Multivisceral Transplant in a Patient With Portopulmonary Hypertension: A Case Report

Portopulmonary hypertension, a type of pulmonary arterial hypertension in the setting of cir-rhotic or noncirrhotic portal hypertension, is associated with elevated morbidity and mortality during and after transplantation. Uncontrolled portopulmonary hypertension may prevent or delay listing for transplant candidates, and the prognosis without treatment and ultimately trans-plant is extremely poor. We present a 29-year-old White woman, who had a post-liver transplant at infancy due to biliary atresia. Later on, she developed extensive portal vein thrombosis and portopulmonary hypertension and underwent a multivisceral transplant (liver, stomach, pancrea-ticoduodenal complex, and small and large intestine). Preoperative mean pulmonary artery pres-sure was <30 mm Hg with a pulmonary vascular resistance of <300 dynes.s/cm5 on oral sildenafil and intravenous epoprostenol. Intraoperatively, management required comprehensive transfusion protocols, a careful balance between correcting blood loss and preventing thrombo-sis. Intravenous epoprostenol, sildenafil, milrinone, and inhaled nitric oxide were used to reduce elevated mean pulmonary artery pressure and right ventricular strain associated with vascular clamping, reperfusion, and massive fluid shifts. Nitric oxide and epoprostenol use unleashed anti -platelet effects on a patient already susceptible to coagulopathy. A multimodal and multidisci-plinary approach continued throughout the surgery and in the postoperative period, which led to a successful outcome.