Background: We investigated the treated prevalence of bicuspid aortic valve in a pediatric population with congenital heart disease and its incident complications. Materials and Methods: A 15-year retrospective data set was analyzed. Selection criteria included age ≤17 years, enrollees in the South Carolina State Medicaid program and diagnosed as having bicuspid aortic valve on one or more service visits. Results: The 15-year-treated prevalence of predominantly isolated bicuspid aortic valve was 2% (20/1000) of pediatric congenital heart disease cases, with a non-African American: African-American ratio of 3.5:1, and a male:female ratio of 1.6:1. Aortic stenosis (28.0%), ventricular septal defect (20.6%), and coarctation of the aorta (20.6%) were the most prevalent coexisting congenital heart lesions. Of the 378 bicuspid aortic valve cases examined, 10.3% received aortic valve repair/replacement, which was significantly more likely to be performed in children with diagnosed aortic stenosis (adjusted odds ratio = 12.90; 95% confidence interval = 5.66–29.44). Cohort outcomes over the study period indicated that 9.5% had diagnosed heart failure, but <1% had diagnosed supraventricular tachycardia, infective endocarditis, aneurysm, dissection, or death. Conclusions: The majority of isolated bicuspid aortic valve cases without aortic stenosis did not require surgical intervention. Outcomes for cases requiring repair/replacement were relatively benign.

Background: Kawasaki disease (KD) is an acute systemic vasculitis syndrome with a high incidence of coronary aneurysms in untreated children. The majority of aneurysms resulting from KD are known to regress with time. Aims: This study aimed to determine the course and outcome of coronary artery dilatation in patients with KD and ascertain whether there are any differences in the outcomes in the different branches. Setting and Design: This is a retrospective cohort study of patients diagnosed with KD with midterm follow-up data. Methods: Serial echocardiography was performed in all KD patients with coronary dilatation for 1–10½ years. The Kaplan–Meier curve was used for statistical analysis. Results: There were 154 patients with coronary dilatation studied. The frequency of coronary dilatation in acute phase was 33.3% and decreased to 7.9% 6–8 weeks later. Each patient could have dilatations at more than one branch, so the total number of dilatations was 245. The median time needed for regression was 2.6 months (mean: 10.5 months) while the median of follow-up duration was 41 months (mean: 23 months). Small- and medium-sized dilatations had more favorable outcomes compared to the giant ones. Location of dilatation did not influence the outcome. Conclusions: The majority (77.4%) of small- and medium-sized dilatations regress within 2 years, but giant aneurysms tend to persist. The outcome of coronary dilatation is determined by the diameter and not by the location. Regression rate is faster in smaller dilatations. Left main coronary artery is the most frequent location for dilatation.

Introduction: Although simulation training has been utilized quite extensively in highincome medical environments, its feasibility and effect on team performance in lowresource pediatric Cardiac Intensive Care Unit (CICU) environments has not been demonstrated. We hypothesized that lowfidelity simulationbased crisis resource management training would lead to improvements in team performance in such settings. Methods: In this prospective observational study, the effect of simulation on team dynamics and performance was assessed in 23 healthcare providers in a pediatric CICU in Southeast Asia. A 5day training program was utilized consisting of various didactic sessions and simulation training exercises. Improvements in team dynamics were assessed using participant questionnaires, expert evaluations, and video analysis of time to intervention and frequency of closedloop communication. Results: In subjective questionnaires, participants noted significant (P < 0.05) improvement in team dynamics and performance over the training period. Video analysis revealed a decrease in time to intervention and significant (P < 0.05) increase in frequency of closedloop communication because of simulation training. Conclusions: This study demonstrates the feasibility and effectiveness of simulationbased training in improving team dynamics and performance in lowresource pediatric CICU environments, indicating its potential role in eliminating communication barriers in these settings.

Background: Sepsis is one of the major contributor of morbidity and mortality in pediatric cardiac surgeries. Aim: The aim of this study was to compare the predictive efficacy of total leukocyte counts (TC), platelet count (PC), and procalcitonin (PCT) for sepsis in patients undergoing cardiac surgeries who are admitted to the Intensive Care Unit. Materials and Methods: This prospective, single-center study included 300 neonates, infants, and pediatric patients who had undergone various open heart surgeries at our center from September 2014 to November 2015. Results: Overall, the incidence of sepsis was 14% in pediatric patients undergoing cardiac surgeries. TC of postoperative 48 h were significantly lower (11889.19 ± 5092.86 vs. 14583.22 ± 6562.96; P = 0.004) in septic patients. The low levels of platelets on postoperative 24 h and 72 h were observed in patients with sepsis as compared to patients without sepsis, whereas the levels of PCT at various time intervals (preoperative, postoperative - 24 h, 48 h, and 72 h) had shown no association with sepsis in the study population. Low PC (24 h) was the strongest predictor of sepsis showing an odds ratio of 1.9 (95% confidence interval [CI]: 1.42–3.51; P = 0.001) and area under curve of 0.688 with 95% CI of 0.54–0.83 (P = 0.018). Conclusion: We may conclude that in Indian pediatric population platelet levels are highly associated with sepsis as compared to any other hematological parameter. The immediate postoperative level of platelet is the strongest predictor of sepsis and could be effectively used in the clinical settings.

