Successful outcomes following living donor liver transplantation for portopulmonary hypertension.

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.

A 45-year-old man with a lung mass and history of charcoal aspiration.

A 45-year-old man was seen in consultation for evaluation of a spiculated right-lower-lobe mass that enlarged over 1 year. The patient had suffered accidental instillation of activated charcoal into the right lung via nasogastric tube 2 years prior to this consultation, with resultant respiratory failure, pneumonia, and pneumothorax. Biopsy of the mass showed anthracosis and granulomatous inflammation. A positron emission tomogram was strongly positive at the lesion, and right-lower-lobectomy with partial diaphragmatic resection was performed. On gross examination of the mass, a charcoal concretion was evident. Histologic examination showed intrinsic and surrounding granulomatous inflammation, but without tumor. The patient recovered uneventfully, and after 1 year had not experienced further complications.