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Successful outcomes following living donor liver transplantation for portopulmonary hypertension
Author(s) -
Bandara Mahesh,
Gordon Fredric D.,
Sarwar Akmal,
Knauft M. Elizabeth,
Pomfret Elizabeth A.,
Freeman Richard B.,
Wirth Joel A.
Publication year - 2010
Publication title -
liver transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1527-6465
DOI - 10.1002/lt.22107
Subject(s) - medicine , portopulmonary hypertension , contraindication , liver transplantation , liver disease , population , transplantation , pulmonary hypertension , intensive care medicine , portal hypertension , chronic liver disease , perioperative , surgery , cirrhosis , pathology , alternative medicine , environmental health
Pulmonary arterial hypertension (PAH) associated with portal hypertension [portopulmonary hypertension (PPHTN)] occurs in 2% to 10% of patients with advanced liver disease and carries a very poor prognosis without treatment. Most hepatic transplantation centers consider moderate to severe PPHTN to be a contraindication to liver transplantation because of the high rate of perioperative complications. We present 3 patients with PPHTN who were managed with intravenous prostacyclin therapy followed by living donor liver transplantation (LDLT). These individuals demonstrated subsequent resolution of their pulmonary hypertension and were weaned off all PAH‐specific medical therapy. We present their demographics, clinical courses, and hemodynamics. We discuss the potential indications for LDLT and risks with respect to this patient population. Limitations of the Model for End‐Stage Liver Disease scoring system and outcome data for this patient population are reviewed. Future studies should be directed toward better defining indications for LDLT in patients with PPHTN, improving medicosurgical management, and assessing long‐term outcomes. Liver Transpl 16:983‐989, 2010. © 2010 AASLD.