Portopulmonary hypertension: imatinib as a novel treatment and the Emory experience with this condition.
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%).
Tapper
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EB
Department of Anesthesiology, Emory University School of Medicine, Atlanta, Georgia, USA. Elliot.tapper@gmail.com
Knowles
D
D
Heffron
T
T
Lawrence
E Clinton
EC
Csete
M
M
D000959:Antihypertensive Agents / Q000627:therapeutic use; D001549:Benzamides; D000077300:Bosentan; D011464:Epoprostenol / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy / Q000209:etiology*; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology*; D000068877:Imatinib Mesylate; D017093:Liver Failure / Q000150:complications; D016031:Liver Transplantation / Q000009:adverse effects*; D008875:Middle Aged; D010879:Piperazines / Q000627:therapeutic use; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D013449:Sulfonamides / Q000627:therapeutic use; D016896:Treatment Outcome
D000959:Antihypertensive Agents; D001549:Benzamides; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D013449:Sulfonamides; D000068877:Imatinib Mesylate; D011464:Epoprostenol; D000077300:Bosentan