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

• Published in: Transplantation proceedings Vol. 41; no. 5; pp. 1969 - 1971
• Main Authors: Tapper, E.B, Knowles, D, Heffron, T, Lawrence, E. Clinton, Csete, M
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier Inc 01-06-2009; Elsevier
• Subjects: Antihypertensive Agents - therapeutic use; Benzamides; Biological and medical sciences; Epoprostenol - therapeutic use; Female; Fundamental and applied biological sciences. Psychology; Fundamental immunology; Gastroenterology. Liver. Pancreas. Abdomen; Humans; Hypertension, Portal - drug therapy; Hypertension, Portal - etiology; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - etiology; Imatinib Mesylate; Liver Failure - complications; Liver Transplantation - adverse effects; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Medical sciences; Middle Aged; Other diseases. Semiology; Piperazines - therapeutic use; Protein Kinase Inhibitors - therapeutic use; Pyrimidines - therapeutic use; Sulfonamides - therapeutic use; Surgery; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Tissue, organ and graft immunology; Treatment Outcome; Antineoplastic agent; Imatinib; Portal circulation disease; Enzyme; Tyrosine kinase inhibitor; Transferases; Enzyme inhibitor; Cardiovascular disease; Transplantation; Vascular disease; Medicine; Experience; Treatment; Surgery; Portal hypertension; Digestive diseases; Graft; Portopulmonary hypertension; Protein-tyrosine kinase
• ISSN: 0041-1345; 1873-2623
• DOI: 10.1016/j.transproceed.2009.02.100

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%).