Portopulmonary Hypertension in Patients Undergoing Liver Transplantation: A Case Report

Abstract

Portopulmonary hypertension (PPH) is a form of pulmonary arterial hypertension, characterized by elevation of pulmonary arterial pressure due to increased resistance to pulmonary blood flow. It is a consequence of portal hypertension, associated or not with liver disease. A retrospective study of patients undergoing liver transplantation was carried out in the Liver Transplantation Unit of the Clinics Hospital of the State University of Campinas (UNICAMP) from 1991 to 2012, covering 389 transplants. Although 50 (12.9%) patients presented with mean pulmonary arterial pressure (MPAP) > 25 mmHg, only one case (0.25%) met the diagnostic criteria for PPH: a 43-year-old female patient with advanced liver disease, caused by primary biliary cirrhosis with portal hypertension, diagnosed as mild portopulmonary hypertension and diagnosed in the operating room. PPH is a serious complication of portal hypertension. It is related to the great difficulty in diagnosis and the high mortality rate due to right heart failure. PPH is an entity with a low frequency of occurrence in individuals undergoing liver transplantation, although there is a wide variation in the prevalence rate presented in the literature and the definitive diagnosis should be confirmed with right heart catheterization.