The management of severe portopulmonary hypertensionin patients undergoing liver transplantation

Abstract

Aim Severe portopulmonary arterial hypertension (PoPAH) is still considered a contraindication for liver transplantation at most transplant centers due to its high morbidity and mortality rate. However, with the advances in specific pulmonary arterial hypertension therapy and transplantation surgery, the outcomes of these patients are significantly improving.Methods All patients who were candidates for liver transplantation underwent a standardized preoperative pulmonary and cardiac evaluation. Assessment and staging severity of PoPAH was based on transthoracic Doppler echo-cardiographic examination. Severe pulmonary hypertension was defined as mean pulmonary artery pressure > 45 mm Hg. A standardized preoperative bridging therapy and intraoperative pulmonary hypertension management were performed in patients with severe PoPAH. Because of the therapeutic potential of inhaled nitric oxide as a selective pulmonary vasodilator, it was used perioperatively for all patients in combination with sildenafil. Clinical and demographic variables and laboratory data were
noted.Results Among 505 patients who underwent liver transplantation, 5 whose mean pulmonary artery pressure > 45 mm Hg were enrolled. While 2 of 5 patients received cadaveric livers, the other 3 were living donor transplantation, and one of them was pediatric. There was no perioperative mortality. During our follow-up only 1 patient died three years after transplantation because of coronary artery disease. Conclusion We recommend that liver transplantation should not be denied to all patients with severe PoPAH if they do not have right ventricular dysfunction. With bridging therapy, advanced anesthetic and surgical management, patients with severe PoPAH can be candidates for liver transplantation.

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