Annals of Pediatric Cardiology
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   2013| January-June  | Volume 6 | Issue 1  
    Online since February 16, 2013

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Pathology of Takayasu arteritis: A brief review
Pradeep Vaideeswar, Jaya R Deshpande
January-June 2013, 6(1):52-58
DOI:10.4103/0974-2069.107235  PMID:23626437
Takayasu arteritis (TA) is a chronic idiopathic and granulomatous vasculitis, manifesting mainly as a panaortitis. Autoimmune cell-mediated immunity is probably responsible for the disease. The inflammation commences from the adventitia and progresses to the intima and leads to, both in adults and children, segmental stenosis, occlusion, dilatation, and/or aneurysm formation. This review focuses briefly on the etiopathogenesis, and describes the pathological and clinical features in adults and children.
  12,646 1,176 43
Pacing in children
Harinder R Singh, Anjan S Batra, Seshadri Balaji
January-June 2013, 6(1):46-51
DOI:10.4103/0974-2069.107234  PMID:23626436
The implantation of cardiac pacing devices in children and young adults can be challenging and different from the adult population due to their smaller size, their longer life expectancy, and anatomical variations associated with congenital heart defects. A knowledge of indications, pacing leads and devices, anatomical variations, and the technical skills are important for those who implant and care for children with pacemakers. In this review we attempt to discuss these specific points of cardiac pacing in children and young adults.
  8,594 696 13
Complete heart block due to diphtheritic myocarditis in the present era
Mithun J Varghese, Sivasubramanian Ramakrishnan, Shyam S Kothari, Akhil Parashar, Rajnish Juneja, Anita Saxena
January-June 2013, 6(1):34-38
DOI:10.4103/0974-2069.107231  PMID:23626433
Diphtheria continues to be reported from many parts of the world. Complete heart block is rare but often fatal complication of diphtheric myocarditis. We report six children with diphtheric myocarditis who presented with complete heart block. Three patients survived, one with persistent complete heart block. Aggressive supportive management including transvenous pacing may result in complete recovery in a significant number of children with diphtheric myocarditis.
  7,868 419 6
Anti-platelet agents in pediatric cardiac practice
Sweta Mohanty, Balu Vaidyanathan
January-June 2013, 6(1):59-64
DOI:10.4103/0974-2069.107236  PMID:23626438
Pediatric patients with a variety of congenital and acquired cardiac conditions receive antithrombotic therapy. Many of the indications are empirical, and have either not been proven in controlled studies or are extrapolated from adult studies. This article reviews the current available literature regarding the use of anti-platelet drugs in the pediatric cardiac population.
  5,609 624 10
Pulsatile neck mass: A rare cause
Dattatreya P.V. Rao, Rajiv Ananthakrishna, Manjunath C Nanjappa
January-June 2013, 6(1):95-96
DOI:10.4103/0974-2069.107247  PMID:23626449
A 20-year-old female presented with a pulsatile neck mass. On evaluation, she was found to have right cervical aortic arch, which is a rare anomaly. We highlight the conventional and Computed tomography angiography features of this vascular anomaly.
  5,608 244 1
Holt-oram syndrome associated with double outlet right ventricle: A rare association
Bhupinder Singh, Mallesh Kariyappa, Ishwarappa Balekundri Vijayalakshmi, Manjunath C Nanjappa
January-June 2013, 6(1):90-92
DOI:10.4103/0974-2069.107245  PMID:23626447
Holt-Oram syndrome is a rare inherited disorder that causes abnormalities of the hands, arms, and the heart. Most commonly, there are defects in the carpal bones of the wrist and in the bones of the thumb along with cardiac defects such as atrial or ventricular septal defects. We report a case of Holt-Oram syndrome with a rare association of double outlet right ventricle.
  5,629 205 2
Differential diagnosis of vascular structures in relation to upper ascending aorta: The retro-aortic innominate vein
Kiron Sukulal, Sasidharan Bijulal, Jaganmohan A Tharakan
January-June 2013, 6(1):97-98
DOI:10.4103/0974-2069.107248  PMID:23626450
The retroaortic course of left innominate vein is a rare entity which can be misinterpreted during echocardiography for other abnormal vascular structures under the arch of aorta. We report the case of a 2 month old infant where the suprasternal window showed 2 vascular structures beneath the aortic arch, one of which was traced to be a retroaortic innominate vein.
