Introduction: Pulmonary atresia with the ventricular septal defect is a rare congenital heart defect with high anatomic variability. The most important management question relates to the sources of pulmonary blood flow. The ability to differentiate between ductal dependence and major aortopulmonary collateral arteries is critical to achieving good outcomes and avoiding life-threatening hypoxia in the postneonatal period. Having accurate information about pulmonary arteries, major aortopulmonary collateral arteries, and sources of blood supply to each pulmonary segment is crucial for choosing the optimal surgical strategy. The purpose of this study is to compare computed tomography angiography (CTA) with cardiac catheterization for anatomic delineation of surgically relevant anatomy in pulmonary atresia with ventricular septal defect with major aortopulmonary collateral arteries. Materials and Methods: Retrospective review of all children with pulmonary atresia with ventricular septal defect with major aortopulmonary collateral arteries cared for at a large tertiary children's hospital who underwent cardiac catheterization with angiography and CTA close to each other without interval therapy. All studies were performed between 2007 and 2011. Results: There were 9 patients who met the inclusion criteria. Pulmonary artery anatomy (confluent vs. nonconfluent) was correctly identified in 9 patients by CTA and 8 patients by catheterization. There were no significant differences between CTA and catheterization in the identification of major aortopulmonary collateral arteries (mean = 3.4 collaterals/study via catheterization; mean = 3.1 collaterals/study via CTA; P = 0.67). CTA was superior to catheterization in the delineation of segmental pulmonary blood flow (P = 0.006). Conclusion: CTA and catheterization are equivalent in their ability to delineate pulmonary artery anatomy and major aortopulmonary collateral arteries.

Background and Objectives: The main effect of pulmonary stenosis is a rise in right ventricular pressure. This pressure overload leads to multiple changes in the shape, dimensions, and volumes of the right ventricle (RV) that are reversed after the relieve of the valve obstruction. We thought to study the changes in the RV in patients undergoing balloon pulmonary valvuloplasty (BPV) using three-dimensional (3D) echocardiography. Subjects and Methods: The study included 50 patients with isolated valvular pulmonary stenosis who underwent BPV at our hospital from December 2016 to August 2017; echocardiography was recorded preprocedural and 3 months after the procedural. Results: The median age of the study group at the time of the procedure was 2.7 years. The indexed RV wall thickness, basal, and mid-right ventricular dimensions decreased significantly after the procedure (P < 0.005), and the longitudinal dimension increased significantly after the procedure (P < 0.005). The end-systolic and the end-diastolic volumes (EDVs) by 3D echocardiography increased insignificantly (P > 0.05), and the right ventricular function increased significantly (P < 0.05), indicating that the changes in the EDVs were more than the changes in the end-systolic volumes. Conclusions: There are several factors that interplay together and result in reverse remodeling of the RV after BPV including regression in the RV hypertrophy; changes in the interventricular septal morphology, bowing, and mobility; and changes in the ventricular geometry and dimensions, rather than changes in the ventricular volumes.

Introduction: In this prospective study, we compared the efficacy and safety of ibuprofen, indomethacin, and paracetamol in the closure of patent ductus arteriosus (PDA) in preterm neonates. Materials and Methods: This randomized prospective study was conducted in the Division of Pediatric Cardiology, M. D. M and Umaid Hospital, Jodhpur. A total of 105 preterm neonates with gestational age <37 weeks and hemodynamically significant PDA (hs-PDA) diagnosed clinically and confirmed by echocardiography were enrolled. All neonates were randomly assigned in a ratio of 1:1:1 to oral indomethacin (Group A, 3 doses at an interval of 12 h with a starting dose of 0.2 mg/kg), oral ibuprofen (Group B, 10 mg/kg ibuprofen followed by 5 mg/kg/day for 2 days), or IV paracetamol (Group C, 15 mg/kg every 6 hourly for 3 consecutive days). After the completion of the first course, neonates were assessed clinically as well as by echocardiography to confirm PDA closure. If PDA remained open, the second course of the same drug was given and repeat assessment was done within 24 h of the last dose. In addition to an echocardiographic examination, complete blood counts, renal and liver function tests were performed. Results: Our study shows that there was no significant difference observed in PDA closure among all the three treatment groups after the completion of two courses of treatment. The cumulative rate of PDA closure was 68% in the indomethacin group, 77.14% in the ibuprofen group, and 71.43% in the paracetamol group (P = 0.716). There were no significant changes found in Hb, platelet, blood urea nitrogen (BUN), creatinine, and liver enzymes after treatment in the paracetamol group (P > 0.05). BUN and serum creatinine levels were significantly increased after treatment in indomethacin and ibuprofen groups (P < 0.0001 and P < 0.05, respectively). Conclusion: Our study shows that IV paracetamol is as effective as indomethacin and ibuprofen in promoting the closure of hs-PDA in premature infants with a better safety profile.

Isolated absent pulmonary valve syndrome with an intact ventricular septum (APVS/IVS) is an extremely rare lesion. The prenatal diagnosis was described in a few reports. In the current work, we report a unique fetal case with this rare anomaly. The family counseling for isolated APVS/IVS is a different challenging process than with fetal Fallot type diagnosis. Moreover, 22q11 deletion has never been found as an association. Parents must also be aware of the expected early heart failure in cases with large patent ductus arteriosus and the need for its closure as early as possible.

A 5-year-old boy with the history of intractable seizure for the past 2 years was transferred to the emergency room for cardiopulmonary resuscitation because of the prolonged seizure and profound cyanosis. He was intubated and resuscitated by cardioversion for a bizarre shape ventricular tachycardia (VT). After noxious stimulation, he showed multiple polymorphic ventricular premature beats that were followed by a bidirectional VT in favor of catecholaminergic polymorphic VT. The genetic assessment was positive for CASQ2 mutation. In the follow–up, the arrhythmia was controlled by nadolol, however with a prominent neurological sequela.

