,9,12,18,24 months.

RESULTS : Forty-nine patients aged 2-32 years (median 5 years) with membranous VSD measuring 6-18 mm (median 8 mm) underwent device closure with duct occluders in 10 patients and asymmetric devices in rest. The median device diameter was 10 mm (6-20 mm). Forty-right patients had procedural success. Two patients aged 19 and 22 years had complete AVB after 12 and 14 mm asymmetric devices 2-3 days after procedure, managed with temporary pacing and oral steroids. AVB recovered after 24 and 48 hours with steroids. One 28-year-old lady developed junctional ectopic tachycardia with AV nodal dissociation at 2 weeks reverted after amiodarone. There was no first or second degree AVB in others. Left anterior hemiblock was seen in 5 patients, right bundle branch block in 3, combination in 1 patient without clinical sequelae.

CONCLUSIONs : Early transient AVB occurred in 4% of patients with full recovery after steroids. No patients needed permanent pacing. Benign intraventricular conduction disturbances were seen in 20% patients. Junctional automatic tachycardia is rarely seen after device closures.

Focus category: Pediatric cardiac surgery

#48 Atrial ventricular septal defect: Surgical steps of "Modified one patch", Nunn or australian technique

Murala JS, Singappuli K, Swain SK, Kavunkal AM, Provenzano SC, Karl TR, Nunn GR

Mater Children's Hospital, Brisbane, Australia

INTRODUCTION : Atrioventricular septal defect is a commonly encountered and challenging surgical problem, especially when associated with tetralogy of Fallot. Repair can be performed according to a number of established techniques, often classified by the number of patches employed. We advocate a direct closure of the ventricular communication combined with a pericardial patch septation of the atrium ("modified one patch", Nunn, or Australian technique). The technique is suitable for most (if not all) balanced atrioventricular septal defects and variants such as "large VSD", borderline left ventricular cavity, and associated tetralogy of Fallot as well.

MATERIALS AND METHODS: Herein we demonstrate with digital images and diagrams the surgical steps of the technique described above with a special emphasis on the variants.

CONCLUSION : The "Nunn technique" has universal applicability in complete atrioventricular septal defect. It is quite reproducible and easy to teach to trainees, as there are fewer decisions to be made regarding geometry, size and configuration of patches.

#49 Aortico-left ventricular tunnel: Double patch repair through the tunnel.

Kottayil BP, Wadhwani M, Pillai VV, Panicker VT, Shyamkrishnan KG, Jayakumar KS

Sree Chitra Tirunal Institute of Medical Science and Technology, Trivandrum, India

INTRODUCTION: Aortico-Left ventricular tunnel is a rare congenital heart disease with a paravalvar communication between the aorta and the left ventricle. Surgical closure has been recommended at the time of diagnosis due to risk of developing severe aortic regurgitation and left ventricular failure in patients repaired later.

MATERIALS AND METHODS: A 9 year old child presented with complaints of dyspnea on exertion and was diagnosed to have an aortico-Left ventricular tunnel. Trans-esophageal echocardiogram showed a 20mm defect in the right aortic sinus just above the sinotubular junction that distally opened into the left ventricle Cardiac catheterization confirmed this as type 11 Hovaguimian defect.

RESULTS : Under cardiopulmonary bypass and ostial cardioplegic arrest, the tunnel was opened longitudinally revealing both its ostia. A Dacron patch was used to close the aortic orifice through the open tunnel with direct visualization of aortic valve through the aortotomy, safeguarding its leaflets. Then the ventricular orifice was closed with Dacron patch through the open tunnel protecting the valve leaflets. The intraoperative transesophageal echocardiogram showed complete closure of the tunnel with no aortic valve regurgitation. Cardiac magnetic resonance imaging done at one year on follow up confirmed the complete exclusion of the tunnel with no aortic regurgitation.

CONCLUSION : Surgical repair undoubtedly needs to be tailored for each individual case, with emphasis on reducing the risk of progressive aortic regurgitation. Direct repair using double patch through the tunnel, visualizing the aortic leaflet is easier to perform and gives excellent results in suitable cases.

#50 Double chambered right ventricle: anatomic profile and surgical outcome

Kasar P, Kotecha M, Verghese R, Aggarwal V, Chavan B, Vimala J, Pavithran S, Valliathu J, Suresh Kumar r

Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India

OBJECTIVE: This study evaluated the clinical features and anatomic profile of Double chambered right ventricle (DCRV) and their impact on management.

MATERIAL AND METHODS: 25 consecutive patients (M:F=17:8, mean age 14.2 yrs) seen during a 6 month period at a tertiary care centre were analysed with regard to age at diagnosis, clinical symptoms, echocardiographic diagnosis, cardiac catheterization and surgical details.

RESULTS : Twelve patients (48%) were known to have congenital heart disease from early childhood (median age at diagnosis 3.2 yrs); all of whom had ventricular septal defect (VSD) except one who had been operated for the same in childhood. Seven (28%) had effort intolerance of NYHA class II or more. One had ventricular tachycardia. VSD was perimembranous in 15 (60%), apical in 5 (20%), malaligned in 3 (12%) and outlet in 2 (8%). Fifteen patients underwent cardiac catheterization for detailed anatomic and hemodynamic assessment, the mean intracavity gradient upon cardiac catheterization was 54.1±24.9 mm of Hg. Seventeen (68%) patients underwent surgical resection of DCRV with VSD closure. Four of 5 patients with DCRV and apical muscular VSD were left unoperated while surgery was unsuccessful in one patient. Follow up echocardiogram (median 3 month duration) in operated patients showed a significant decrease in intracavity gradient from 78.4±28.3 mm of Hg preoperatively to 17.2±13.0 mm of Hg postoperatively (p <</i>0.05).

CONCLUSION : All cases of VSD require careful imaging of right ventricular cavity to exclude abnormal muscle bundle which forms the substrate of DCRV.

#51 Ectopia cordis a case report

Mahindrakkar P, Gangopadhyay D, Chattopadhyay A, Bandyopadhyay B

Rabindranath Tagore International Institute of Cardiac Sciences, Kolkata, India

INTRODUCTION: Ectopia cordis is a rare congenital malformation of the heart where the heart is displaced so that it passes out of the thorax and comes either on the outer surface of the body or in the abdominal cavity.

METHODS AND RESULTS : We report a one day old male newborn, born to a primi mother by caesarian section in a remote village. He was referred with a huge pulsatile swelling over his anterior chest. Fetal anomaly scan was negative. On examination the pulsatile mass turned out to be his heart which was lying completely outside of his chest cavity. His heart rate and saturation were 134/min and 90% (in room air) respectively. Central cyanosis was noted and pedal pulses were well palpable. Echocardiography revealed complete AV canal defect, with a 4mm PDA and neonatal pulmonary hypertension. Computed tomography of chest with contrast confirmed the echocardiography diagnosis and revealed absence of upper two third of sternum. Blood investigations revealed sepsis for which intravenous antibiotic therapy was started. Surgical closure was contemplated but eventually the patient died of severe sepsis involving multiple organ systems.

CONCLUSION : Ectopia cordis is a rare and frequently fatal congenital anomaly of the heart. Often it is associated with some other extracardiac anomalies Strict aseptic care has to be maintained until any definitive surgical correction can be undertaken.

#52 Fontan completion - A feasible option at late presentation

Rajagopalan BK, Kottayil BP, Pillai VV, Shyamkrishnan KG, Jayakumar K, Dharan BS

Sree Chitra Tirunal Institute of Medical Science and Technology, Trivandrum, India

INTRODUCTION: Palliation of single ventricle hearts with modified Fontan procedure has been the trend with preference for early surgical intervention. The burden of multistage procedures and immense socioeconomic burden associated with it results in patients coming for Fontan in late childhood and early adulthood in our country.

MATERIALS AND METHODS: Retrospective review of 83 patients who had undergone modified Fontan procedure from 1998 to 2009 (68 Lateral tunnel and 15 Extracardiac Fontan procedures) were included in the study. Group A aged less than 8 years (n=43) while group B were above 8 years of age (n=40). Median age was 5 years (2 - 7.5 years) and 11 years (8- 24 years) with median weight of 15kg (5.5-27 kg) and 23 kg (16-59 kg) in-group A and B respectively. Both groups were followed up for a period of 7.6+/- 3.9 years. Extracardiac Fontan was exclusively done during the last 2 years of the study period.

RESULTS : Mortality was 9.3% in group A and 10% in group B. Fontan takedown was required in 5 patients in group A and 6 patients in group B. On follow-up 83.7% of patients in group A and 90% of patients in group B were in functional class 1. Four patients in group A and 8 patients in group B required medical management for various arrhythmias.

CONCLUSION: Modified Fontan operation can be offered even for those unfortunate patients who present in late childhood and early adulthood without fear of added morbidity or mortality.

#53 Initial experience with the Jostra-Quadrox extracorporeal life support system in a new pediatric cardiac unit

Murala JS, Swain SK, Kavunkal AM, Singappuli K, Karl TR, Nunn GR, Provenzano SC

Mater Children's Hospital, Brisbane, Australia

INTRODUCTION: Extracorporeal Life Support (ECLS) is an important resource in the care of critically ill children. Herein we describe our experience.

MATERIAL AND METHODS: Our centre started in 2008, and ECLS was introduced concurrently. Our team consists of cardiac surgeons, intensivists, cardiologists, perfusionists, coordinator, and specialist nurses. We only used Quadrox D oxygenator and Jostra Rotaflow centrifugal pump (Jostra Medizintechnik AG, Hirrlingen, Germany). All data were reported to ELSO, with comprehensive internal review and educational initiatives for staff.