  5,419 250 1
Functional pulmonary atresia in newborn with normal intracardiac anatomy: Successful treatment with inhaled nitric oxide and pulmonary vasodilators
Gürkan Altun, Kadir Babaoglu, Köksal Binnetoglu, Nazan Kavas, Ayse Engin Arisoy
January-June 2013, 6(1):83-86
DOI:10.4103/0974-2069.107243  PMID:23626445
Functional pulmonary atresia is characterized by a structurally normal pulmonary valve that does not open during right ventricular ejection. It is usually associated with Ebstein's anomaly, Uhl's anomaly, neonatal Marfan syndrome and tricuspid valve dysplasia. However, functional pulmonary atresia is rarely reported in newborn with anatomically normal heart. We report a newborn with functional pulmonary atresia who had normal intracardiac anatomy, who responded to treatment with nitric oxide and other vasodilator therapy successfully.
  5,359 266 -
Idiopathic giant right atrial aneurysm
Santosh C Uppu, Ritu Sachdeva, Michiaki Imamura
January-June 2013, 6(1):68-70
DOI:10.4103/0974-2069.107238  PMID:23626440
A 2-year-old boy with an incidental finding of massive cardiomegaly on a chest X-ray was diagnosed with a giant right atrial aneurysm upon further investigation with echocardiography. The patient underwent successful surgical reduction of the right atrium and closure of the patent foramen ovale to prevent thromboembolic complications and to lower the risk of atrial arrhythmias. The resected atrium had paper-thin walls and pathological features of interstitial fibrosis with endocardial thickening.
  5,148 236 5
Conotruncal anomalies in the fetus: Referral patterns and pregnancy outcomes in a dedicated fetal cardiology unit in South India
Balu Vaidyanathan, Shine Kumar, Abish Sudhakar, Raman Krishna Kumar
January-June 2013, 6(1):15-20
DOI:10.4103/0974-2069.107227  PMID:23626429
Objective: To describe the referral patterns and pregnancy outcomes of fetuses with conotruncal anomalies (CTA) from a fetal cardiology unit in South India. Methods: Records of 68 women identified to have diagnosis of CTA on fetal echocardiography (mean gestational age 26.8 ± 5.9 weeks; range 17-38 weeks) during the period 2008-2011 were reviewed. Results: The most common indication for referral was suspected congenital heart disease during routine antenatal scan (89.7%). The various CTA diagnosed included Tetralogy of Fallot (TOF, 44.1%), Double outlet right ventricle (DORV, 27.9%), Transposition of great vessels (TGA, 8.8%), TOF with pulmonary atresia (TOF-PA, 8.8%), TOF absent pulmonary valve (TOF-APV, 7.4%) and truncus arteriosus (TA, 2.9%). Extra cardiac anomalies were reported in 4 fetuses (7.1%). Pregnancy outcomes included pregnancies not culminating in live-birth (54.4%), delivery at term (41.2%) with 3 patients (4.4%) being lost to follow-up. Proportion of pregnancies not culminating in live-birth lesion wise include: TOF (53.3%), DORV (52.6%), TGA (50%), TOF -APV (80%), TOF-PA (50%), and TA (50%).Twenty-four babies (35.3%) received post-natal cardiac care with 5 (7.4%) undergoing neonatal surgical procedures. Seven babies (10.3%) died in neonatal period, including 2 who underwent surgery. The accuracy of fetal echo was 96.4% for primary lesion and 67.9% for complete segmental diagnosis. Conclusions: Pre-natal diagnosis of CTA, despite a high diagnostic accuracy, prompted utilization of post-natal tertiary cardiac care in a limited proportion of patients, including those with reparable lesions. Focus in developing countries should shift towards earlier referral, improving awareness about treatment options and a comprehensive evaluation for associated anomalies.
