Use of Isolated Right Ventricular Assist Device for Refractory Graft Failure Following a Pediatric Orthotopic Heart Transplantation

Abstract

<h3>Introduction</h3> Mechanical circulatory support for isolated right ventricular (RV) failure following heart transplantation (HT) is well described in the adult literature. However, there are no similar pediatric reports . We report a case of successful use of paracorporeal CentriMag ventricular assist device as a bridge to recovery for acute RV failure following HT. <h3>Case Report</h3> This is a 12-month-old with complex congenital heart disease; single ventricle (SV), common AV valve, pulmonary atresia, heterotaxy, with dextrocardia, situs inversus and interrupted inferior vena cava with azygous continuation to a left superior vena cava. Stage 1 palliation with a ductal stent was performed in the neonatal period. She was deemed not a suitable candidate for SV palliation due to poor ventricular function as such was listed for HT. She underwent a complex HT operation including hilum-to-hilum pulmonary artery reconstruction. Intraoperative echocardiogram showed reasonable left ventricular function but poor RV function with high central venous pressure(CVP). Despite increasing inotropic support she remained hypotensive. A decision was made to place the patient on RVAD using a CentriMag device, with configuration from the right atrium to the main pulmonary artery. The patient hemodynamics improved immediately and decreased CVP. Patient was supported on RVAD for 72h before explantation. She was extubated on post op day 4 and discharged home after two weeks. At six months post HT she remains with normal graft function. <h3>Summary</h3> Acute RV dysfunction after HT is attributed to either an increase in pulmonary vascular resistance or loss of contractility in the donor heart. In this patient, a combination of pulmonary hypertension, diffuse hypoplasia of the pulmonary arteries and long donor ischemic time contributed to RV dysfunction. The present case is unique in that this patient was successfully supported with isolated RVAD, avoiding VA ECMO which is traditionally the modality of choice in such a case scenario. To the best of our knowledge, this is first case report of an infant supported on RVAD following HT. Consideration of RVAD to manage refractory primary graft failure related to RV dysfunction following HT should be considered especially in the absence of need for pulmonary or left ventricular support.