RESULTS: From June 2008 to June 2010, 27 patients (neonates=12, pediatric=15) required ECLS (33 runs). Indications included respiratory failure (5), and postoperative low cardiac output syndrome (LCOS) (15). In 7 patients ECLS was instituted during cardiopulmonary resuscitation (ECPR). Major complications included hemorrhage (n=12, 44%, CL=26-63%), thrombus in circuit (n=14, 52%, CL=33-71%), neurologic abnormalities (6/27, 22%, CL=7-38%). Probabilities of successful separation from ECLS and hospital discharge were 67% (CI=49-84%), and 60% (CL=42-78%), respectively. 11/15 patients with LCOS survived (73%, CL=51-95%), as did 4/7 patients supported after ECPR (57%, CL=20-94%). For the respiratory group survival was 3/5 (60%, CI 17-100%). Two patients were transported on ECLS to another Australian cardiac unit with successful outcomes.

CONCLUSION : Assuming that survival probability in patients with ECLS approached zero, the use of ECLS has made a significant impact on outcome for our patients. All aspects of ECLS can be rapidly learned and applied in a new unit.

# 54 Intrapericardial diaphragmatic hernia after arterial switch operation

Panda B, Sunil GS

Amrita Institute of Medical Sciences, Kochi, India

INTRODUCTION: Intrapericardial diaphragmatic hernia after median sternotomy for cardiothoracic procedures is a rare complication. It can cause cardiac compression, producing hemodynamic compromise.

METHOD AND RESULTS : A six-month-old child on echocardiographic evaluation for mild tachypnea was found to have an intrapericardial mass compressing the right atrium and right ventricle resulting in early diastolic collapse. She had undergone arterial switch operation with ventricular septal defect closure at the age of two months for transposition of great arteries. CT scan of the thorax showed a paracardiac mass with cardiac compression. On redo median sternotomy, the paracardiac mass was found to be the left lobe of liver and the omentum herniating through a 3cm X 2cm defect in the anteromedial part of the right dome of diaphragm. The liver and the omentum were repositioned back into the abdominal cavity and the diaphragmatic defect was closed with polypropylene mesh using 4.0 polypropylene sutures.

CONCLUSION : An incision far down below the xiphoid process carries a potential for diaphragmatic damage with inadvertent breach in the peritoneum resulting in late herniation of abdominal viscera into the pericardial cavity. Intraoperative insertion of peritoneal dialysis catheter by peritoneal puncture in the subxiphoid area as well as inadvertent puncture of the peritoneum during pericardial and mediastinal drainage tube insertion can also add to the risk. Although the disease is a very rare entity, it must be kept in mind as a possible complication of surgery.

#55 Long standing pulmonary venous baffle obstruction post Sennings: An Indian perspective

Menon S, Panicker VT, Jayakumar K, Shyamkrishnan KG

Department of Cardio Vascular Thoracic surgery, Sree Chitra Tirunal Institute of Medical Science and Technology, Trivandrum, India

INTRODUCTION : Baffle obstruction is a common complication post Senning procedure and can involve either the pulmonary or systemic venous baffle. We present a case of long standing pulmonary venous baffle obstruction who had initially refused surgery and presented years after the initial diagnosis.

A 15 year old boy post Senning procedure (with in-situ pericardium at 3 years age) for d-TGA, VSD presented with functional class II symptoms and 2 episodes of haemoptysis. His past history was significant for a left parieto occipital brain abscess (manifesting as hemiplegia) which was drained at 1 year age followed by hydrocephalus which was treated with V-P shunt. Further, he developed pulmonary venous baffle obstruction at 1 year post Senning surgery. Parents refused surgical intervention at that time. This time he was taken up for baffle correction. The small circumferential constriction in the pulmonary venous baffle was enlarged. The post operative course was uneventful and patient remains asymptomatic at 3 month follow up.

CONCLUSION: In the present era with very few real indications for Senning procedure; pulmonary venous baffle obstruction is a rare presentation providing the surgeon with a surgical challenge. Patients presenting nearly a decade after initial diagnosis is rare but not surprising in Indian population. Our experience shows that even in these patients baffle obstruction can be surgically relieved with good results.

#56 Long term results of truncus arteriosus repair in infancy

Kasar P, Kotecha, Lal P, Vimala J, Pavithran S, Verghese R, Suresh Kumar R

Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India

OBJECTIVE: There is scanty data regarding major morbid events following Truncus Arteriosus (TA) repair in infancy in India. The present study evaluates the long term results with focus on conduit stenosis, pulmonary artery (PA) stenosis and other structural complications.

MATERIAL AND METHODS : 17 patients were identified from hospital data base operated for TA from January 2002 to June 2009.Their detailed chart review was done with respect to their age at surgery, diagnosis, postoperative complications and subsequent morbidity during follow up. Freedom from reoperation or re-intervention was assessed.

RESULTS : A total 17 patients with TA (15 type I, 2 type II) underwent primary repair with reconstruction of RVOT with conduit. Median age and weight at surgery was 3.5 months and 3.8kg respectively. One patient confirmed to have DiGeorge syndrome. Nine patients underwent RVOT reconstruction with aortic homograft where as 7 patients had Contegra bovine jugular vein conduit. There was 1 operative death (6.25%). One patient had a pulmonary hypertensive crisis which was managed with Nitric Oxide. Four patients (25%) required interventions in the form of balloon angioplasty of branch PAs (n=2), bilateral PA stenting (n=1) and radiofrequency ablation (n=1) for SVT. Total 5 (31.2%) patients required re-operation for branch PA and/or conduit obstruction at median of 60 months from first surgery (range 34-100 months). Freedom from all re-interventions and re-operations at 5 years was 60% (95% confidence limits, 30-91%) and 50% (95% confidence limits, 30-91%), respectively.

CONCLUSIONs : Initial results of TA surgery were rewarding with surgical mortality of 6.25%. However freedom from re-interventions at 5 year was 60%. Commonest re-interventions were conduit replacements, balloon angioplasty and PA stenting.

#57 Management of tetralogy of Fallot with absent LPA

Mahindrakar P, Bannerjee P, Das M, Bandyopadhyay B

Rabindranath Tagore International Institute of Cardiac Sciences, Kolkata, India

INTRODUCTION: Although Tetralogy of Fallot is most common cyanotic congenital anomaly with straightforward management options, its association with unilateral absence of pulmonary artery makes it difficult subset to treat.

MATERIAL AND METHODS: The purpose of this retrospective study is to summarize our experience of surgical treatment of TOF with absent LPA. In our institute from March 2006 to May 2010, 13 patients were diagnosed to have TOF with absent LPA.

RESULTS: Of these 10 patients were amenable to total correction (Nakata's Index>200). Eight of them had transannular patch with monocusp in RVOT. Remaining 2 cases underwent total correction with pulmonary valve replacement as they had absent pulmonary valve. Of the remaining three, 2 patients had hypoplastic RPA (Nakata's index<200) hence needed intermediate surgery i.e. Right modified BT Shunt followed by total repair to right lung. The third child, a 2.5 year old boy had hypoplasia of left ventricle hence he had palliative RMBTS. There was one overall mortality related to severe right ventricular failure. It was a 2 year old boy who had total repair with borderline RPA diameter. This child also developed a complete heart block in the post-operative period. One 19 year old girl needed right pleurodesis for recurrent right sided pleural effusion. Another 12 year old boy who initially had total repair with RPA plasty, was found to have significant RPA stenosis after 18 months of surgery requiring stent implantation.

CONCLUSION: Size of RPA and morphology of pulmonary valve play central role in management of these patients.

#58 Valve surgeries in less than 18 Yr old rheumatic patients - Narayana Hrudayalaya experience

Kumar A, Patil P, Raghunath CN, Hegde R, Rao PV, Suresh PV

Narayana Hrudhayalaya, Bangalore, India

INTRODUCTION: Rheumatic heart disease is the leading cause of acquired cardiac morbidity in adolescence and young adults. As the age advances deterioration in valve functions requiring surgical interventions increases. In Narayana Hrudayalaya the number of Rheumatic valve surgeries exceeds seven times in patients of age>18yrs as compared to patients of age<18yrs. However the burden of surgery especially valve replacement is more in younger patients.

OBJECTIVE: To assess the various types of valve surgeries done in rheumatic patients, younger than 18yrs of age.

MATERIAL AND METHODS: A retrospective study done between October 2001 and September 2008. All rheumatic heart disease patients who had undergone cardiac surgery in Narayana Hrudayalaya were included in the study. Type of surgery done, number of valves repaired or replaced, outcome and age profile were documented.

RESULT: 105 patients of age<18yrs had undergone valvar surgeries for rheumatic heart disease in Narayana Hrudayalaya. Mitral valve was most commonly operated valve followed by aortic valve. Mitral valve replacement was more common than repair (n=62 vs. 26). Aortic valve replacement outnumbered valve repair significantly (n=30 vs. 4). Dual valve replacement (MVR +AVR) was carried out in 13 (12%) patients. Tricuspid valve was repaired in 12 patients as an additional procedure during the surgery. There were 3 triple valve surgeries with 1 death. Two patients underwent the Ross procedures. There were 3 deaths (2.8%). The youngest age to undergo valve surgery was 7 years of age.

CONCLUSION : Even in patients younger than 18 yrs of age, valve replacement is more common than repair.