  4,919 365 9
Stenosis of the branches of the neopulmonary artery after the arterial switch operation: A cardiac magnetic resonance imaging study
Boban Thomas, José Diogo Ferreira Martins, Nuno Jalles Tavares, Artur Lopes, Fátima F Pinto, José Fragata
January-June 2013, 6(1):29-33
DOI:10.4103/0974-2069.107230  PMID:23626432
Background: The neonatal arterial switch operation (ASO) is now the standard of care for children born with transposition of the great arteries. Stenosis of the neopulmonary artery on long-term follow up is a known complication. Methods: We performed a retrospective analysis of eleven patients who underwent a cardiac magnetic resonance imaging (MRI) due to echocardiographic evidence suggestive of stenosis of the neopulmonary artery or its branches (mean estimated Doppler gradient 48 mmHg, min 30 mmHg, max 70 mmHg). A comprehensive evaluation of anatomy and perfusion was done by cardiac MRI. Results: The branches of the neopulmonary artery (neo PA) showed decreased caliber in three patients unilaterally and in two patients, bilaterally. Magnetic resonance (MR) perfusion studies showed concomitant decreased flow, with discrepancy between the two lungs of 35/65% or worse, only in the three patients with unilateral obstruction, by two different MR perfusion methods. Conclusions: Cardiac MR can be used as a comprehensive non-invasive imaging technique to diagnose stenosis of the branches of the neopulmonary after the ASO, allowing evaluation of anatomy and function of the neoPA, its branches, and the differential perfusion to each lung, thus facilitating clinical decision making.
  4,563 241 2
Asymptomatic right ventricular dysfunction in surgically repaired adult tetralogy of fallot patients
Krishna Kumar Mohanan Nair, Sanjay Ganapathi, Bijulal Sasidharan, Anees Thajudeen, Harikrishnan Sivadasan Pillai, Jaganmohan Tharakan, Thomas Titus, Ajitkumar Valaparambil Kumaran, Sivasankaran Sivasubramonian, Kavassery Mahadevan Krishnamoorthy
January-June 2013, 6(1):24-28
DOI:10.4103/0974-2069.107229  PMID:23626431
Background: Right ventricular (RV) dysfunction after surgical repair of Tetralogy of Fallot (TOF) is often asymptomatic and may be detected by tissue Doppler imaging (TDI). The severity of RV dysfunction is more after intracardiac repair with transannular patch (TAP). Methods: One hundred seventy-three adult patients who have undergone surgical repair for TOF were prospectively analyzed for RV function using 2D echocardiography and TDI. RV function was compared between patients who have undergone intracardiac repair with and without TAP. Results: In both the patient sub-groups, TDI derived myocardial performance index (MPI) and myocardial velocities were abnormal even when 2D echocardiography derived RV functional area change was normal. TDI derived MPI was significantly higher (0.5 ± 0.1 vs. 0.4 ± 0 P0 < 0.001) and Systolic tricuspid annular velocity (Sa) (9.2 ± 1.3 vs. 10.8 ± 1.6 P < 0.001) was significantly lower in the TAP group. Older age at surgery and severity of pulmonary regurgitation on follow-up were among the significant predictors of TDI derived MPI. Conclusions: Asymptomatic RV dysfunction in surgically repaired adult TOF atients can be detected by TDI. Extent of RV dysfunction was significantly greater with patients requiring TAP, in those operated at older age, and in patients with severe pulmonary regurgitation.
  4,252 456 2
One-stage total repair of anomalous origin of right pulmonary artery from aorta by the double-flap technique, followed by coarctation repair using extended end-to-end arch reconstruction
Yu-Ning Hu, Yu-Jen Yang, Chung-Dann Kan
January-June 2013, 6(1):71-73
DOI:10.4103/0974-2069.107239  PMID:23626441
The anomalous origin of the right pulmonary artery from the ascending aorta combined with coarctation of aorta is a rare congenital malformation. The method chosen for performing a prompt surgery to correct the multiple disease lesions is important. Here we report one-stage surgical strategy which involved a double-flap technique alongside an extended end-to-end arch reconstruction in a newborn baby.
  4,472 231 -
Unusual association of aortic valve stenosis with ventricular septal defect and pulmonary atresia: Differentiation from truncus arteriosus with truncal valve stenosis
Saktheeswaran Mahesh Kumar, Sasidharan Bijulal, Sivasubramonian Sivasankaran
January-June 2013, 6(1):87-89
DOI:10.4103/0974-2069.107244  PMID:23626446
Stenosis of systemic semilunar valve in cyanotic congenital heart defects is rare. It can happen in truncus arteriosus with truncal valve stenosis and the very rare anomaly of tetralogy of fallot with aortic valve stenosis. Here we describe a neonate with pulmonary atresia, ventricular septal defect and associated aortic valve stenosis and discuss the points of differentiation from truncus arteriosus.
