Orthotopic liver transplantation in a patient with severe portopulmonary hypertension


Address:Dr. Peter Mair
Department of Anaesthesia and Intensive Care Medicine
University Hospital Innsbruck
Anichstrasse 35
A-6020 Innsbruck


Liver transplantation in patients with severe portopulmonary hypertension (PPH) has been associated with mortality rates in the range of 70% to 80%. Preoperative long-term epoprostenol therapy reverses pulmonary hypertension and may be a valuable possibility to reduce mortality in patients with severe PPH undergoing orthotopic liver transplantation. We want to report a patient with severe PPH, who was treated with intravenous epoprostenol for an 8-month period, after which pulmonary vascular resistance had decreased from 12 to 3 Wood units. Nevertheless, the patient developed intractable perioperative right heart failure necessitating transient mechanical circulatory support. The patient was weaned from mechanical circulatory support, but died from another episode of acute right heart failure after 28 days. Complicated liver transplantation associated with major cardiovascular stress is obviously not tolerated in patients with severe portopulmonary hypertension even after preoperative long-term epoprostenol therapy.