#59 Minimally invasive repair of congenital heart defects

Vaidyanathan KR, Chandrakumar A, Saravanan, Periasamy

Sooriya Heart Insitute Chennai, India

BACKGROUND : We describe our experience with the use of partial sternotomy for the repair of simple congenital heart defects.

MATERIALS : Ten children with ages ranging from 6 months to 10 years (weighing 5kg to 22 kg) underwent repair of simple congenital heart defects (VSD-6, ASD-4) through a partial sternotomy (8 patients) or a sub xiphoid sternotomy (2 patients with ASD).

RESULTS : The mean procedure time ranged from 75 to 122 min and the CPB time ranged from 36 to 64 min. All patients spent less than 6 hours on the ventilator and less than 24 hours in the ICU. All patients had an uneventful recovery and were discharged home between the third and sixth post operative days.

CONCLUSIONs : Partial sternotomy is feasible and safe for the repair of simple congenital heart defects.The advantages are mainly cosmetic but not at the cost of compromising exposure and without the need for any special equipment.

#60 ECMO: A 10 year experience

Malik V, Talwar S

All India Institute of Medical Sciences, New Delhi, India

BACKGROUND: The indications for extracorporeal membrane oxygenation (ECMO) after cardiac surgery have been increasing persistently despite the not very encouraging reports on survival after ECMO support. We reviewed the impact of ECMO on survival in relation to indication and timing of intervention (Integrated ECMO or Emergency ECMO) at our centre.

MATERIAL AND METHODS: Medical records of all patients receiving ECMO support between January 1, 2000 till July 30, 2010 were analyzed.

RESULTS : 94 patients received ECMO support during the 10 year period. Overall survival was 64.8% (61 out of 94). Survival was better in patients with transposition of great arteries when integrated ECMO circuit was used {73% (48 out of 63 patients)}. Survival in patients with cardiac arrest in the postoperative period was 44% (4 out of 9). The major causes of mortality were bleeding and sepsis.

CONCLUSION : Survival chances after ECMO support improve significantly when integrated ECMO circuit is used especially for patients with transposition of great arteries. Better risk stratification leading to use of integrated ECMO circuit during cardiac surgery can improve survival in patients with other correctable congenital heart disease.

# 61 Pulmonary hypertensive crisis after surgical repair of total anomalous pulmonary venous connection - temporal trends and predictors: A single institution experience

Mishra J, Rakhi.B, Nair SG, Sunil GS 1 , Shivaprakasha K 1 , Kumar RK, Vaidyanathan B

Departments of Pediatric Cardiology and 1 Pediatric Cardiovascular surgery, Amrita Institute of Medical sciences, Kochi, India

INTRODUCTION: Pulmonary hypertensive crisis is common after surgical repair of Total anomalous pulmonary venous connections (TAPVC) contributing significantly to mortality and morbidity. There is limited data regarding the temporal trends and predictors of PA crises after TAPVC repair.

METHODS AND RESULTS : 40 patients underwent TAPVC repair during the study period (March 2009 -April 2010). Eleven (27.5%) were neonates. TAPVC types included: supracardiac 15(37.5%), infracardiac 7(17%), cardiac 11(27.5%) and mixed 7(17%); 50% were obstructed. Pre-operative morbidity included congestive heart failure (90%), sepsis (12.5%) and respiratory failure requiring mechanical ventilation (15%). Mean cardio-pulmonary bypass (CPB) and cross-clamp times were 135+74.2 and 64+43.6 minutes respectively. Fifteen patients (37.5%) had ≥2/3 systemic PA pressures while coming off CPB. There were 2 deaths (sepsis -1; low cardiac output without pulmonary hypertension). Postoperative pulmonary hypertensive crisis was seen in 14 patients (35%). PA pressures peaked between 48 - 96 hours after surgery with significant fall after the 4 th post-operative day [Figure 1]. Predictors of post-operative PA crises included: presence of obstruction ( p =0.001) and younger age at surgery ( p =0.03). Patients with PA crisis had significantly longer duration of mechanical ventilation and ICU stay without associated with mortality.

CONCLUSION : Pulmonary Hypertensive crisis is common after surgical repair of TAPVC, predicted by younger age at operation and presence of obstruction. The temporal trends in the PA pressures were characterized by peak values occurring 24 hours after surgery with significant decline after 4 days.

#62 Pulmonary stenosis with intact ventricular septum: evolving surgical strategy and outcome in patients with systemic or suprasystemic right ventricular pressure

Kottayil BP, Balasubrahmonium N, Panicker VT, Jayakumar K, Dharan BS, Shyamkrishnan KG

Sree Chitra Tirunal Institute of Medical Science and Technology, Trivandrum, India

INTRODUCTION: Systemic or suprasystemic right ventricular pressure is thought to be associated with an adverse outcome in patients with pulmonary stenosis with intact ventricular septum. They have a higher incidence of arrhythmia, sudden death, turbulent postoperative course and the long-term outcome following surgical repair is unknown.

MATERIAL AND METHODS: Between 1990 and 2010, 45 patients with pulmonary stenosis and intact ventricular septum in whom echocardiography or catheterization study demonstrated systemic or suprasystemic RV pressure were included in the study. The median age was 17 years (Range 2.5 - 49 years) with median weight of 36 kg. Various procedures were employed in these patients for relief of pulmonary stenosis. Peroperatively 13 patients, following surgical correction showed right ventricular pressure of more than 50% of systemic pressure.

RESULT: There was no mortality at a median follow up of 6.5 years with 43 patients being asymptomatic. RV failure was noticed in 8 patients in the immediate postoperative period. Postoperative significant pulmonary regurgitation ( P <0.001), prolonged cardiopulmonary bypass ( P =0.003) and higher gradient across the obstruction ( P =0.45) were associated with early RV failure. Patients operated recently, in whom a polytetrafluroethylene valve was inserted in the RV outflow tract following relief of obstruction, had a smoother postoperative recovery. On follow up only one patient had right ventricular pressure of more than 50mm of Hg.

CONCLUSION : Tailored surgical procedure with judicious myocardial resection can give excellent mid term results. Avoidance of significant pulmonary regurgitation in the immediate postoperative period facilitates smooth postoperative recovery.

# 63 Successful left ventricular reconditioning and anatomical correction of double discordance in adolescence

Chaudhuri M, M Jayranganath, Chandra VS, Devananda NS

Manipal Hospitals, Bangalore, Inida

INTRODUCTION: Corrected transposition without VSD and normal LVOT is a therapeutic dilemma in older patients. Standard double switch operation fails as morphological LV is fails to handle the systemic afterload after so many years. We report successful LV retraining and subsequent double switch in an adolescent girl .

METHODS AND RESULTS : We report a 12 year old girl with dextrocardia, situs inversus, congenitally corrected transposition of great arteries (CTGA), intact ventricular septum, unobstructed outflows and moderate tricuspid regurgitation presenting with NYHA class II dyspnea at age 8yrs. Her morphological left ventricle had regressed after prolonged exposure to low pressure pulmonary circulation. In view of her symptomatic status, echocardiographic findings and AV valve regurgitation, Pulmonary Artery Banding was done at age 8yrs; it was tightened 6 months later. This iatrogenic pressure overload retrained the LV to function as systemic ventricle and reduce tricuspid regurgitation. Cardiac catheterization 1 years later showed equal LV and RV systolic pressures. She successfully underwent Double Switch Operation at age 10yrs. Two years later, she is thriving well with LVEF of 50% and mild neoaortic regurgitation.

CONCLUSION: The double switch procedure is a superior physiological option in CTGA. In scenarios with regressed LV, the concept of LV retraining is interesting. The question is up to which age the LV can be retrained. We present this case as a hope for late presenters with CTGA.

# 64 Surgical repair of ventricular septal defect and aortic valve regurgitation

Gupta RK, Ralhan S, Singh V, Mittal C, Tandon R, Aslam N, Mohan B, Sood NK, Sharma VK, Wander GS

Dayanand Medical College and Hospital Unit Hero DMC Heart Institute, Ludhiana, India

INTRODUCTION : Different groups have tried to give the precise reasons for propensity of aortic valve regurgitation in patients with subarterial ventricular septal defects. Surgical techniques depend upon the clear interpretation of the anatomy and pathophysiology of the defect.

MATERIALS AND METHODS: Between January 2009 and June 2010 we operated upon 14 patients with a subarterial VSD with a mean age of 12 years. All the defects were located between the commissures of right coronary cusp. In 5 patients the defects were large - either a circular defect or a cresentic defect underneath the right coronary cusp of the aortic valve. In one patient the defect was large enough to include part of non coronary cusp also. Remaining defects can be described as perimembranous or conal VSD. The grade of the aortic regurgitation increased with the age of the patients. The surgical techniques used were as described by Brizard et al. in majority of the cases. We tried to restore the normal height of the cusp, hence a normal surface of apposition and the reduction of the free edge of the right coronary cusp if it is elongated. Three patients were treated with two patch technique where the VSD is closed by a patch anchored to another patch through the prolapsed cusp. This second patch is pulled up with the prolapsed cusp and is then fixed to the aortic sinus. Three patients required valve replacement. Trans-esophageal echocardiography is must during the procedure and none of the patients left operating room with more than mild aortic regurgitation.

RESULTS: There were no hospital deaths. Follow up echo revealed one patient with moderate AR and 3 patients with trivial to mild A

CONCLUSION : It is difficult to achieve perfect results because damage to aortic cusp is irreversible. Operating when the regurgitation is mild is a better option.

