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Extracorporeal Membrane Oxygenation and Severe Portopulmonary Hypertension following Liver Transplantation: Brief Report

Background Patients with severe portopulmonary hypertension (PoPH) responsive to medical therapy may be considered for liver transplantation. We present a case of extracorporeal membrane oxygenation (ECMO) resuscitation for PoPH crisis in a child following liver transplantation (LT), and review the literature on management of this challenging setting. Case report A 7-year-old girl, with previous Kasai portoenterostomy and subsequent severe PoPH responsive to pulmonary vasodilator therapy, underwent orthotopic LT. Five days following surgery, she had an asystolic arrest with suprasystemic pulmonary hypertension, and was resuscitated with ECMO therapy. Multi-modal strategies included sildenafil, ambrisentan, nitric oxide, intravenous Epoprostenol infusion, levosimendan, and atrial septostomy. Ten days after her LT, exploration for bleeding was necessary following abdominal drain removal. By 10 days of ECMO support, she was reviewed and considered for lung transplantation. Unfortunately, she deteriorated precipitously with abdominal compartment syndrome and multi-organ failure; sadly, life support was withdrawn 23 days after transplantation. Discussion Patients with severe PoPH may need combined thoracic organ and liver transplantation either at single or serial events. Case reports on ECMO use include resuscitation after massive pulmonary embolism during liver transplantation, bridge until the goal of vasodilatory therapy was reached in worsening PoPH following LT, and bridge to lung or repeat liver transplantation for severe pulmonary hypertension. Conclusions ECMO resuscitation and support may be deployed as rescue therapy around the period of liver transplantation. We highlight the importance of patient selection and high risk of complications during ECMO therapy as a bridge to PoPH control.