World Journal for Pediatric and Congenital Heart Surgery

As recovery of congenital heart surgery programs begins during this COVID-19 pandemic, we review key considerations such as screening, protection of patients and health care workers (HCWs), case prioritization, barriers to reactivation, redesign of patient care teams, contribution of telemedicine, modification of trainees’ experiences, preparation for potential resurgence, and strategies to maintain HCW wellness. COVID-19 has tested the resolve and grit of our specialty and we have an opportunity to emerge more refined.

Left ventricular outflow tract (LVOT) obstruction is a component of many forms of congenital heart disease, including hypertrophic cardiomyopathy, membranous subaortic stenosis, tunnel subaortic stenosis, and outflow tract obstruction related to atrioventricular septal defects. We have gained a particularly extensive experience with the diagnosis and treatment of hypertrophic cardiomyopathy, having performed septal myectomy in over 3,800 patients. In the setting of this review of LVOT obstruction, we use hypertrophic cardiomyopathy as a template by which other pathologies causing LVOT obstruction can be understood. We review important surgical issues in patient selection, diagnostic evaluation, interpretation of imaging, and operative management. To this end, the review focuses on obstructive hypertrophic cardiomyopathy and then broadens to discuss other pathologies causing LVOT obstruction, with important similarities and differences in their management. These other pathologies share some similar presentations and operative techniques, and at times can be confused with hypertrophic cardiomyopathy, but also have important distinctions of which the surgeon should be aware.

Monocusp valves are thought to reduce early operative mortality and morbidity associated with pediatric tetralogy of Fallot repair. As there are no published randomized clinical trials comparing outcomes with and without a monocusp valve, we performed a meta-analysis of observational studies in accordance with established protocols. After systematically searching PubMed, the Cochrane Library, and Google Scholar, 12 studies were included. The operative mortality was compared in 695 patients, and we found no difference between patients with and patients without a monocusp valve. Monocusp valves may not improve operative mortality of tetralogy of Fallot repair in pediatric patients.

We present the case of an nine-year-old girl with double outlet right ventricle with noncommitted ventricular septal defect and malposition of the great arteries who had undergone repair at the age of seven months. Six years later, the patient presented with right ventricular failure, conduit calcification with obstruction, and obstruction of the left ventricular outflow tract. Three-dimensional models reconstructed by Digital Imaging and Communications in Medicine (DICOM) images of the patient were visualized in a virtual reality system to help plan the surgical correction of the intracardiac congenital anomalies. This tool allowed us to inspect the intracardiac anatomy in an immersive environment with a clearer sense of perspective.

The need for concomitant aortic annular enlargement and aortic root replacement may be necessary in situations of multilevel left ventricular outflow tract obstruction and/or prior aortic or aortic valve surgery, particularly in the setting of congenital heart disease. It can be extremely tedious and fraught with numerous complications. Major challenges include bleeding in difficult-to-reach areas and the potential need for subsequent operations. This article describes a technique of concomitant aortic annular enlargement and aortic root replacement that minimizes the risk of significant blood loss and facilitates future surgery should it be necessary.

Avoiding cardiopulmonary bypass during palliation of single ventricle has the advantages of minimizing transfusions, pulmonary vascular resistance, and avoiding the inflammatory response from cardiopulmonary bypass. It is however not always straightforward, and the technique may be faced with challenges.

Left juxtaposition of the right atrial appendage (RAA) is a rare congenital cardiac malformation, where both atrial appendages are located leftward of the great arteries. Despite well-described echocardiographic markers, this diagnosis remains challenging to establish, especially for the novice imager. We describe the apical view sign in three patients with juxtaposed RAA. We noted that the normal left atrial appendage was visualized from the apical four-chamber or equivalent coronal view at the level of the atrioventricular valves.

A 15-year-old presented in cardiogenic shock secondary to viral myocarditis requiring venoarterial extracorporeal membrane oxygenation (ECMO) support. He developed large thrombi of the left ventricle and aortic root. Anticoagulation was increased, and medications were initiated to decrease the likelihood of aortic valve opening. He underwent balloon atrial septostomy followed by placement of a left atrial vent. A pigtail catheter was placed in the ascending aorta for direct heparin infusion. Serial echocardiograms showed progressive resolution of the thrombi. He was successfully weaned from ECMO and discharged home without neurological deficits.