#65 Surgical experiences with cor triatriatum and its sequelae

Kottayil BP, Wadhwani M, Panicker VT, Shyamkrishnan KG, Jayakumar K, Dharan BS

Sree Chitra Tirunal Institute of Medical Science and Technology, Trivandrum, India

INTRODUCTION : Cor triatriatum is a rare congenital cardiac anomaly with fewer than 250 cases reported in the literature. The purpose of this study is to review our experience with this rare anomaly with respect to its varying clinical presentation, problems encountered during diagnosis, operative results and outcome.

METHODS AND RESULTS: During the study period from 1990 to 2010, 23 patients underwent evaluation and surgical management of Cor triatriatum in our institute. Age of the patients ranged from 3 month to 23 years. The most common presenting complaint was poor exercise tolerance, while 2 patients were asymptomatic. Five patients underwent emergency surgery. Sixty-five percent had associated congenital cardiac anomalies. There were 5 patients with Lam type A, 8 patients Lam subtype A1, 9 patients Lam subtype A2 and 1 patient of Lam type B. Cor triatriatum was missed in the initial transthoracic echocardiography in 6 atypical cases. Under cardiopulmonary bypass all patients underwent repair through the right atrium. 15 patients underwent a total of additional 21 procedures.

RESULTS: Follow up was 87% complete with mean follow up of 6.5 years. There was one early mortality in a 3-month-old child with obstructed cor triatriatum and cardiac failure. On follow up, the patients are aymptomatic and in sinus rhythm.

CONCLUSION: Cor triatriatum is often associated with other congenital cardiac anomalies and can present with varying clinical picture, often overlooked during evaluation and surgery. Surgical correction offers good early and long term outcome in both classical and atypical cases.

#66 The tale of a missing branch pulmonary artery at bidirectional Glenn shunt

Kottayil BP, Bodhey NK, Pillai VV, Shyamkrishnan KG, Jayakumar K, Dharan BS

Sree Chitra Tirunal Institute of Medical Science and Technology, Trivandrum, Inida

INTRODUCTION: Anomalies of pulmonary arteries and systemic venous drainage may preclude the usage of the normal pathway for creation of bi-directional Cavopulmonary shunt and demand an alternative technique for the procedure.

A 3-year-old child was diagnosed to have isolated dextrocardia, single ventricle physiology, severe pulmonary stenosis, with bilateral superior venacava and was planned for bilateral superior cavopulmonary shunt surgery. Peroperatively the right pulmonary artery was found to arise from the posterior aspect of the left pulmonary artery with an abnormal course to the right inferior to the right pulmonary veins, like a pulmonary artery sling. But, unlike in a true sling, it was coursing anterior to the right bronchus. Under cardiopulmonary bypass, the main pulmonary artery was mobilized, transected and proximal end oversewn. The distal end of main pulmonary artery was overturned and the right-sided Glenn shunt was created by end-to-end anastamosis of the proximal divided end of the right superior venacava to the main pulmonary artery. On the left side the anastamosis was completed in the usual manner. On follow up 1 year after surgery child is doing well with room air saturation of 80%. Cardiac MRI and venous angiogram on follow-up demonstrated functional bilateral cavopulmonary shunt with anomalous course of the right pulmonary artery.

CONCLUSION: The creation of bi-directional cavopulmonary shunt using main pulmonary artery to bridge the gap between the superior venacava and right pulmonary artery helped us to retain the growth potential in the pulmonary tree and hence preserving the patient for a future Fontan procedure.

#67 Vascular ring-variants, Imaging and surgical outcome

Murala JS, Swain SK, Caplin N, Phillips M, Kavunkal A, Provenzano SC, Karl TR, Nunn G

Mater Children's Hospital, Brisbane, Australia

INTRODUCTION: Symptomatic vascular rings are rare congenital lesions with the potential for operative cure. We present a review of recent experience in a paediatric cardiac referral centre with emphasis on the different types of vascular rings, pre-operative imaging to delineate the anatomy, and it's correlation with operative findings.

MATERIAL AND METHODS : The authors reviewed ten children (aged 3 months to 4 years) with vascular rings operated from June 2009 to June 2010 in our institution. The predominant presentations were biphasic stridor and dysphagia. Investigations included CXR in all, barium esophagogram in 4, CT angiogram in 4, bronchoscopy in 5, and MRI in 6. There were 3 patients with double aortic arch,4 with right aortic arch and an aberrant left subclavian artery, 1 patient with left aortic arch with an aberrant right subclavian artery, 2 with right aortic arch with mirror-image branching and a left ligamentum arteriosum and prominent Kommerell's diveticulum.

RESULTS: All patients underwent surgery through left (n=9) or right (n=1) thorocotomy. The intra-operative findings correlated with the pre-operative imaging in all patients. The median follow-up was 6.4 months (1-12 months). There was significant improvement in 6 patients, mild improvement in 3 patients and no improvement or worsening in one patient. This patient had severe tracheo-bronchomalacia.

CONCLUSION: Use of magnetic resonance imaging and computed tomography defines the anatomy accurately and correlates with operative findings. Surgical risk is minimal however long-term follow up is required.

#68 Warden procedure for anomalous pulmonary venous connection to superior vena cava

Dharan BS, Kottayil BP, Menon S, Pillai VV, Jayakumar K, Shyamkrishnan KG

Sree Chitra Tirunal Institute of Medical Science and Technology, Trivandrum, India

INTRODUCTION : Repair of anomalous pulmonary venous connection to superior vena cava with internal pericardial patch may be complicated by obstruction of the superior vena cava or pulmonary veins and sinus node dysfunction. Various techniques are practiced which have reduced the incidence of obstruction to the venous drainage, but sinus node dysfunction continues to be an issue. We reviewed our experience with Warden technique, predominantly in children for various types of anomalous pulmonary venous connection to superior vena cava.

MATERIAL AND METHODS: Between 2006 and 2010, 32 patients with anomalous drainage of the pulmonary veins to superior vena cava underwent repair by the Warden technique in our institute. The median age at operation was 4 years with a mean weight of 15 kg. Partial anomalous pulmonary venous connection was present in 28 patients, while 4 patients had total anomalous pulmonary venous connection to superior vena cava.

RESULTS : At a median follow-up of 24 months there was no mortality. One patient had transient rhythm disturbance. On follow up all patients are in sinus rhythm with no evidence of systemic or pulmonary venous obstruction. Holter analysis done in 9 randomly selected patients on follow-up revealed no rhythm disturbances.

CONCLUSION : By avoiding incision and suturing in the vicinity of superior vena cava right atrial junction, Warden procedure is least likely to cause SA node dysfunction and should be the preferred option for all cases with anomalous pulmonary venous connection to Superior vena cava.

Focus category: General pediatric cardiology including RHD and cardiomyopathies

# 69 An evaluation of efficacy of oral lbuprofen in closing moderate to large ductus in term babies with heart failure: An initial experience from a rural heart centre

Philip S, Sekar P1, Cherian KM1

Division of Pediatric Cardiology, St. Gregorios Cardiovascular Center, Dr. K.M. Cherian Heart Foundation, Parumala, Kerala, 1 International centre for Cardio-thoracic and Vascular Disease Chennai, Inida

AIM : To evaluate the efficacy of oral ibuprofen suspension in closing large patent ductus arteriosus (PDA) in 5-14-days old term babies presented with signs of heart failure.

MATERIAL AND METHODS: A total 14 term-babies, aged 5-14days who were referred from the peripheral neonatology units with heart murmur, respiratory distress and difficulty in feeding were evaluated. The cases diagnosed with other CHDs other than small PFO were excluded from this study. Clinical evaluation with chest x-ray, echocardiography was performed in all cases for the size of PDA, ventricle dimension, La/Ao ratio etc. Out of 14 cases, 8 cases (Group A) had received oral ibuprofen 10mg/kg/Bwt/dose in two doses per day for 7 days in addition to decongestive regime for heart failure, and other 6 cases (Group B) were retrospectively evaluated from the data base of cardiac registery had received decongestive regime alone.

RESULT: Both Groups were evaluated before and after 10days of oral ibuprofen with decongestive regimen for weight, size of the PDA, La/Ao ratio, LV dimension. GroupA (n=8) results pre and post ibuprofen were 2500±565gm vs 2300±260gm weight, 3.21±0.2.2±0.1mm PDA size, 1.3±0.18 vs 1.1±0.12 La/Ao ratio, 1.8±1.1cm vs 1.6±0.7cm LV dimension. Group B (n=6) with decongestive measures showed 2600±425 vs 2200±200gm weight, 3.5±1.2 vs3.2±0.6mm PDA size, 1.2±0.27/1.2±0.17 LA/Ao ratio, 1.9±0.9/1.8±0.9cm LV dimension respectively. No babies showed any adverse effect except feed regurgitation in 3 children was managed symptomatically. Closure of PDA was achieved in 5 of 8 (62.5%) and size of PDA was reduced to less than 2.5mm in the remaining three babies in group A. Where as in Group B, no PDA closeds pontaneously.

CONCLUSION : The seven days of oral ibuprofen therapy in addition to the decongestive regimen we used in this study was easy to administer and no significant adverse effects observed. It was successful for either pharmacological closure or reduction in size of moderate to large ductus in term babies with heart failure, and decongestive regime was tapered in group A much earlier than group B.

# 70 Delineating the spectrum of visceral heterotaxy syndrome

Kotecha M, Ravikumar R, Kasar P, Vimala J, Pavithran S, Lal P, Suresh Kumar R

Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India

BACKGORUND: Anatomic information on heterotaxy syndrome is generally based on pathologic data. The advent of MDCT angiography has given the opportunity to study visceral heterotaxy in detail ante mortem.