  4,481 220 -
Stage one Norwood procedure in an emerging economy:Initial experience in a single center
Rakhi Balachandran, Suresh G Nair, Sunil S Gopalraj, Balu Vaidyanathan, Brijesh P Kottayil, Raman Krishna Kumar
January-June 2013, 6(1):6-11
DOI:10.4103/0974-2069.107225  PMID:23626427
Objective: The evolution of surgical skills and advances in pediatric cardiac intensive care has resulted in Norwood procedure being increasingly performed in emerging economies. We reviewed the feasibility and logistics of performing stage one Norwood operation in a limited-resource environment based on a retrospective analysis of patients who underwent this procedure in our institution. Methods : Retrospective review of medical records of seven neonates who underwent Norwood procedure at our institute from October 2010 to August 2012. Results: The median age at surgery was 9 days (range 5-16 days). All cases were done under deep hypothermic cardiopulmonary bypass and selective antegrade cerebral perfusion. The median cardiopulmonary bypass (CPB) time was 240 min (range 193-439 min) and aortic cross-clamp time was 130 min (range 99-159 min). A modified Blalock-Taussig (BT) shunt was used to provide pulmonary blood flow in all cases. There were two deaths, one in the early postoperative period. The median duration of mechanical ventilation was 117 h (range 71-243 h) and the median intensive care unit (ICU) stay was 12 days (range 5-16 days). Median hospital stay was 30.5 days (range 10-36 days). Blood stream sepsis was reported in four patients. Two patients had preoperative sepsis. One patient required laparotomy for intestinal obstruction. Conclusions:Stage one Norwood is feasible in a limited-resource environment if supported by a dedicated postoperative intensive care and protocolized nursing management. Preoperative optimization and prevention of infections are major challenges in addition to preventing early circulatory collapse.
  4,296 314 4
Treating hypoplastic left heart syndrome in emerging economies: Heading the wrong way?
Krishna S Iyer
January-June 2013, 6(1):12-14
  3,915 259 1
Right superior vena cava draining in the left atrium associated with tetralogy of Fallot and pulmonary atresia
Mohammed A Al-Biltagi, Amjad Kouatli, Faris Al-Mousily
January-June 2013, 6(1):65-67
DOI:10.4103/0974-2069.107237  PMID:23626439
We report a case of an anomalous drainage of the right superior vena cava to the left atrium with intact atrial septum associated with Tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries.
  3,932 204 -
Pseudoaneurysm of mitral-aortic intervalvular fibrosa in a child: Demonstration by MDCT and MRI
Naseer A Choh, Feroze Shaheen, Hilal Rather, Khursheed Aslam
January-June 2013, 6(1):80-82
DOI:10.4103/0974-2069.107242  PMID:23626444
Subaortic left ventricular outflow tract pseudoaneurysms are rare lesions that are associated with aortic valve diseases, infective endocarditis, trauma or surgery. We present dynamic multidetector computerized tomography and cine magnetic resonance imaging features of a case of subaortic aneurysm arising from interaortic-mitral valvular region in a child with past history of infective endocarditis.
  3,915 209 4
Pre-excitation induced left ventricular dysfunction: A less known cause of cardiomyopathy in children
PR Bhima Shankar, Chidambaram Shanthi, Kottarathu Mammen Cherian
January-June 2013, 6(1):77-79
DOI:10.4103/0974-2069.107241  PMID:23626443
A 7-year-old boy was diagnosed to have dilated cardiomyopathy with severe left ventricular (LV) dysfunction at 1 year of age. Although well compensated, his LV function remained impaired over the follow-up years. His electrocardiogram (ECG) was initially misinterpreted as left bundle branch block. However, on follow-up, it was recognized as a pre-excited ECG. The accessory pathway causing pre-excitation was implicated as the cause of LV dysfunction and dysynchrony as the mechanism. Radiofrequency ablation was done. His LV function and dimensions improved significantly over 6 months follow up.
  3,440 205 3
Learning from Bristol - National database for congenital heart disease in India?
