Berlin heart EXCOR® implantation for Fontan failure in right-sided heterotaxy with ventricular septal protuberance:...

A 4-year-old boy with a diagnosis of right-sided heterotaxy syndrome was transferred to our hospital under the extracorporeal membrane oxygenation (ECMO). He had undergone an extracardiac total cavopulmonary connection (TCPC) with a fenestration. Approximately 6 months after the TCPC procedure, common atrioventricular valve regurgitation worsened and persisted despite multiple valve repair procedures. ECMO support was initiated 318 days after the TCPC procedure for progressive heart failure and multiple organ dysfunction. Although ECMO was initially discontinued after transient improvement, recurrent circulatory failure necessitated reinitiation, and the patient remained ECMO-dependent thereafter. We implanted a Berlin Heart EXCOR® Pediatric ventricular assist device. In the operation, the fenestration of the TCPC conduit was closed. An expanded polytetrafluoroethylene conduit for the arterial cannula was anastomosed to the ascending aorta in an end-to-side fashion. The apex of the left-sided ventricle was incised, followed by the meticulous resection of residual ventricular septal tissue to obtain effective venting from both ventricles. An inflow cannula was anastomosed to the coring site. Ventricular assist support was initiated without complications. On the postoperative day 433, intracerebral hemorrhage was abruptly developed, necessitating management in the intensive care unit. Two hematoma evacuation procedures of decompressing surgery for intracerebral hemorrhage were required. Despite these interventions, the patient ultimately died on postoperative day 488. The autopsy confirmed that the residual ventricular septum-like tissue had been almost completely resected and that the inflow cannula tip was positioned away from any muscular structures, suggesting that the resection enabled effective ventricular drainage without obstruction or suction.