OBJECTIVE: To evaluate the anatomic features of visceral heterotaxy syndrome by MDCT angiography and echocardiography.

MATERIAL AND METHODS: Right isomerism (RI) was diagnosed when echocardiographically aorta and IVC were juxtaposed in a patient with complex CHD. Left isomerism (LI) was diagnosed when there was IVC interruption and/or Polysplenia. Cardiac anatomy was evaluated using echocardiography. Systemic and pulmonary venous connections, abdominal viscera and bronchial anatomy were assessed using MDCT angiography.


CONCLUSION : Antemortum establishment of anatomic details is today feasible by a combination of imaging techniques including echocardiography and MDCT angiography. Incidence figures are more truly representative of disease than previously reported pathologic data.

#71 Echocardiographic evaluation of neonates with murmur

Sharma P, Aundhkar CD, Kshirsagar SS

Department Of Neonatology and Cardiology, Krishna Hospital, Karad, India

OBJECTIVE: To evaluate the correlation between murmurs and cardiac diseases in neonates by echocardiography.

MATERIAL AND METHODS: This is a cross sectional study carried out in a tertiary neonatology set up. Neonates were screened for murmur and all neonates with murmur underwent a echocardiography evaluation.

RESULTS : One hundred and eight three neonates with murmur were included in study of which 71 were found to have pathologic cardiac condition. Forty-nine of the 71 had a harsh loud murmur while 4 had a continuous murmur. Of 112 neonates with an innocent murmur, patent foramen ovale, patent ductus arteriosus and moderate tricuspid regurgitation were found in 42. In 70 neonates, the echocardiogram was normal. In those with pathological murmurs the incidence of various cardiac diseases were VSD-34%, Tricuspid aresia- 3.4%, TOF- 13.5%, TAPVC-4.5%, PDA-10.5%, Pulmonary stenosis-3%, ASD-9%, TGA-4.5%, DORV-1.5%, VSD+PS-1.5%, VSD+RVOTobs-4.5%, VSD+ASD+PDA-1.5%, PDA+PS-1.5%, ASD+VSD-4.5% and Pulmonary atresia 1.5%.

CONCLUSION : In neonatal period auscultation remains an important tool for detection of heart disease. In present era with easy availability and affordability of echocardiographic machines, it is advisable to screen all babies with a murmur by echocardiogram and rule out underlying cardiac lesion before discharge.

#72 Kawasaki disease - clinical and echocardiographic findings at diagnosis and follow up

Dande V, Prabhu S, Venkatesh S, Kulkarni S

B. J. Wadia Hospital For Children, Mumbai, India

Kawasaki disease (KD) is an important cause of acquired heart disease in children.

OBJECTIVE : The clinical spectrum of KD, incidence and risk factors associated with coronary aneurysms were analysed.

PATIENTS AND METHODS: Records of 37 children diagnosed with KD were evaluated for an initial coronary involvement and their outcome on follow up. Bieser and Harada scoring system was used to predict the outcome. IVIG and high dose aspirin were advised as initial therapy.

RESULTS: Eight-three percent were less than 5 years of age, the youngest being 4 months. The male/female ratio was 2:1. 54.05% presented between November to February with delay in diagnosis ranging from 6 to 44 days (median 9 days). Incomplete presentation was noted in 64.9%. Cardiac abnormalities were found in 72.97% of patients by echocardiography, with ectasia at ostium (37.07%) and aneurysm (18.15%) and segmental dilatation in 27 (72.97%). Patients who were diagnosed late had later regression of dilated segments ( p =0.007). High Bieser score was predictive of later regression of the dilated segments ( p =.021) with no such correlation noted using Harada score. High platelet count post IVIG and high absolute eosinophil count increased the risk of having aneurysms. On 6 month follow-up, 2 new aneurysms developed, one previous aneurysm regressed. Twenty-five percent and 51.85% of dilated segments regressed by one year and post one year follow up respectively. Ninety percent of the ectatic segments and 20% of aneurismal segments eventually showed regression. No deaths were noted.

CONCLUSION: Prevalence in age less than 5 years, male predominance, occurrence in winter months and incomplete presentation were noted in this study. High Bieser score was predictive of later regression of the dilated coronary segments.

#73 Lifestyle factors in childhood obesity

Patankar N, Prabhu S, Venkatesh S, Chhabria D

Division of Pediatric Cardiology, Department of Pediatric Medicine, B. J. Wadia Hospital for Children, Mumbai, India

Obesity is the new age epidemic and a slow killer with its roots predominantly in our lifestyle and behaviour.

MATERIAL AND METHODS: Thirty overweight children who met inclusion criteria were evaluated over a period of 1 year. A detailed history physical examination was done in all. Body Mass Index Percentile (BMI % tile) for their age and sex as per the NCHS/CDC charts was used for classification. Relevant investigations to assess metabolic parameters were done.

RESULTS : Thirteen of 30 patients had onset of obesity before one year of age, with 10 being obese and 3 overweight ( P =0.036). No correlation was found between parental obesity and BMI of children. Fourteen of the obese children belonged to nuclear families (66.7%) as compared to 9 (100%) who stayed in a joint family ( P =0.048). Poor level of motivation was observed in 22/30 children and average level in 8/30 ( P =0.037). Excess carbohydrates and fats consumed and the decrease in protein intake by the children correlated significantly with the BMI ( P values of 0.001, 0.002, and 0.003 respectively). Amount of protein consumed by those children who were overweight was significantly more than those who were obese ( P value 0.001). 93.3% consumed food prepared using saturated fat as compared to 2 (6.7%) who used unsaturated fat ( P =0.008). Sedentary hours spent correlated significantly with the BMI ( P =0.01), with statistically significant hours spent outdoors by overweight group than in the obese group ( P =0.007). Children with high BMI had increased Left ventricular mass index (LVMI). LV Mass showed a positive correlation with systolic and diastolic blood pressure readings. Total Chol/HDL ratio was found to be statistically significant in relation to BMI ( P =0.032).

CONCLUSION : Grade of obesity statistically correlates with age of onset, type of family, degree of physical activity, diet and raised total Cholesterol/HDL ratio and motivational level. Childhood obesity definitely has a significant correlation with the lifestyle factors.

#74 Management of coronary artery fistulas in current era

Kasar P, Kotecha M, Vimala J, Pavithran S, Suresh Kumar R

Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India

OBJECTIVE : This study evaluated strategies in managing coronary artery fistulae (CAF) with focus on transcatheter interventions.

MATERIAL AND METHODS: All cases of CAF diagnosed in a tertiary centre from June 2007 to June 2010 formed the study group. The diagnosis, anatomy, clinical presentation and further management was assessed.

RESULTS : 18 patients (M: F-5:13, Age range 11 months to 68 year) were studied. The fistulae originated from the right and left coronary artery in 7 and 11 patients respectively. Drainage was to the right atrium in 10 (4 at SVC-RA junction), right ventricle (6), left ventricle (1) and main pulmonary artery (1). MDCT angiography was performed in 9 patients. These patients were divided into Category A: Medical follow up (n=3), Category B: Transcatheter closure of fistulae (n=8) and Category C: Surgical closure of fistulae (n=7). Patients with small fistulae and age less than one year were followed medically. Surgery was offered when there was other concomitant surgery {valve replacement (n=2), iatrogenic fistulae (n=2) or large fistulae (n=2)} or risk to major coronary or its branch on transcatheter intervention (n=1). Transcatheter closures of fistulae were done at the exit point by creating an arteriovenous loop and using a duct device (Amplatzer 3, Cocoon 5) from the venous end. Both surgical and transcatheter closure resulted in complete occlusion within 24 hours in all patients. Follow up studies at median of 15 months showed complete closure of fistulae without opening of any new channels. All the patients had normal ECGs. All patients with transcatheter closure received aspirin.

CONCLUSION : Coronary artery fistulae require careful evaluation for age of the patient, location of fistule and its hemodynamic consequences for optimal choice in management. Options include non interventions, surgical closure and transcatheter occlusion. Transcatheter occlusion produces excellent results in selected cases.

#75 Role of socio demographic factors on the distribution of blood pressure in children

Manu Raj, Sundaram KRa, Paul M, Sudhakar A, Kumar RK, Francis E

Departments of Pediatric Cardiology and Biostatisticsa, Amrita Institute of Medical Sciences and Research Centre, Kochi, India

OBJECTIVE : The study aims to determine the role of socio demographic factors on the distribution of blood pressure in school children.

MATERIAL AND METHODS: Stratified random sampling method was used to select children from schools in Ernakulam District, Kerala, South India. Blood pressure and anthropometric data were collected from 20,263 students of 5-16 years age during 2005-06. Blood pressure Z scores in relation to sex, age, weight and height were calculated using polynomial models. Mean of the Z scores were used for comparisons between socio demographic subgroups.

RESULTS: Rural children exhibited significantly higher blood pressure levels when compared to urban children. Government school children exhibited significantly higher blood pressure levels when compared to private school children. The prevalence of high systolic blood pressure (SBP) was significantly more for rural girls than urban girls (2.5% Vs 1.8%). The prevalence of high diastolic blood pressure (DBP) was significantly more for rural boys than urban boys (1.9% Vs 1.4%). The prevalence of high SBP was significantly more for both boys and girls from government schools when compared to those in private schools (3.2% Vs 2.4% among boys and 2.3% Vs 1.0% among girls). The prevalence of high DBP was significantly more in government school girls than in private school girls (2.1% Vs 1.2%).