A broom straw was ingested and penetrated the esophageal wall, the pericardial space and its tip became lodged in the coronary sinus. Bacterial pericarditis and then fungal endocarditis ensued but were temporally separated by an asymptomatic 6-month period. On transthoracic echocardiography, the straw was mistakenly identified to be a “prominent Thebesian valve.” This child survived both life-threatening infections. The occurrence of infections caused by unusual organisms in the setting of immunocompetence highlights the need for a high index of suspicion for the presence of a causative foreign body.

Neonatal ventricular tachycardia (VT) is an extremely rare condition. We present a 35-week-old gestation neonate who developed tachycardia following maternal exposure to terbutaline. Upon transfer to our neonatal intensive care unit, an electrocardiogram (ECG) was obtained which was consistent with VT. The arrhythmia did not respond to vagal maneuvers or adenosine but resolved following cardioversion demonstrated on postcoversion ECG. At outpatient follow-up, the infant had no further episodes of arrhythmia. To the best of our knowledge, this represents the first case describing terbutaline-induced fetal or neonatal VT.

The pediatric cardiologist now has an important role to play in fetal medicine. They are often called upon to manage fetal cardiac problems such as arrhythmias or perform fetal cardiac interventions such as balloon valvuloplasty or atrial septostomy. In these scenarios, it becomes very important for the pediatric cardiologist to understand the concepts of “fetus as a patient,” “viability,” etc., and their implications in management. We try to shed light on these principles through our case scenario of managing supraventricular tachycardia in one of the fetuses of a twin pregnancy.

We report an eight month old infant who presented with incessant Fascicular tachycardia and heart failure which was refractory to drugs and cardioversion. Sinus rhythm was restored by radio frequency ablation and this resulted in improvement in clinical status and cardiac function. Role of catheter ablation in this situation is discussed

Embryologically, the common arterial trunk (CAT) is due to the failure of septation by the major outflow cushions. This invariably leads to the presence of an associated large juxta-arterial ventricular septal defect. Overriding of the ventricular septum leads to its biventricular origin in more than 2/3^rd of cases. Uncommonly, CAT may be associated with an intact ventricular septum (IVS) and univentricular origin. We describe an infant with CAT, intact IVS, hypoplastic right ventricle, and Ebstein's anomaly of the tricuspid valve who presented to us and palliated successfully. The embryological mechanisms are discussed in this report.

Lymphangiomas are relatively uncommon lesions of the lymphatic channels which can arise in virtually any part of the body. Although the most common site is the head/neck region, they could be found in the mediastinum. If mediastinal lymphangiomas are said to be rare, the thymic subentity is even scanty. We describe one case of mediastinal lymphangioma with a true intrathymic localization, which to our knowledge has been reported just once in the literature. This case report elucidates the surgical management of the first lymphangioma reported in an infant.

Pulmonary artery (PA) discontinuity with ductal origin of a major pulmonary branch is a rare congenital anomaly that can be diagnosed as an isolated lesion or in association with major cardiac malformations. Arterial duct (AD) closure results in complete disappearance of the dependent PA, thus leading to the misdiagnosis of “congenital PA absence.” Neonatal AD transcatheter recanalization is considered a cost-effective approach in view of later, lower-risk surgical recruitment of the disconnected PA. However, repeat percutaneous recanalizations of a completely occluded PA, the first one as native duct-dependent lesion and the second one several months after its surgical reimplantation, have so far never been reported in the literature. This paper reports on a neonate who serendipitously received at a few weeks of age the diagnosis of “congenital” absence of the right PA. She was successfully submitted to transcatheter AD recanalization and then surgical recruitment of the dependent PA about 8 months later. However, complete occlusion of the reconnected PA was diagnosed some few months after the surgical repair. This vessel was once again recruited by percutaneous approach and it is still patent and in catch-up growth after 6 months from the second recanalization procedure.

We report a case of right isomerism with a complex congenital heart disease associated with hiatus hernia in a 19-week-old fetus with relevant review of literature. This report highlights the importance of having a proper checklist for prenatal identification of extracardiac manifestations of isomerism syndromes. This will enable us to provide an effective family-centered counseling for perinatal management of these complex lesions. To our knowledge, prenatal sonographic detection of a sliding hiatal hernia in a fetus with right isomerism has not been reported previously.

Diffuse and multiple coronary cameral fistulas are very rare and with very few case reports of its association with left ventricular noncompaction are published. Here, we report a 6-year-old child of multiple diffuse coronary cameral fistulas to both the right and left ventricle in association with the left ventricular noncompaction. A possible common embryological link between the two uncommon entities is also discussed.

Double orifice mitral valve (DOMV) is a rare congenital anomaly of the mitral valve apparatus consisting of an accessory bridge of fibrous tissue, which partially or completely divides the mitral valve into two orifices. It usually occurs as an associated lesion. Encountering a DOMV as an isolated finding meriting intervention is indeed rare. We, here, report a case of “complete bridging type DOMV” occurring as an isolated entity and presenting as severe congenital MR requiring surgery.

Isolation of the right subclavian artery (RSCA), defined as loss of continuity between the RSCA and aorta, is a rare anomaly and its association with dextrotransposition of great arteries (D-TGA) is exceedingly unusual. We present the case of a 5-day-old neonate with cyanosis from birth and a transthoracic echocardiographic diagnosis of D-TGA, where on computed tomography angiography, the RSCA was seen arising from the proximal part of the ipsilateral pulmonary artery via a patent right ductus arteriosus.