Multivisceral Transplant in a Patient With Portopulmonary Hypertension: A Case Report |
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Affiliation: | 1. Department of Anesthesia, University of Miami/Jackson Memorial Hospital, Miami, Florida;2. Miami Transplant Institute, University of Miami/Jackson Memorial Hospital, Miami, Florida;3. Department of Surgery, University of Miami/Jackson Memorial Hospital, Miami, Florida;4. Department of Medicine, University of Miami/Jackson Memorial Hospital, Miami, Florida;1. Liver Transplant Division, Federal District Institute of Cardiology, Brasília, Brazil;2. General Surgery Department, Doutor Celio de Castro Metropolitan Hospital, Brazil;1. Division of Gastroenterology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand;2. Ramathibodi Excellence Center for Organ Transplantation, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand;3. Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand;1. Department of Anesthesia, University of Miami, Jackson Memorial Hospital, Miami, FL, USA;2. Miami Transplant Institute, University of Miami, Jackson Memorial Hospital, Miami, FL, USA;3. Department of Surgery, Miami Transplant Institute, University of Miami, Jackson Memorial Hospital, Miami, FL, USA;1. Department of Hepatology and Gastroenterology, Charité - Universitätsmedizin Berlin, Campus Virchow-Klinikum (CVK) and Campus Charité Mitte (CCM), Augustenburger Platz 1, 13353, Berlin, Germany;2. Institute of Pathology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin and Berlin Institute of Health, Berlin, Germany;3. Department of Surgery, Campus Charité Mitte | Campus Virchow-Klinikum-Charité-Universitätsmedizin Berlin, 13353, Berlin, Germany;1. Nephrology Department, Coimbra Hospital and University Centre, Coimbra, Portugal;2. Faculty of Medicine, University of Coimbra, Coimbra, Portugal;3. Nephrology Department, University Hospital Center of Algarve, Algarve, Portugal;4. Internal Medicine Department, Coimbra Hospital and University Centre, Coimbra, Portugal;5. Urology and Renal Transplant Department, Coimbra Hospital and University Centre, Coimbra, Portugal |
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Abstract: | Portopulmonary hypertension, a type of pulmonary arterial hypertension in the setting of cirrhotic 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 transplant 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, pancreaticoduodenal complex, and small and large intestine). Preoperative mean pulmonary artery pressure 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 thrombosis. 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 antiplatelet effects on a patient already susceptible to coagulopathy. A multimodal and multidisciplinary approach continued throughout the surgery and in the postoperative period, which led to a successful outcome. |
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