CONCLUSION: There exists an inverse relationship between socio economic status and blood pressure in children. Low socioeconomic status appears to be a risk factor for higher blood pressures in school children.

# 76 Risk factors for prosthetic heart valve thrombosis in children and the results of thrombolytic therapy

Ramakrishnan S, Dabral H, Kothari SS, Kukreti BB, Gupta SK, Juneja R, Choudhary SK, Saxena A, Bahl VK, Airan B

All India Institute of Medical Sciences, New Delhi, India

INTRODUCTIONINTRODUCTION: Optimal therapy of prosthetic valve thrombosis (PHVT) is still unclear. Thrombolytic have been promoted as an alternate to surgery. No data is available for thrombolytic therapy in paediatric population.

MATERIAL AND METHODS: We retrospectively studied children aged 12 years or younger who underwent prosthetic valve replacement in our institution over the last 10 years. Data of similar aged children who underwent thrombolytic therapy for prosthetic valve thrombosis during the same time period was evaluated and analyzed.

RESULTS : A total of 108 patients were identified who had PHV replacement, of which there were 18 episodes of thrombosed PHV treated with thrombolytic in 14 patients. Fifteen valves (12.29%) out of the 122 valves replaced had thrombosed. Age, sex, indication for surgery (compared for RHD and CHD), size of prosthesis was not significantly different in the two groups. Following thrombolytic treatment, 7 (50%) patients showed complete clinical success, 6 patients showed partial clinical response and 1 patient had failed thrombolytic therapy. Only one patient (7.1%) who presented with class IV symptoms died. Two (14.28%) patients had embolic complications.

CONCLUSIONs : PHVT incidence is similar in pediatric age group, as compared to adults. Thrombolytic therapy is feasible for PHVT in children but may be less effective.

# 77 Potential prophylactic value of bovine colostrum in children with congenital heart disease presenting with frequent respiratory tract infections; An initial experience from a rural heart centre

Philip S, Cherian KM 1

1 Division of Pediatric Cardiology, St. Gregorios Cardiovascular Center, Mannar, Kerala, A Unit of Frontier Life Line and Dr. K.M. Cherian Heart Foundation, Chennai, India

OBJECTIVE : To evaluate the efficacy of bovine colostrums-hyper immune milk powder in preventing frequent allergic episodes and respiratory tract infections in children with congenital heart disease.

MATERIAL AND METHODS: We studied 75 children aged 1month to 5 years with congenital heart disease who had recurrent episodes of respiratory tract infections. There were 53 children with acyanotic heart disease without heart failure who formed the test group. There were 14 children with heart failure on decongestive regime and 13 children with cyanotic heart disease with frequent wet cough waiting electively for intra cardiac repair. The study group children received supplementation Bovine Colostrum (Pedimune; ) 3gm per serving per day for 3months. The control group (with frequent chest infections (n=22) not due to cardiac cause) received placebo 3gm milk powder. Total no of episodes of recurrent infections, hospitalization rate, overall well being and adverse events were assessed at every 4 weeks.

RESULTS : Respiratory infections, including frequent wet cough and wheezing episodes were reduced significantly by 80 to 86% at the end of therapy. Frequent therapy with antibiotics in test group was significantly reduced and better oxygen saturation was observed when supplemented with colostrums. The control group in contrast required frequent antibiotic therapy, and had less oxygen saturation due to recurrent infections. High to average improvement in overall well-being in 96% of the patients in both cyanotic and acyanotic congenital heart disease were found by pediatrician while overall well-being stated by the patient/care taker was very good to good in 90.56% of the patients.

CONCLUSION: Bovine colostrum was highly effective in preventing allergic chest infections such as recurrent wet cough and wheezy episodes in association with congenital heart disease. The number of episodes requiring antibiotic therapy was significantly reduced and better oxygen saturation level was observed in cyanotic heart disease when supplemented with colostrums milk powder. This initial observation may need further multi center double blind studies.

#78 Spectrum of adult congenital heart disease: Initial experience at a new centre

Jadhav M, Garekar S, Kulkarni S, Radhakrishnan B, Ranganathan A, Mohanty SR, Rao SG

Children's Heart Centre, Kokilaben Dhirubhai Ambani Hospital, Mumbai, India

We describe demographics in adult patients with CHD presenting to the our pediatric cardiac OPD since its inception. From July 2009 to May 2010, we had 32 adult (>18years age) patients with CHD. Of these 13 were females, 19 were males. Eleven of the females were married and had atleast one living child with an uneventful pregnancy. The most common defects were Atrial septal defects (n=11), Tetralogy of Fallot (n=5), Large Ventricular septal defects (n=3), DORV (n=3), Ebstein's anomaly (n=2), Tricuspid atresia (n=2), bicuspid aortic valve with aortic stenosis, RVOTO, Unruptured LSOV, Corrected TGA, Idiopathic pulmonary arterial hypertension (n=1 each). Twenty one patients were symptomatic at first visit. Breathlessness was the commonest symptom. Twenty three were unoperated at initial visit due to various reasons.

# 79 Quadrivalvar rheumatic disease - A rare entity

Karunakaran V, Shah S, Patil P, Maheshwari S, Suresh PV, Rao PV

Narayana Hrudhayalaya, Bangalore, India

In the current scenario where the severity of rheumatic heart disease is declining, it is rare to find a patient with quadrivalvar involvement. We report a child of rheumatic heart disease who presented to us with exertional dyspnea. On clinical examination, patient had elevated mean jugular venous pressure with prominent V wave and Y descent. Cardiac auscultation revealed loud second heart sound with diastolic murmurs at apex and left lower parasternal area. Echocardiography showed stenosis of all four cardiac valves with thickening of leaflets. The knowledge of the possible involvement of even the pulmonary valve in children is essential for preoperative evaluation in rheumatic heart disease. Involvement of all four cardiac valves in rheumatic heart disease is a rarity even in developing countries, especially in the current era when the incidence and severity of rheumatic heart disease has reduced.

# 80 Atypical presentations of infective endocarditis

Jadhav M, Garekar S, Kulkarni S, Radhakrishnan B, Ranganathan A, Mohanty SR, Rao SG

Children's Heart Centre, Kokilaben Dhirubhai Ambani Hospital, Mumbai, India

A 14 year old previously healthy girl presented with progressive dyspnea and palpitations for 7 days prior to admission. She had a history of pharyngitis and fever 1 month prior to admission. On examination she had tachycardia, tachypnoea, orthopnea, normal BP and peripheral pulses. She had a loud P2, grade 3/6 PSM and grade 2/6 MDM at the apical area and bilateral basal crepts. Her ECG and Chest X ray were normal. Her ASO was borderline positive and did not show an increasing trend upon repeat testing. Echocardiography showed moderate mitral regurgitation with a ruptured mitral chorda. There was subvalvar crowding. The mitral valve did not appear rheumatic afflicted. Her blood specimen grew Streptococcus Sanguis (Streptococcus viridans). She was given a 6week course of appropriate antibiotics. Mitral valve repair using artificial chordae was performed at a later date.

A 9 year old boy with a complex congenital cyanotic heart disease previously palliated with a BT shunt was admitted for high grade fever, abdominal pain, and jaundice for 4-5 days prior to admission. On examination he had tachycardia, tachypneoa, icterus, tender hepatomegaly and splenomegaly. He had single 2 nd heart sound and grade 3/6 PSM at LLSB. LFT showed elevated liver enzymes and bilirubin. His echocardiogram was suspicious for a 'thickened' appearance of the atrial septum. The blood cultures were negative. He was treated as culture negative IE with a complete course of antibiotics. He responded to the treatment.

Infective endocarditis is one of the most dreaded complications of structural heart disease. Its presentation may be atypical. Ruptured mitral chorda and hepatitis are rare presentations.

#81 The spectrum of cardiac manifestations in Noonan syndrome

Mahadevan S, Shah S 1 , Nampoothiri S 2 , Bhat M

Centre for Human Genetics, Bangalore, 1 Department of Pediatric Cardiology, Narayana Hrudayalaya Institute of Cardiac Sciences, Bangalore, 2 Department of Medical Genetics, AIMS and RC, Kochi, India

Noonan Syndrome (NS) is an autosomal dominant genetic disorder with facial dysmorphism, congenital cardiac defects and short stature. The incidence is approximately 1 in 2,000 making it the most common CHD syndrome after Down's syndrome. Mutations in the PTPN11 gene are causative in approximately 40% of cases. Pulmonary valvular stenosis (PS), atrial septal defect (ASD) and hypertrophic cardiomyopathy (HOCM) are the most frequent cardiac defects. Less frequent lesions include ventricular septal defects (VSD), coarctation of Aorta, mitral insufficiency (MI) and Patent ductus arteriosus (PDA). 67 clinically diagnosed Indian patients with NS were tested for PTPN11 gene. Mutations were identified in 18 individuals (26.9%). Cardiac evaluation with echocardiography was performed in all by a paediatric cardiologist. The commonest cardiac defect in clinically diagnosed 23/49 (46.9%) and mutation positive 11/18 (61.1%) individuals was PS. ASD was seen in 44.4% and 28.5% of mutation positive and negative individuals respectively. Other structural defects included PDA 7.4% (5/67), MI 7.4% (5), VSD in 4.5% (3), HOCM in 4.5% (3) and coarctation of aorta in 3.3% (2).Structural cardiac defects were reported in 85% of PTPN11 mutation positive cases in a recent study of 274 cases. We report cardiac defects in 95% cases (n=64). ASD was present in 44.4% of our mutation positive group versus 26.9% in Sznajer et al, 2007. HOCM was seen in only 4.5% of our cases as compared with 33% in other studies. Right sided heart defects are more common with PTPN11 mutations. Peripheral pulmonary stenosis was not reported in our study.

