Congenital heart disease (CHD) is one of the major debilitating birth defects resulting in significant impact on neonatal and child mortality globally. The etiology of CHD is complex and multifactorial. Many causative genes responsible for CHDs have been identified from the familial forms previously. Still, the non-Mendelian inheritance and predominant sporadic cases have stimulated research to understand the epigenetic basis and environmental impact on the incidence of CHD. The fetal epigenetic programming affecting cardiac development is susceptible to the availability of key dietary factors during the crucial periconceptional period. This article highlights the need and importance of in-depth research in the new emerging area of maternal nutritional epigenetics and CHD. It summarizes the current research and underlines the limitations in these types of studies. This review will benefit the future research on nutrition as a modifiable environmental factor to decrease the incidence of CHD.

Fontan connection with intermittent compression by wrapped latissimus dorsi (LD) was tested in vivo, in vitro and by means of computational fluid dynamics (CFD). Experimental study: LD was conditioned in four pigs for three weeks before Fontan connection by valved conduit wrapped with LD. Mock circuit: Inflatable cuff wrapped around valved conduit provided intermittent external compression, with pressure and flow measured at driving pressure of 8 or 16 mmHg. CFD study: A circuit was tested for possible increase above basal flow (4 l/min) with intermittent external compression. Experimental study: Intermittent conduit compression by LD provided mean 7% decrease of baseline PA pressure, with simultaneous flow increase of 2%. Mock circuit: By raising the driving pressure from 8 to 16 mmHg, the flow increased with baseline PVR (56%) and with elevated PVR (80%). Total pulmonary flow was reduced during intermittent external compression with both baseline and elevated PVR. CFD study: Compression with 13.0 mmHg provided 4.9% increase of total pulmonary flow with substantial increase of the peak flow (92%). In vivo and in vitro, the increased flow produced by compressing a conduit was confounded by the inevitable intermittent flow restriction. Mathematical model using lower pressure for intermittent external compression showed potential for increase in pulmonary flow.

Aneurysms of descending thoracic aorta following surgical repair of coarctation have been reported in literature. Almost always, they are seen in repairs involving prosthetic patch aortoplasty. We report a neonate who underwent resection and an extended end to end anastomosis repair of coarctation and subsequently developed a huge pseudoaneurysm at a 3-month follow-up. He underwent a repair of the same through a sternotomy approach under hypothermic low flow cardiopulmonary bypass. An autologous pericardial patch aortoplasty was done successfully.

Aortopulmonary window (APW) is a relatively rare cardiac lesion representing approximately 0.2%–0.4% of all cardiac malformation. It is a cardiac abnormality that results from abnormal communication between the proximal aorta and the main pulmonary artery in the presence of two normally separated aortic and pulmonary valves. In the past, the diagnostic and surgical approach to APW was almost always preceded by cardiac catheterization. With recent advances in noninvasive approach and techniques of two-dimensional echocardiography diagnosis of the defect and associated anomalies are facilitated without a cath study. We report a 4-month-old infant with a distal APW who was referred to our center for surgical repair. We emphasize the usage of transesophageal echocardiography as a valuable intraoperative tool which not only confirms the preoperative diagnosis but also helps in assessing the surgical repair of an APW.

Double-chambered right ventricle (DCRV) is a developmental cardiac anomaly in which anomalous muscle bundles divide the right ventricular (RV) cavity into two chambers. It is usually associated with other congenital cardiac defects, of which ventricular septal defect is the most common association. Isolated DCRV with an intact interventricular septum is very rarely reported. It manifests itself usually in adolescence and adults as the obstruction progresses gradually. It is important to recognize this anomaly as it can progress to severe RV failure if unaddressed. We report a 13-year-old male who presented to us with an isolated DCRV. He successfully underwent a resection of the RV cavity anomalous muscle bundles. We also stress on the utility of transesophageal echocardiography intraoperatively which accurately delineates the location of the muscle bundles and helps in evaluating the adequacy of resection postsurgery.

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Background: Congenial heart defects (CHDs) have multifactorial etiology with complex interplay of genetic and environmental factors. Environmental impact can have epigenetic mechanism of CHD development. Many studies have reported the causal association between CHD and distinct DNA methylation profile which is one of the key epigenetic events, which has vital role in normal embryonic development. The products of DNMT1, DNMT3A, DNMT3B, and MBD2 are important regulators of DNA methylation process.Changes in the expression of these genes are implicated in congenital structural cardiac defects. Hence, in this proof-of-concept study, we have compared the expression levels of these genes in the blood samples of healthy controls and CHD cases while investigating the etiology of CHD. Methods: In this study with 48 CHD cases and 47 healthy controls, total RNA was isolated from the whole blood samples using TRI reagent. Quantitative RT PCR (qRT-PCR) was used to analyze the mRNA levels of DNMT1, DNMT3A, DNMT3B, and MBD2. The expression levels have been analyzed by relative quantification. Results: We observed that DNMT3B (fold change = À2.563; p = .0018) and DNMT3A (fold change = À2.169; p = .05) were significantly downregulated in CHD patients, whereas the expression of DNMT1 and MBD2 was not significantly different between cases and controls.Conclusions: Lower expression of de novo methyltransferases, namely, DNMT3B and DNMT3A in CHD cases, may be an important contributor to the mechanism of CHD pathogenesis. Further studies with age-matched controls and analysis of global DNA methylation profile are required to investigate the proposed causal association.

Tetralogy of Fallot is a cyanotic heart disease wherein aortopulmonary collaterals serve as source of pulmonary blood flow to maintain oxygenation. We report an incidentally detected isolated left subclavian artery supplying a compensatory ductus in a child with Tetralogy of Fallot that effectively contributed as a de novo palliative systemic to pulmonary artery shunt. Clinically, the entity could not be suspected, as the child did not have symptoms suggestive of arterial insufficiency of the left arm or weak pulses or neurological symptoms. The child underwent successful intracardiac repair with a reimplantation of left subclavian artery to left common carotid artery.