Orthotopic liver transplantation in a patient with severe portopulmonary hypertension

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