Focus category: Pediatric cardiac intensive care including pre operative issues and anesthesia

# 82 Anomalous left coronary artery from the pulmonary artery with left ventricular dysfunction: Successful early outcomes without ECLS

Kumar G, Dinkar S, Kaw A, Gupta R, Girhotra S, Iyer PU, Radhakrishnan S, Shrivastava S, Iyer KS

Escorts Heart Institute and Research Centre, New Delhi, India

BACKGROUND: Excellent early outcomes have been described in Anomalous left coronary artery from the pulmonary artery (ALCAPA) repairs mostly with the use of assist devices or ECLS for failure of separation from cardiopulmonary bypass (del Nido P.J et al).

OBJECTIVE: To evaluate the early outcome of ALCAPA repair without resorting to post-operative mechanical support. Design: A prospective observational study (Jan 1996 - Mar 2009).

MATERIAL AND METHODS: Twenty patients underwent aortic re-implantation for ALCAPA during this period - median age 108 days, 45 days - 45 years. Sixteen of 20 had significant left ventricular systolic dysfunction (EF<40%) and 12/ 16 had severe LV dysfunction (EF<30%, median 20%). Four of the 16 required preoperative stabilization (ventilation -1, inotropic support-4). All 16 underwent emergency surgery. Surgical strategies included 1) Judiciously deferring mitral valve repair (3/5), 2) Minimizing CPB times - median 96 min (48-160 min) and myocardial ischaemia (aortic cross clamp time) - 38 min (30-75min), 3) Deferred sternal closure (8/16), 4) Aggressive afterload reduction and 5) Epicardial echo guided gradual weaning from CPB. Post repair epicardial echo revealed varying degrees of LV dysfunction in 15/16 and 3/5 had significant residual mitral regurgitation. Perioperative low cardiac output was managed pre-emptively using multiple, simple, inexpensive conventional strategies - continued afterload reduction, milrinone, calcium infusion, corticosteroids for refractory low output, elective and prolonged noninvasive ventilation and avoidance of catecholamines.

RESULTS : In-hospital mortality was 0/16 and there were no late deaths. Median ventilatory requirement was 122 hours and median length of hospital stay was 15.5 days. 3/16 had significant ventricular arrhythmias and 4/16 had reversible renal injury.

CONCLUSION : Successful ALCAPA repair in sick infants with severe ventricular dysfunction is feasible without ventricular assist with minimum morbidity.

#83 Bosentan in post operative patients with pulmonary arterial hypertension

Jadhav M, Garekar S, Kulkarni S, Radhakrishnan B, Ranganathan A, Mohanty SR, Rao S

Children's Heart Centre, Kokilaben Dhirubhai Ambani Hospital, Mumbai, India

We describe our experience of the use of Bosentan in six post operative patients with severe pulmonary arterial hypertension. The underlying cardiac anatomy was TAPVC (2), dTGA (2) and one each of Truncus Arteriosus and large PDA. The patients had preoperative PAH as well. After surgery they had PA crises and failed repeated extubation attempts (n=5). The PAH was demonstrable either as PA line display or on echocardiography (TR jet; septal movement). One patient presented with new onset PAH on 1month OPD follow up (DTA, VSD. Bosentan was begun (dosage as per literature description) after continuous oxygen or sildenafil failed to adequately impact the PAH. Liver function tests were obtained prior to beginning therapy. Post Bosentan, four of the six patients showed improvement. Of the remaining two, one required reoperation while one expired. Endothelin appears to be important in the pathogenesis of PAH. The dual endothelial receptor antagonist Bosentan decreases endothelin induced smooth muscle cell contraction, hypertrophy and hyperplasia and fibrosis. The use of bosentan in pediatric patients with PAH after cardiac surgery is not well documented. Here we have shown that bosentan could be safely used in our postcardiac surgery patients and short term results demonstrate its efficacy in this situation. A controlled trial is the need of the hour.

#84 A Rare cause of pulmonary haemorrhage in intraoperative period

Srivastava A, Warrier G, Trehan M, Sivadasan A, Radha

Apollo Hospital, Hyderabad, India

INTRODUCTION : The complications following cardiac surgery can be many. Timely identification of the problem helps in altering the treatment strategy and improving the outcome.

MATERIAL AND METHODS: A symptomatic 12 year-old girl with Rheumatic Heart Disease and Severe Mitral Regurgitation was scheduled for elective Mitral valve repair/ replacement . After narcotic based anaesthesia, arterial and central venous (neck) lines were secured. Under normothermic cardiopulmonary bypass and cardioplegic arrest, mitral valve replacement was done as the valve was unsuitable for repair. After coming off bypass, there was transient hypotension which did not respond to volume replacement and inotropes. At the same time, there were copious pink frothy secretions from the endotracheal tube. Transesophageal echocardiography (TEE) was done which was unremarkable. But the unexplained endotracheal tube bleeding persisted. Chest X Ray revealed good position of endotracheal tube, neck line and chest tubes with diffuse haziness of right lung. Causes of unilateral pulmonary edema were considered.

RESULTS : On a repeat TEE, we realised that the echo contrast was coming into left atrium (LA). Surprisingly, the tip of the neck line was found to be in right upper pulmonary vein. So, part of the drugs and volume administered were entering LA and part of it seeping into the lung. This would explain unilateral haziness on chest radiograph and hypotension not responding to inotropes or volume replacement. The neck line was then removed followed by rapid improvement.

CONCLUSION : Logical thinking and stepwise analysis is cornerstone for problem solving. An open mind is helpful to identify uncommon causes.

#85 Arrhythmias following congenital heart disease surgery

Chavan B, Selvakumar G, Murmu U, Pavithran S, Vimala J, Varghese R, Aggarwal V, Suresh Kumar R

Institute of Cardiovascular Disease, Madras Medical Mission, Chennai, Inida

BACKGROUND: Arrhythmias increase the mortality and morbidity after CHD surgery. The incidence, risk factors and treatment require careful study.

AIM: To assess the incidence, predisposing factors, response to treatment and outcome of early postoperative arrhythmias following CHD surgery.

MATERIAL AND METHODS:This is a prospective evaluation of 100 consecutive cases admitted to a PICU following CHD surgery from 1 st April 2010 to 14 th June 2010. Diagnosis, operation procedures, intra operative parameters (CPB and aortic cross clamp time) and postoperative parameters were recorded. Continuous ECG monitoring was done for all patients throughout their PICU stay. Isolated PVCs were not included for analysis.

RESULTS : Of the total 100 cases (M: F=69: 31), 86 were corrective surgeries and 14 were palliative surgeries. Arrhythmias were noted in 20/100(20%) - 17/20(85%) developed<24 hours of surgery. Underlying CHD was cyanotic in 14/20 (70%) and acyanotic in 6/20 (30%). Among children with arrhythmias 2/20 (10%) had palliative surgeries done. 6/20(30%) of arrhythmias occurred in the age group of 1 - 5 years. Arrhythmias commonly occurred after surgeries for TOF (55%), VSD (20%), ASD (10%) and TGA (10%). The most common arrhythmias noted were junctional rhythm (20%), complete heart block (20%), JET (10%) and ventricular tachycardia (10%). The mean CPB was 150±87 min ( P <0.05) and (AXC) time was 100±56 ( P <0.05)min, which was significantly higher compared to group without arrhythmias (CPB-85.31±50.21) and (AXC-48.18±34.17). At the time of appearance of arrhythmias, the mean values for electrolytes and temperature in the normal range. Treatment was given in 10/20 (50%) cases, which included anti arrhythmic drugs in 6/10 (60%) (Digoxin in 67% and amiodarone in 33%), temporary pacing in 3/10 (30%) and permanent pacemaker was implanted in 1/10(10%). There was only one mortality due to ventricular tachycardia after arterial switch surgery.

CONCLUSION : i n an unselected group of patients who underwent CHD surgery, significant arrhythmias were recognized in 20%. Cyanotic CHD, young age, prolonged CPB time - AXC time were more likely to be associated with postoperative arrhythmias. Anti arrhythmic drugs and epicardial pacing were generally effective in controlling arrhythmias though there was one mortality.

#86 Early extubation failure in postoperative pediatric cardiac patients and their immediate outcome

Patil P, Raghunath CN, Kumar A, Maheshwari S

Narayana Hrudyalaya, Bangalore, India

OBJECTIVE: To determine risk factors associated with early extubation failure in pediatric postoperative cardiac patients and assess immediate outcome.

MATERIALS AND METHODS: This is prospective, observational study. We observed 1360 consecutive post operative patients over period of SIX months. Out of these, 76 patients had extubation failure in 48 hours. Preoperative and postoperative data were analyzed and outcome was assessed in terms of days of post extubation ventilation, PICU stay and mortality. All values are in median and ranges unless otherwise stated. Chi square test was used for categorical data.