Eisenmenger syndrome (ES) has been considered a cause of inoperability in patients with congenital heart disease (CHD). Even if long-standing vasodilators are efficient to reduce pulmonary vascular resistance (PVR), the best approach to grant stable repair of these patients is still unknown. We describe the case of a 28-year-old man with a diagnosis of untreated large ventricular septal defect and established ES. After few years of vasodilator therapy, the patient underwent pulmonary banding with significant reduction of his PVR. His CHD was then repaired, with clinical and functional improvement at 2-year follow-up.

Coronary ostial stenosis is a rare congenital cardiac anomaly, frequently associated with hypoplasia of the proximal coronary artery. This condition is potentially life-threatening, as it may present with myocardial ischemia and sudden death. We present a case of left coronary ostial stenosis in a 48-day-old infant symptomatic for sudden cardiac arrest, who successfully underwent surgical angioplasty. Any cardiac arrest in a neonate or young infant should raise suspicion of coronary ostial stenosis/atresia, considering the difficulty in diagnosing this congenital heart defect.

A newborn was diagnosed with type C esophageal atresia and d-transposition of the great arteries (d-TGA). In this report, we discuss the management implications of the co-occurrence of two rare congenital abnormalities, including extracorporeal membrane oxygenation support prior to surgical intervention for d-TGA.

Although infective endocarditis rarely appears during infancy, fungal infections remain a concern in the preterm neonatal population as the result of compromised immunity, prolonged hospitalization, and invasive procedures. The increased awareness of fungal colonization and improvements in medical therapy have not reduced the high mortality rate of mycotic infections in premature newborns, especially in developing countries, and there is no consensus on the surgical timing to treat neonatal fungal endocarditis. We present the case of a two-month-old boy with a history of three weeks of hospitalization for prematurity. After being discharged, the patient developed symptomatic candidemia resulting from a large right atrial abscess obstructing the tricuspid valve; despite antifungal therapy, urgent surgical resection was required.

Parasitic diseases may occasionally affect the cardiovascular system while it is rarely seen in childhood. Parasites may directly or indirectly affect the heart in the form of myocarditis, pericarditis, pancarditis, or pulmonary hypertension. Therefore, it should be kept in mind that parasites may be responsible for myocardial and pericardial disease anywhere around the globe. Herein, we report an adolescent boy with myocarditis associated with enteric amebiasis.

We report long-term outcome after two-stage, “one lung repair” in a four-year-old boy with tetralogy of Fallot and congenital absence of the left pulmonary artery. The operation was carried out two years after a palliative aortopulmonary central shunt and was uneventful. Twenty-six years later, the patient is in excellent clinical condition, with normal peripheral oxygen saturation. A recent radionuclide lung scan and cardiac magnetic resonance imaging show the pulmonary flow entirely directed into the right lung. In selected cases, the long-term prognosis of patients with tetralogy of Fallot and true absence of left pulmonary artery after “one lung repair” may be excellent.

Discrete fibromembranous subaortic stenosis is a common type of subaortic stenosis causing clinically significant left ventricular outflow obstruction. Surgery for discrete subaortic stenosis is most often performed through a typical midline sternotomy. Herein, we present our experience with an adult patient who underwent a right mini-thoracotomy for subaortic membrane resection with central cannulation under direct operative vision.

Prominent Eustachian valves, with obligate right-to-left shunts, have been reported as a cause of neonatal hypoxemia. This anomaly can present as an obstructive structure that inhibits antegrade flow through the tricuspid valve and furthermore contributes to right-to-left atrial shunting in the presence of a patent foramen ovale or atrial septal defect. This case highlights the evaluation and diagnostic workup for chronic hypoxemia in an adolescent female patient and considerations for percutaneous atrial septal defect closure.

Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries is a rare congenital heart lesion in which pulmonary blood supply may arise from different segments of the aorta. We report an unusual case of a newborn with a major collateral artery originating from the proximal ascending aorta. Successful reparative surgery was undertaken.

Left-sided partial anomalous pulmonary venous return (left pulmonary veins to left vertical vein) was repaired in a 70-year-old patient by ligation of the vertical vein and connection of the pulmonary veins to the left atrial appendage using a ringed polytetrafluoroethylene tube graft. The graft made the connection technically easier and facilitated a torque-free and tension-free anastomosis that was “stented” open by the rings.

The world as we once knew it has been drastically altered secondary to coronavirus disease 2019 (COVID-19). The impact of these changes, particularly for those practicing in the medical profession, extends beyond the physical to the psychological, emotional, and spiritual. We discuss the factors that contribute to these stresses, way to manage them, and how we as leaders of our teams can inspire resilience and help our colleagues endure these most difficult times.