title: Portopulmonary hypertension in liver transplant candidates.
abstract: Pulmonary vascular disorders including portopulmonary hypertension (PoPHT) are among the common complications of liver disease and are prognostically significant. Survival is very low without medical treatment and liver transplantation. With advances in medical therapy for elevated pulmonary artery pressure (PAP) and liver transplant surgery, survival of patients with PoPHT and advanced liver disease is significantly improved. Because of the prognostic significance of PoPHT and the limited donor pool, a comprehensive preoperative cardio-pulmonary assessment is of great importance in cirrhotic patients prior to transplant surgery. Therefore, a detailed transthoracic Doppler echocardiographic examination must be an essential component of this evaluation. Patients with mild PoPHT can safely undergo liver transplant surgery. In cases of moderate to severe PoPHT, right heart catheterization (RHC) should be performed. In patients with moderate to severe PoPHT on RHC (mean PAP 35-45 mmHg), vasodilator therapy should be attempted. Liver transplantation should be encouraged in cases that demonstrate a positive response. Bridging therapy with specific pulmonary arterial hypertension treatment agents should be considered until the transplant surgery and should be continued during the peri- and post-operative periods as needed.
authors.affiliation: Serife Savas Bozbas, Department of Pulmonary Disease, Baskent University Faculty of Medicine, 06490 Ankara, Turkey.
authors.forename: Serife Savas
authors.initials: SS
authors.lastname: Bozbas
authors.affiliation: Serife Savas Bozbas, Department of Pulmonary Disease, Baskent University Faculty of Medicine, 06490 Ankara, Turkey.
authors.forename: Huseyin
authors.initials: H
authors.lastname: Bozbas
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D006328:Cardiac Catheterization; D015150:Echocardiography, Doppler; D058625:End Stage Liver Disease / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D008103:Liver Cirrhosis / Q000150:complications* / Q000175:diagnosis; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D018570:Risk Assessment; D012307:Risk Factors; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents
keywords: Liver disease; Liver transplantation; Portal hypertension; Portopulmonary hypertension; Pulmonary arterial hypertension