title: The impact of treatment of portopulmonary hypertension on survival following liver transplantation.
abstract: Pulmonary hypertension in the setting of cirrhosis and portal hypertension is known as portopulmonary hypertension (PPHTN). Moderate or severe PPHTN is uncommon, but has a poor prognosis and is considered to be a contraindication to liver transplantation. We assessed the impact of vasodilation therapy on pulmonary hemodynamics and outcome after liver transplant in these patients. Eighty-six patients evaluated for liver transplant between 1997 and 2005 had an estimated right ventricular systolic pressure >40 mm Hg or a clinical suspicion of PPHTN. Right heart catheterization confirmed PPHTN in 30 patients (ten mild, eight moderate, and 12 severe). Sixteen of the 20 with moderate-to-severe pulmonary hypertension (mPAP >or= 35) were otherwise considered suitable liver transplant candidates and were treated with vasodilation therapy. mPAP fell to less than 35 mm Hg in 12 patients (75%) and 11 of them then underwent orthotopic liver transplantation. One- and five-year survivals in the transplanted patients were 91% and 67%, respectively. Nine of 11 were off vasodilator therapy after a median of 9.2 months following transplantation. None of the patients who failed vasodilator therapy survived (median survival, 8 months). Effective pharmacologic control of PPHTN before liver transplant is associated with excellent posttransplant survival that is similar to patients transplanted for other indications.
authors.affiliation: Department of Medicine, Baylor Regional Transplant Institute, Baylor University Medical Center, Dallas, TX, USA.
authors.forename: M
authors.initials: M
authors.lastname: Ashfaq
authors.forename: S
authors.initials: S
authors.lastname: Chinnakotla
authors.forename: L
authors.initials: L
authors.lastname: Rogers
authors.forename: K
authors.initials: K
authors.lastname: Ausloos
authors.forename: S
authors.initials: S
authors.lastname: Saadeh
authors.forename: G B
authors.initials: GB
authors.lastname: Klintmalm
authors.forename: M
authors.initials: M
authors.lastname: Ramsay
authors.forename: G L
authors.initials: GL
authors.lastname: Davis
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D001794:Blood Pressure / Q000187:drug effects / Q000502:physiology; D000077300:Bosentan; D004110:Diltiazem / Q000627:therapeutic use; D011464:Epoprostenol / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D008103:Liver Cirrhosis / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D016031:Liver Transplantation / Q000401:mortality* / Q000502:physiology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013449:Sulfonamides / Q000627:therapeutic use; D015996:Survival Rate; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D014665:Vasodilator Agents; D011464:Epoprostenol; D004110:Diltiazem; D000077300:Bosentan