Annals of Pediatric Cardiology
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   2014| January-April  | Volume 7 | Issue 1  
    Online since February 5, 2014

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Anticipation and management of junctional ectopic tachycardia in postoperative cardiac surgery: Single center experience with high incidence
Osama Abdelaziz, Salem Deraz
January-April 2014, 7(1):19-24
DOI:10.4103/0974-2069.126543  PMID:24701080
Background : Junctional ectopic tachycardia (JET) often occurs in the early postoperative period following surgery for congenital heart diseases and may lead to hemodynamic compromise. Its exact etiology is unknown, however, longer cardiopulmonary bypass (CPB) time, aortic cross clamp (ACC) time, catecholamines, and hypomagnesemia are known risk factors. JET is associated with increased postoperative morbidity and mortality. Materials and Methods: A prospective cohort study of 194 consecutive children who underwent open heart surgery on CPB over 1 year period, patients was divided into three groups; JET, non-JET arrhythmia, and no arrhythmia groups. Information on patient's demographics (sex, age, and body weight), type of surgical interventions, duration of CPB and ACC, the use of inotropic support, duration of intensive care unit (ICU) stay, and response to different therapeutic methods were collected. Results: JET was documented in 53 patients (27%) most commonly following tetralogy of Fallot (TOF) repair and was associated with longer CPB and ACC times (118 and 77 min, respectively) as compared to non-JET arrhythmia (93.9 and 55.3 min, respectively) and no arrhythmia groups (94.9 and 54.8 min, respectively). Patients with JET required more inotropic support and longer ICU stay as compared to other groups. Amiodarone was safe and effective in treatment of JET. Atrial electrocardiogram (ECG) and Lewis lead ECG were helpful tools in JET diagnosis. The mortality was 11.5% in JET patients. Conclusions: Incidence of JET was 27% possibly due to the large number of Fallot repair and Senning operation. Longer CPB and ACC times are risk factors for JET.
  6,509 509 2
Risk factors for morbidity in infants undergoing tetralogy of fallot repair
Alexander C Egbe, Alexander J Mittnacht, Khanh Nguyen, Umesh Joashi
January-April 2014, 7(1):13-18
DOI:10.4103/0974-2069.126539  PMID:24701079
Background: Primary repair of tetralogy of Fallot (TOF) has low surgical mortality, but some patients still experience significant postoperative morbidity. Aim: To review our institutional experience with primary TOF repair, and identify predictors of intensive care unit (ICU) morbidity. Settings and Design: Medium-sized pediatric cardiology program. Retrospective study. Subjects and Methods: We retrospectively reviewed all the patients with TOF and pulmonic stenosis who underwent primary repair in infancy at our institution from January 2001 to December 2012. Preoperative, operative, and postoperative demographic and morphologic data were analyzed. ICU morbidity was defined as prolonged ICU stay (≥7 days), and/or prolonged duration of mechanical ventilation (≥48 h). Statistical Analysis Used: Multiple logistic regression analysis. Results: Ninety-seven patients underwent primary surgical repair during the study period. The median age was 4.9 months (1-9 months) and the median weight was 5.3 kg (3.1-9.8 kg). There was no early surgical mortality. Incidence of junctional ectopic tachycardia (JET) and persistent complete heart block was 2 and 1%, respectively. The median length of ICU stay was 6 days (2-21 days) and median duration of mechanical ventilation was 19 h (0-136 h). By multiple regression analysis, age and weight were independent predictors of length of ICU stay, while surgical era was an independent predictor of duration of mechanical ventilation. Conclusion: Primary TOF repair is a safe procedure with low mortality and morbidity in a medium-sized program with outcomes comparable to national standards. Age and weight at the time of surgery remain significant predictors of morbidity.
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Post cardiac surgery junctional ectopic tachycardia: A 'Hit and Run' tachyarrhythmia as yet unchecked
Jaganmohan A Tharakan, Kiron Sukulal
January-April 2014, 7(1):25-28
DOI:10.4103/0974-2069.126545  PMID:24701081
  5,245 328 2
Prenatal diagnosis of isolated interrupted inferior vena cava with azygos continuation to superior vena cava
Do Thi Cam Giang, Gayatri Rajeesh, Balu Vaidyanathan
January-April 2014, 7(1):49-51
DOI:10.4103/0974-2069.126558  PMID:24701087
Absence of inferior vena cava is an uncommon congenital abnormality. It is usually associated with other structural anomalies, typically left isomerism. We report a case of interrupted inferior vena cava with azygos continuation diagnosed as an isolated finding during routine prenatal ultrasound scan, confirmed by post-natal echocardiography. Detailed ultrasound examination of the fetal anatomy failed to demonstrate other anomalies. The neonatal course of this fetus was uneventful.