Background: Congenital aortic stenosis (AS) is a rare disease. Treatment options for newborns are challenging. Newborns may have higher reintervention rate and mortality. Objectives: The study aimed to identify the factors predictive of reintervention following balloon aortic valvuloplasty (BAV) for AS during infancy. Methods: Retrospectively, between 2001 and 2016, echocardiography (echo) and cardiac catheterization data for infants with AS were analyzed, including follow-ups and reinterventions. Percentage reduction was defined as the ratio between the drop of aortic valve (AV) peak gradient and the baseline peak gradient. Results: Sixty infants were included and 48 were followed up. Sixteen (27%) patients were neonates. Peak-to-peak gradient at AV was 64 ± 27 mmHg, which was reduced to 27 ± 13 mmHg. Percentage reduction was 53% ±24%. Forty-nine (82%) patients had adequate results (residual AV gradient <35 mmHg). There was no significant aortic insufficiency (AI) before procedure, while 6 (10%) patients had increased AI immediately after BAV. Of 48 patients, 14 (29%) required an additional BAV. Of 48 patients, 8 (17%) required surgical interventions following BAV. Reintervention was associated more with small left ventricular outflow tract (LVOT), high residual AV, and low percentage reduction. Mortality was 8.3%. Conclusions: BAV in infancy has a reasonable success rate (82%) with high rate of reintervention. Patent ductus arteriosus-dependent neonates carried the highest risk of mortality. Small LVOT, high AV residual gradient, and low percentage reduction resulted in more reinterventions.

This article reviews important features for improving the diagnosis of fetal arrhythmias by ultrasound in prenatal cardiac screening and echocardiography. Transient fetal arrhythmias are more common than persistent fetal arrhythmias. However, persistent severe bradycardia and sustained tachycardia may cause fetal hydrops, preterm delivery, and higher perinatal morbidity and mortality. Hence, the diagnosis of these arrhythmias during the routine obstetric ultrasound, before the progression to hydrops, is crucial and represents a challenge that involves a team of specialists and subspecialists on fetal ultrasonography. The images in this review highlight normal cardiac rhythms as well as pathologic cases consistent with premature atrial and ventricular contractions, heart block, supraventricular tachycardia (VT), atrial flutter, and VT. In this review, the details of a variety of arrhythmias in fetuses were provided by M-mode and Doppler ultrasound/echocardiography with high-quality imaging, enhancing diagnostic accuracy. Moreover, an update on the intrauterine management and treatment of many arrhythmias is provided, focusing on improving outcomes to enable planned delivery and perinatal management.

The rapid changes that have taken place in recent years in relation to techniques used to image the fetal heart have emphasized the need to have a detailed knowledge ofnormal cardiac anatomy. Without such knowledge, it is difficult, if not impossible, to recognize the multiple facets of congenital cardiac disease. From the inception of fetal echocardiographic screening, the importance of basic knowledge of cardiac anatomy has been well recognized. The current machines used for imaging, however, now make it possible potentially to recognize features not appreciated at the start of the specialty. So as to match the advances made in imaging, we have now revisited our understanding of normal cardiac anatomy in the mid-gestational fetus. This was made possible by our dissection of 10 fetal hearts, followed by production of addition histological sections that mimic the standard ultrasound views. The fetuses ranged in gestational age from between 20 and 28 weeks. We then correlated the obtained anatomic images with the corresponding ultrasonic images used in the standard fetal screening scan. We also interrogated the anatomic sections so as to clarify ongoing controversies regarding detailed features of the normal cardiac anatomy. We have been able to show that the views now obtained using current technology reveal many details of anatomy not always appreciated at earlier times. Knowledge of these features should now permit diagnosis of most congenital cardiac malformations. The anatomic-echocardiographic correlations additionally provide a valuable resource for both the understanding and teaching of fetal echocardiography.

Professor IM Rao, from All India Institute of Medical Sciences, New Delhi, was a pioneering cardiac surgeon who, against overwhelming odds, developed congenital heart surgery in India. He made many contributions to the evolution of his specialty, spanning more than four decades and three countries. This is a brief report of his professional life and accomplishments.

Systemic-to-pulmonary artery collateral networks commonly develop in patients with single-ventricle physiology and chronic hypoxemia. Although these networks augment pulmonary blood flow, much of the flow is ineffective and contributes to cardiac volume loading. This volume loading can have detrimental effects, especially for single-ventricle patients. Some data suggest that occluding collaterals may improve outcomes after subsequent operations, especially when the volume of collateral flow is significant. Traditional practice has been to coil occlude the feeding vessel. We perform particle embolization of these collateral networks for two primary reasons. First, access to the feeding vessel is not blocked as collaterals may redevelop. Second, particles occlude the most distal connections. Thus, embolization with particles should be considered as an alternative to coil occluding the proximal feeding vessel.

The need for transvenous pacing (patients who have exhausted epicardial options) after a Fontan-type operation has been recognized. Many novel strategies have been proposed, but currently, all of them require additional maneuvers or rerouting of the leads to the pacemaker pocket. In this report, we describe a novel direct approach to transvenous pacing after a Fontan-type operation from a standard, prepectoral approach.

