Successful Bridge to Heart Transplantation in a Pediatric Patient with Biventricular Heart Failure Associated with Barth Syndrome: a Case Report

Long waiting times for donor organs in pediatric patients have increased the need for mechanical circulatory support as a bridge to heart transplantation. We report the case of a one-year-old boy with Barth syndrome who presented with cardiogenic shock due to severe biventricular heart failure. Emergency biventricular assist device support using centrifugal pumps was initiated. Under severe pulmonary hypertension and pulmonary regurgitation, the right ventricular assist device outflow was temporarily directed to the left atrium to optimize left ventricular preload. As pulmonary hemodynamics improved, the right ventricular assist device outflow was redirected to the pulmonary artery, allowing evaluation of native pulmonary circulation and subsequent right ventricular assist device weaning. After recovery of end-organ function, the left ventricular assist device was converted to a Berlin Heart EXCOR 76 days after biventricular assist device implantation. The patient remained stable on Berlin Heart EXCOR support for 374 days and subsequently underwent successful heart transplantation. This case highlights the importance of early right ventricular assist device support and strategic management of right ventricular assist device outflow to optimize left ventricular assist device performance in pediatric patients with severe pulmonary hypertension. Such an approach may facilitate successful bridge-to-candidacy and eventual heart transplantation in high-risk pediatric patients.