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.