We report a rare and serious complication of cardiac strangulation arising from the implantation of epicardial pacing leads in a newborn. Patient's follow-up 9-month postsurgery revealed compression under the pulmonary valve annulus by a pacemaker lead, causing progressive stenosis of the right ventricular outflow tract. The epicardial leads were replaced to relieve compression, and stenosis of the right ventricular outflow tract was rectified. Pacemaker implantation in newborns is not without challenges; epicardial leads should be carefully positioned to avoid any compression of cardiac structures.

We report a term female infant with congenital heart block and total anomalous of pulmonary venous return. The results of single nucleotide polymorphism oligonucleotide microarray analysis showed an interstitial duplication of approximately 818 Kb, which involved 11 genes, including the entire LAMB3 gene which is known to associate with cardiac conduction defect. Our report adds to the collective knowledge that the cardiac conduction defect is a clinical feature of chromosome 1q32.2 duplication.

Paragangliomas are rare neuroendocrine tumors where hypoxia-inducible factor plays a critical role in tumorigenesis. It has been suggested that patients with congenital heart disease, in particular, may have cellular environment and relative hypoxia favorable to the development of these neuroendocrine tumors. Here, we present a case of an 11-year-old child with hypoplastic left heart syndrome previously palliated with Fontan procedure, diagnosed with paraganglioma on surveillance imaging. We present the clinical course, intervention, and outcome as well as review the possible contributory mechanisms. As we continue to improve long-term survival for single ventricle patients, awareness of these tumors during surveillance may be warranted as timely intervention may lead to cure.

Better anatomical understanding and conceptualization of complex congenital heart defects using three-dimensional (3D) printing may improve surgical planning, especially in rare defects. In this report, we utilized 3D printing to delineate the exact cardiac anatomy of a neonate with an aortopulmonary window associated with interrupted aortic arch to devise a novel approach to the repair.

The clinical detail, echocardiographic features, angiographic evaluation, and surgical repair of double-outlet left atrium (LA) in a 2-year-old boy are discussed. In a solitus position and normally connected systemic and pulmonary venous drainage setting, the only outlet of the right atrium was a secundum atrial septal defect. The LA thus received the entire venous return and then drained into both ventricles through a common atrioventricular valve.

Endomyocardial fibrosis (EMF) is an important cause of restrictive cardiomyopathy in tropical countries. The etiopathogenesis of EMF remains obscure. The role of eosinophilia in the etiopathogenesis of EMF has been debated extensively, but remains unproven. Accordingly, we present a case wherein a patient with documented eosinophilia and heart failure at the age of three-and-a-half years presented with endomyocardial fibrosis at the age of nine years. Such documentation is important to highlight the central role of eosinophils in the pathogenesis of EMF.

A case of the right pulmonary artery-to- left atrial fistula with atrial septal defect (ASD) is presented. The fistula was detected after the patient developed desaturation following surgical closure of the ASD. It was managed with a transcatheter (trans-RPA route) closure of the fistula using a 12-mm Amplatzer ventricular septal defect closure device.

Homocystinuria is a genetic inborn error of metabolism due to the deficiency of cystathionine β-synthase resulting in increased serum homocysteine and methionine and decreased cysteine which predisposes affected individuals to arterial and venous thromboembolic phenomena. We present a case of homocystinuria who presented to us as a calcified right atrial mass during the evaluation for lower respiratory tract infection. Our case reveals an unusual mix of findings using imaging with multiple detector computed tomography and radiographs.

A 9-year-old boy with hypereosinophilic syndrome (HES) was referred for cardiac magnetic resonance (CMR) imaging following an abnormal echocardiogram that showed a large mass layered on the inferolateral wall of the left ventricle, causing secondary severe mitral regurgitation. Cardiac involvement in HES usually affects the ventricular apex. In our case, CMR confirmed the presence of a large mural thrombus of 0.9 cm × 4.2 cm. This unusual cardiac involvement in HES was diagnosed in its intermediate thrombotic stage. CMR is very sensitive and specific in staging the disease. It explained the etiology of mitral regurgitation and guided therapy, especially when echocardiography was nonconclusive.

Long QT syndrome (LQTS) is a congenital disorder characterized by prolongation of QT interval in the electrocardiogram (ECG) and a propensity to develop ventricular arrhythmias, which may lead to syncope, cardiac arrest or sudden death. T-wave alternans (TWA), a phenomenon of beat-to-beat variability in the repolarization phase of the ventricles, has been closely associated with an increased risk of ventricular tachyarrhythmic events (VTE) and sudden cardiac death (SCD).

Congenital diveticula and aneurysm of the heart are rare and most often located at the apex of the left ventricle. They pose a significant risk for cardiac failure and arrhythmias. In contrast, nonapical diverticula of the right ventricle (RV) have a much more benign course. We present a child with Trisomy 21, atrioventricular septal defect, and large nonapical diverticulum of the RV that was neither addressed during surgery nor needed any medical treatment during 1-year follow-up.