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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.