RESULTS : The median age of study group was 10.5 months (10 days-5years) with average weight was 6.8kg (2.5kg- 24kg). Intracardiac repair followed by arterial switch operation were most commonly associated with reintubation. The average CPB time required for surgery was 103+/-23.2 minutes. The mean duration of ventilation prior to extubation was 60+/-14.48hrs with 46.68% patients requiring prolonged ventilation (>72hrs). Severnty two percent patients had wet lung fields prior to extubation with 10.7% having consolidation and 15.72% having diaphragmatic palsy with 6 requiring diaphragm plication. Eight out of 44 paralyzed patients required reversal prior to extubation. Fifty two patients were on fentanyl infusion with average duration of 44.23hrs (17hrs-118hrs). Twelve patients (15.7%) had neurological abnormality. The average inotropic score was 13.22+/-7.2 (5-36). The most common indications for reintubation was respiratory distress, cardiac arrest, PAH crisis and diaphragm palsy. The average duration of ventilation after reintubation was 8+/-2.2days. The study group has prolonged PITU stay in study as compared to control group. ( P value -0.032). The mortality among study group was 18.82% compared to average mortality of 3.2%.( p -0.02).

CONCLUSION : The factors associated extubation failure are age group<2 years, weight<10kgs, prolonged CPBtime and prolonged ventilation prior to extubation. Extubation failure is associated with increased morbidity and mortality.

#87 How reliable is occlusion alarm of syringe pump infusing inotropes?

Patil P, Kumar A, Raghunath CN, Hegde R

Narayana Hrudyalaya, Bangalore, India

INTRODUCTION : Postoperative pediatric cardiac surgical patients have myocardial dysfunction for initial 12-24 hrs. Therefore inotropic support is vital for initial smooth recovery. The syringe pump has been designed to alarm if occlusion pressure exceeds desired limits.

OBJECTIVE: To evaluate the occlusion alarm time of syringe pump at various sensitivity and flow rate.

MATERIAL AND METHODS: Three calibrated syringe pumps (PERFUSOR COMPAQ of B.BRAUN) were used in the study. Syringe size used was 20ml and 50ml. To mimic the clinical setting, 200cm extension tubing was attached to syringe and to the other end a three-way stopcock was attached. Syringes were filled with normal saline and the tubings including the 3-way were flushed with normal saline. The alarm time was recorded with occluded 3-way at the rate of 1ml/hr, 2ml/hr, 5ml/hr and 10ml/hr using alarm setting of P-1, P-2 and P-3.

RESULTS : At a low flow rate of 1 ml/hr, the time for alarm occlusion (min:sec) were 7:55:07+/- 51.99, 14:28:47+/-1:43:01, 34:06:64+/-5:09:18 with 50 ml syringe at P1, P2, P3 sensitivity setting respectively. The corresponding values for the 20 ml syringe were 5:17:57+/-45:55, 8:33:07+/- 28:82, 10:09:59+/- 36:50. At high flow rate of 10 ml/hr, time for alarm occlusion were 50:93+/-6:67, 2:55:80+/-10:76, 3:18:75+/- 8:75 for 50 ml syringe. At 5ml/hr and 2 ml/hr flow rates, findings were intermediate between 1 ml/hr and 10ml/hr flow rate. The occlusion alarm times for 20 ml syringe were shorter than those for 50 ml syringe ( p -0.02). At a lower flow of 0.5 mL/hr, none of the pumps alarmed despite 30 minutes of distal occlusion, regardless of syringe size and sensitivity.

CONCLUSION : Occlusion alarms of syringe pump infusing inotropes are not reliable and have a high variability. To detect early occlusion, smaller syringe size, higher flow rate and higher sensivity setting (p1) should be used for inotrope infusion.

#88 Initial experience with high frequency oscillatory ventilation in paediatric cardiac surgery

Sonthalia A, Rakhi B, Nair SG, Sunil GS, Vaidyanathan B

Amrita Institute of Medical Sciences, Kochi, Kerala, India

Sepsis is one of the major complications following complex congenital heart surgery in neonates and infants. Secondary infection of the lung leading to ARDS (Acute Respiratory Distress Syndrome) and causing severe hypoxemia is not an uncommon feature in these patients. Various modes of ventilation can be used to overcome the low oxygen levels in blood although there is no single mode of ventilation, which is favoured. Minimal literature is available on the use of alternate modes of ventilation including high frequency oscillatory ventilation (HFOV) especially following cardiac surgery. We report 4 cases of severely hypoxemic children following sepsis and lung infection after congenital cardiac surgery who were managed on high frequency oscillatory ventilation. HFOV was used for an average of 100 hours. We could salvage 2 patients whereas other 2 died of septic shock.

HFOV is a useful alternative to conventional ventilation in paediatirc cardiac surgical patients who have severe hypoxemia due to lung infection in the post-operative period.

#89 Recombinant activated factor vii in the management of excessive bleeding following open-heart surgery for congenital heart disease

Changlani D, Chavan B, Murmu U, Kumar S, Varghese R, Agarwal V, Vimla J, Pavithran S, Suresh Kumar R

Institute of Cardiovascular Diseases, Madras Medical Mission, Chennai, India

BACKGROUND: Postoperative bleeding requiring the administration of blood products, haemostatic drugs and/or re-exploration are associated with increased morbidity, mortality, and resource consumption. Recombinant activated factor VII (rFVIIa) is new therapeutic agent which offers an effective treatment strategy for patients with excessive bleeding.

OBJECTIVE: To evaluate the efficacy of recombinant activated factor VII for excessive bleeding following open-heart surgery for congenital heart disease.

METHODS: This is a prospective study of 5 consecutive cases (median age of 4.9 yrs, median CPB time of 303 min) of excessive postoperative bleeding following congenital heart surgery admitted to pediatric ICU during period from August 2009 to June 2010. Their demographic, procedural, and hematologic data were analyzed. All patients had persistent intraoperative bleeding not responding to blood products or excessive postoperative chest tube drainage (>10ml/kg/hr). All five patients received 80-90mcg/kg single dose of rFVIIa intravenously over 2-3 min. Time to cessation of bleeding or chest tube drainage<1ml/kg/hr was accessed. No patient required surgical reexploration.


CONCLUSION : In five cyanotic patients undergoing complex open-heart surgery, rFVIIa was effective in controlling excessive bleeding within a mean period of 45mins, avoiding the need for re-exploration. Recombinant FVIIa is an important drug in the pharmacologic management of postoperative bleeding.

#90 The severely pulmonary hypertensive ventricular septal defect: Simplifying postoperative care

Kumar G, Gupta R, Dinkar S, Kaw A, Girotra S, Radhakrishnan S, Shrivastava S, Iyer KS, Iyer PU

Escorts Heart Institute and Research Centre, New Delhi, India

BACKGROUND : The severely pulmonary hypertensive large ventricular septal defect in the older child continues to be a major post operative challenge in the non-industrialized world. Various surgical and expensive medical strategies are randomly used.

OBJECTIVE : To evolve a simple, inexpensive and reproducible model of peri-operative care which can be applicable in most parts of the non- industrialized world.

DESIGN : A prospective pilot observational case series.

MATERIAL AND METHODS : 12 patients (median age 8 years 6 months, 3 years 4 months - 26 years), (median weight 24 Kg, 11.5-68 Kg), underwent closure of a large ventricular septal defect in 2008. Median PVRI was 8.2 wood units(3.4-13.3 wood units) - all responding favorably to oxygen with reduction in PVRI by 25% to 70%. Surgical strategies included decompressive PFO in 3 and flap valve ASD in 1. Serial post repair ECHO revealed varying degrees of LV systolic dysfunction in all (median EF 40%). Perioperative pulmonary hypertension was managed pre emptively using multiple, simple, inexpensive conventional strategies (alogorithm based) - optimal lung recruitment, relatively early extubation, dobutamine, nitroglycerine and milrinone in 6/12 patients. Inhaled nitric oxide and other expensive vasodilators were not used.

RESULTS: In-hospital mortality was 0/12 with no late deaths. Morbidity was acceptable - median ventilatory requirement (40.5 hours, 16-95 hours), length of stay (9.5 days, 6-17 days) and inotrope score (10.2, 5-23.5). There was no renal failure, acute lung injury or sepsis.

CONCLUSION: Preliminary observations suggest that a simple and inexpensive model of perioperative care is feasible in patients with large sevely hypertensive ventricular septal defects. These observations need to be validated in larger numbers of patients in multiple centres.

#91 Utility of routine echocardiography in ICU in the immediate post operative period

Alva P, Suresh PV, Colin John, Shah S, Karunakar, Patil P, Nayak HK, Maheshwari S

Narayana Hrudayalaya, Bangalore, India

INTRODUCTION : A prospective longitudinal study done to asses the utility of routine echocardiography in ICU in the immediate post operative period management.

MATERIAL AND METHODS : A total of 526 consecutively operated cases of congenital and acquired heart disease between April 1 st 2009 and June 20 th 2009 were enrolled. Intra cardiac repair for TOF (110) VSD repair (85), ASD repair (85) BT shunt (36) TAPVC repair (28) Arterial Switch Repair (18) AV Canal repair (19) constituted the majority of the cases. All patients were evaluated by echocardiography in the immediate post operative period defined in our study as within the first 24 hours. Repeat ECHO done in unstable patients were also included.

RESULTS: Post operative echocardiography influenced decision making in 45.8% (241) cases. Decision to increase ionotropic support was taken in 18.5% (96) cases. In 15.58% (82) echo influenced the decision to extubate. In 9.88% (52) extubation was delayed based on echo findings of poor ventricular function. Heparin infusion was stopped in 0.57%(3) post BT shunt. ICD was inserted for pleural effusion in 57% (3) patients detected on 2D echocardiography. Surgical re-exploration was done in 38% (2)cases significant pericardial fluid collection. Revision of BT shunt was done in 0.19% (1) for blocked shunt.

CONCLUSION : Echocardiography is a useful tool for the immediate postoperative management of complex congenital heart disease patients . However routine post -operative echocardiography has limited role in simple congenital heart diseases.

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