  4,866 247 1
Clinical spectrum of infantile scimitar syndrome: A tertiary center experience
Hadeel Al Rukban, Mohammed Al Ghaihab, Omar Tamimi, Suhail Al-Saleh
January-April 2014, 7(1):29-33
DOI:10.4103/0974-2069.126547  PMID:24701082
Aim: Infantile scimitar syndrome is a rare condition, with most of the literature reports being limited to case reports and a few case series. The aim of this study was to review patients with infantile scimitar syndrome who presented to our hospital from July 2000 to January 2011. Materials and Methods: In this retrospective study, we evaluated the medical records of patients aged 0-14 years who were symptomatic before the age of 1 year and were subsequently diagnosed with the syndrome. A total of 16 patients with the infantile form of scimitar syndrome were identified from the database. Results: The median age at presentation was 14 days, with a median age at diagnosis of 55 days. Fifty-six percent of the patients were females. Tachypnea was the major presenting symptom and 13 out of 16 patients had pulmonary hypertension. Of the 13 patients with pulmonary hypertension, 7 had systemic collaterals, which were treated by coil occlusion together with medications, and 3 had corrective surgery. The mortality rate was 3/16 (18.8%) over the 10.5 years study period. Conclusions: Infantile scimitar syndrome is a rare congenital anomaly that needs a high degree of suspicion for early referral and management. The association of the syndrome with pulmonary hypertension leads to recurrent and prolonged hospitalization.
  4,727 370 9
Early and 1-year outcome and predictors of adverse outcome following monocusp pulmonary valve reconstruction for patients with tetralogy of Fallot: A prospective observational study
Deepa Sasikumar, Bijulal Sasidharan, Jaganmohan A Tharakan, Baiju S Dharan, Thomas Mathew, Jayakumar Karunakaran
January-April 2014, 7(1):5-12
DOI:10.4103/0974-2069.126538  PMID:24701078
Background and Objectives: Repair of tetralogy of Fallot (TOF) with monocusp pulmonary valve reconstruction prevents pulmonary regurgitation (PR) for a variable period. Since postoperative outcome is governed by PR and right ventricular function, we sought to assess the severity of pulmonary regurgitation and right ventricular outflow (RVOT) gradient in the immediate postoperative period and at 1 year and attempted to identify the anatomical substrates responsible for adverse outcomes. Methods: The study included 30 patients. Transthoracic echocardiography was performed before surgery, within 5 days of surgery, and 1 year later. Presence and severity of PR, RVOT gradient, and residual branch pulmonary stenosis were assessed. Right ventricular and monocusp valve functions were studied. Results: Median age was 36.5 months (3-444 months). There were no deaths. Pulmonary regurgitation was mild in 18, moderate in 10, and severe in 2 patients immediately following surgery. At 1 year, 10 patients had severe PR and one had significant RVOT gradient. None of the variables like age, presence of supravalvar pulmonary branch stenosis, main pulmonary artery diameter, or mobility of monocusp valve was found to have any significant association with the progression of PR. McGoon index <1.5 showed a trend toward more PR, while patients with more residual RVOT gradient had lesser regurgitation. Conclusions: Repair of TOF with monocusp pulmonary valve reduces immediate postoperative PR. At 1 year, the monocusp valve underwent loss of function in a significant proportion and PR also progressed. This study could not identify any predictors of progression of PR, though patients with McGoon index <1.5 tended to have more PR while those with more outflow gradient had lesser PR.
  4,093 369 2
Crisscross heart with dextrocardia and intact interventricular septum
P Kader Muneer, Sajeer Kalathingathodika, Govindan Sajeev Chakanalil, Manuel M Sony
January-April 2014, 7(1):70-71
DOI:10.4103/0974-2069.126571  PMID:24701094
Crisscross heart is a rare congenital heart disease characterized by a twisted atrioventricular connection, as a result of rotation of the ventricular mass along its long axis. We report an asymptomatic 48-year-old woman referred to us for evaluation of a cardiac murmur. Further evaluation showed situs solitus, dextrocardia with normal atrioventricular and ventriculoarterial connection, and a crisscross relation of the atrioventricular valves. Unlike the usual case of crisscross heart, our patient had an intact ventricular septum.
