Portopulmonary Hypertension: Imatinib as a Novel Treatment and the Emory Experience With this Condition

Abstract Portopulmonary hypertension (PoPH) is a common and feared complication of end-stage liver disease, and imposes increased risk of perioperative morbidity in the liver transplant patient. Herein, we present the first successful use of Imatinib in the perioperative management of a patient who was not responding to conventional treatments as well as our institution's experience with this devastating complication. Of patients evaluated for transplant, 4.1% were identified with PoPH, half of which were listed, and one quarter of which were transplanted. Patients with PoPH were twice as likely to be transplanted than all other candidates (48% vs 25%), though less likely to survive their first year (69% vs 86.4%).