Thomas E Witter, Rhian R Brimmell, Shakeel A Qureshi
January-June 2013, 6(1):3-5
DOI:10.4103/0974-2069.107224  PMID:23626426
  3,389 235 -
Syncope in a child
Goutam Datta
January-June 2013, 6(1):93-94
DOI:10.4103/0974-2069.107246  PMID:23626448
Acute rheumatic fever (ARF) is a well-characterized illness. However, syncope in ARF due to advanced heart block is very rare. A 10-year-old boy was admitted with recurrent syncope for 12 h. The patient was diagnosed as ARF because of arthritis, elevated acute phase reactants, advanced heart block, high antistreptolysin O titer, and echocardiographic evidence of mitral regurgitation. On the 9 th day of hospitalization, the electrocardiogram revealed normal sinus rhythm.
  3,298 296 -
Rhythm disturbances in childhood obstructive sleep apnea during apnea-hypopnea episodes
Anant Khositseth, Jittamas Chokechuleekorn, Teeradej Kuptanon, Anchalee Leejakpai
January-June 2013, 6(1):39-42
DOI:10.4103/0974-2069.107232  PMID:23626434
Background: Obstructive sleep apnoea (OSA) can result in cardiovascular complications. Nocturnal arrhythmias are reported up to 50% of adult OSA patients. Arrhythmias and heart rate variability in children with OSA have not been well studied. Aims: We sought to study rhythm disturbances in childhood OSA and also to analyze the relationship of heart rate variability to the severity of OSA in children. Methods: In a retrospective cross sectional study, records of children aged < 15 years with history of snoring and suspected OSA, who had undergone polysomnography (PSG) for first time were analyzed. The cardiac rhythm and heart rate variability were studied during PSG. Results: A total of 124 patients diagnosed with OSA were grouped into mild ( n = 52), moderate ( n = 30), and severe ( n = 42) OSA. During PSG, all had sinus arrhythmias and only three patients had premature atrial contractions (PACs). The standard deviation of heart rate (SD-HR) during rapid eye movement (REM) sleep in severe OSA (9.1 ± 2.4) was significantly higher than SD-HR in mild OSA (7.5 ± 1.3, P < 0.0001). The maximum heart rate (max-HR) during REM-sleep in severe OSA (132.1 ± 22.1) was significantly higher than the max-HR in mild OSA (121.3 ± 12.6 bpm, P = 0.016). Conclusions: There was no significant arrhythmia in children with OSA during their sleep. Heart rate variability correlated with the severity of OSA.
  3,272 237 2
Auditing our 'selves'
Shyam S Kothari
January-June 2013, 6(1):1-2
DOI:10.4103/0974-2069.107223  PMID:23626425
  3,174 298 -
Staged pacemaker implantation in a preterm with hydrops fetalis due to complete heart block
Tanuja Karande, Swati Garekar, Snehal Kulkarni, Suresh Rao
January-June 2013, 6(1):99-100
DOI:10.4103/0974-2069.107249  PMID:23626451
  3,283 185 1
Persistent electrocardiographic ST segment elevation from previous myocarditis
Efrén Martinez-Quintana, Fayna Rodriguez-Gonzalez, Paula Junquera-Rionda
January-June 2013, 6(1):74-76
DOI:10.4103/0974-2069.107240  PMID:23626442
Various conditions may present with an electrocardiographic pattern of ST segment elevation simulating myocardial infarction. We present an asymptomatic 16-year-old male patient, whose ECG showed persistent anterolateral ST segment elevation and magnetic resonance imaging showed wall motion abnormalities from previous myocarditis but no evidence of inflammation.
  3,291 159 -
Does fetal echo help the fetus?
Snehal Kulkarni
January-June 2013, 6(1):21-23
  3,147 282 -
Intraoperative hybrid left pulmonary artery stenting
Budhaditya Chakraborty, Donald Hagler, Harold M Burkhart, Joseph A Dearani
January-June 2013, 6(1):43-45
DOI:10.4103/0974-2069.107233  PMID:23626435
  2,838 185 1
Selected abstracts presented at the Annual Conference of the Paediatric Cardiac Society of India 2012

January-June 2013, 6(1):105-108
  2,435 191 -
Selected Summaries
Bhava RJ Kannan
January-June 2013, 6(1):101-104
DOI:10.4103/0974-2069.107250  PMID:23626452
  2,204 156 -