  4,157 260 1
Transcatheter device closure of a traumatic ventricular septal defect
Mohamed Kasem, Hari Krishnan Kanthimathinathan, Chetan Mehta, Richard Neal, Oliver Stumper
January-April 2014, 7(1):41-44
DOI:10.4103/0974-2069.126552  PMID:24701085
A traumatic ventricular septal defect (VSD) resulting from blunt chest injury is a very rare event in children. The clinical symptoms and timing of presentation are variable, so diagnosis and management of traumatic VSD may be challenging. Decision to close the traumatic VSD is usually based on a combination of severity of heart failure symptoms, hemodynamics, and defect size. We present a case of a 7-year-old boy who was run over by a truck and presented with head and liver injury initially. He was subsequently found to have a traumatic VSD. The VSD was closed percutaneously.
  3,667 139 3
Transvenous closure of large aortopulmonary collateral
Parag W Barwad, Gurpreet Singh Gulati, Saurabh K Gupta, Anita Saxena, Balram Airan, Sivasubramaniam Ramakrishnan
January-April 2014, 7(1):34-36
DOI:10.4103/0974-2069.126549  PMID:24701083
Aortopulmonary collaterals (APCs) are occluded either preoperatively or at the time of cardiac surgery in patients with pulmonary atresia and ventricular septal defect (PAVSD). If left untreated, APCs are an important cause of deterioration in the early postoperative period. We present here an unusual case with a large residual APC causing refractory low-output state in the early postoperative period. Usual arterial approach failed due to extensive angulation with ostial narrowing. The large residual APC was successfully closed with an Amplatzer duct occluder (ADO) device delivered through the transvenous route.
  3,182 222 -
Ebstein anomaly and Trisomy 21: A rare association
Stephanie L Siehr, Rajesh Punn, James R Priest, Alexander Lowenthal
January-April 2014, 7(1):67-69
DOI:10.4103/0974-2069.126569  PMID:24701093
This is a case report of a patient with Trisomy 21 with Ebstein anomaly, a ventricular septal defect, and acquired pulmonary vein stenosis; a rare combination, diagnosed during a routine neonatal examination.
  3,189 177 1
Transcatheter therapy for Lutembacher's syndrome: The road less travelled
Ramalingam Vadivelu, Saujatya Chakraborty, Shiv Bagga
January-April 2014, 7(1):37-40
DOI:10.4103/0974-2069.126551  PMID:24701084
An 18-year-old male with Lutembacher's syndrome underwent balloon mitral valvotomy (BMV) and device closure of the atrial septal defect (ASD). BMV necessitated technical modification of taking the Inoue balloon over the wire (OTW) into the left ventricle (LV). The procedure was complicated by slippage of ASD device into the right atrium, which was managed successfully by percutaneous retrieval, and deployment of a larger device. The case highlights the challenges associated with the seemingly easy transcatheter therapy for this disease entity.
  3,153 196 -
Umbilical venous catheter retrieval in a 970 gm neonate by a novel technique
Arima Nigam, Vijay Trehan, Vimal Mehta
January-April 2014, 7(1):52-54
DOI:10.4103/0974-2069.126559  PMID:24701088
Umbilical venous catheterization is a necessity for the advanced care of very low birth weight neonates. Even with utmost care, few complications cannot be avoided. Fractured and retained catheter fragments are one of them. Endoluminal retrieval of such a catheter is an uncommon and challenging procedure for the interventionist. The only alternative is an open exploration of these patients. Various techniques have been described for retrieval of such foreign bodies. We describe a novel technique for percutaneous retrieval of an embolized umbilical venous catheter from a very low birth weight neonate.
  3,153 188 1
Successful management of multiple infected sub-mitral aneurysms of left ventricle
Vinitha Viswambharan Nair, Rajat Kalra, Rajiv Narang, Balram Airan
January-April 2014, 7(1):45-48
DOI:10.4103/0974-2069.126555  PMID:24701086
Sub-mitral aneurysms are rarely reported clinical entity. Though a variety of etiologies are proposed, congenital weakness of the mitral valve annulus is the most widely accepted one. A 17-year-old boy with sub-mitral aneurysm presented with severe mitral regurgitation, which was diagnosed by echocardiography and successfully managed surgically. The aneurysm wall was positive for Staphylococcus aureus, and patient was treated with intensive antibiotics.
  3,172 148 1
Dysphagia Lusoria with atrial septal defect: Simultaneous repair through midline
Rithin Rathnakar, Saket Agarwal, Vishnu Datt, Deepak K Satsangi
January-April 2014, 7(1):58-60
DOI:10.4103/0974-2069.126562  PMID:24701090
An aberrant right subclavian artery from the descending aorta is almost always reported as an isolated anomaly. We present the case of a four-year-old child with an anomalous origin of the right subclavian artery from the descending aorta, associated with an ostium secundum atrial septal defect. The patient underwent simultaneous repair of both the anomalies through median sternotomy, with implantation of the subclavian artery into the right common carotid artery. We believe that median sternotomy is the optimal surgical approach for the management of these lesions. Other operative approaches are also discussed.
  3,176 143 2
Percutaneous closure of patent ductus arteriosus in interrupted inferior caval vein through femoral vein approach
Endale Tefera, Ramon Bermudez-Cañete
January-April 2014, 7(1):55-57
DOI:10.4103/0974-2069.126560  PMID:24701089
Percutaneous closure of the patent arterial duct in patients with interrupted inferior caval vein poses a technical challenge. A 12-year-old girl with a patent ductus arteriosus (PDA) and interrupted inferior caval vein is described in this report. The diagnosis of interrupted inferior caval vein and azygos continuation was made in the catheterization laboratory. A catheter was advanced and snared in the descending aorta. An exchange wire was advanced through the catheter and snared in the descending aorta. Then, an Amplatzer TorqVue 2 delivery sheath was advanced over the wire from the venous side and again snared in the descending aorta. An Amplatzer duct occluder (ADO) size 8/6 was advanced through the sheath while still holding the sheath with a snare. The device was opened. The sheath was then unsnared once the aortic disc was completely out. The sheath and the device were pulled back into the duct and the device was successfully implanted. The device was then released and it attained a stable position. An aortic angiogram was performed which showed complete occlusion.
  2,945 186 2
Redux valvular surgery with coronary artery bypass graft in familial hypercholesterolemia
Ziadi Jalel, Mleyhi Sobhi, Ben Omrane Skander, Khayati Adel
January-April 2014, 7(1):61-63
DOI:10.4103/0974-2069.126563  PMID:24701091
Familial hypercholesterolemia (FH) is a dominantly inherited disorder caused by mutation at the locus for the low-density lipoprotein (LDL) receptor and is frequently associated with premature coronary artery disease and aortic valve involvement. The surgical treatment of these complications is accompanied by a high degree of risk, even in skillful hands. An intensive cholesterol-lowering therapy and LDL aphaeresis in association with surgery may be useful. The case of a 12-year-old girl, with a medical history of familial hypercholesterolemia is reported here, operated two years previously for valvular aortic stenosis; Ross intervention was done. She was readmitted for acute coronary syndrome. Three coronary artery bypass grafting was performed with saphenous veins with positive results.
  2,792 167 2
Pseudo interruption of the inferior vena cava complicating the device closure of patent ductus arteriosus: Case report and short review of venous system embryology
Venkateshwaran Subramanian, Krishnamoorthy Kavassery Mahadevan, Sivasankaran Sivasubramonian, Jaganmohan Tharakan
January-April 2014, 7(1):64-66
DOI:10.4103/0974-2069.126567  PMID:24701092
A nineteen-month-old girl was taken up for patent ductus arteriosus (PDA) device closure. A diagnostic catheter from the right femoral venous access entered the superior vena cava (SVC), through the azygos vein suggesting interruption of inferior vena caval with azygos continuity. Therefore, the PDA device was closed from the right jugular venous access. However, a postprocedure echocardiogram (echo) showed a patent inferior vena caval connection into the right atrium. An angiogram from femoral veins showed communication between the iliac veins and the azygos system, in addition to normal drainage into the inferior vena cava (IVC). Congenital communication between the iliac veins and the azygos system can mimic IVC interruption. An attempt to theoretically explain the embryological origin of the communication has been made.
  2,666 159 2
Distilling wisdom from our collective experience
R Krishna Kumar
January-April 2014, 7(1):1-4
DOI:10.4103/0974-2069.126537  PMID:24701077
  2,597 209 -
Branching vertical vein with multiple sites of obstruction in supracardiac total anomalous pulmonary venous connection
Navaneetha Sasikumar, Shanthi Chidambaram, Raghavan Subramanyan, Koothurathu Mammen Cherian
January-April 2014, 7(1):72-73
DOI:10.4103/0974-2069.126575  PMID:24701095
We present the case of an infant with total anomalous pulmonary venous connection and a branching vertical vein with multiple points of narrowing, draining the confluence into the innominate vein. The embryology and clinical relevance of this interesting anatomy is discussed.
  2,286 154 -
Infliximab as rescue therapy in refractory Kawasaki disease
Moisés Rodríguez-González, Miguel A Matamala-Morillo, Antonio Segado-Arenas
January-April 2014, 7(1):74-75
DOI:10.4103/0974-2069.126578  PMID:24701096
  1,792 172 3