Portopulmonary hypertension as an indication for combined heart, lung, and liver or lung and liver transplantation: literature review and case presentation.

End-stage liver disease with severe portopulmonary hypertension (PPHTN), which is refractory to vasodilator therapies, is a contraindication for isolated liver transplantation (LT) because of the high mortality rate. Combined heart, lung, and liver transplantation (CHLLT) and combined lung and liver transplantation (CLLT) can be lifesaving options for these patients; however, these procedures have rarely been performed. A 52-year-old man had end-stage liver disease due to hepatitis C and PPHTN; the latter showed a suboptimal response to pulmonary vasodilator therapy with continuous intravenous treprostinil sodium and oral sildenafil citrate and was considered a contraindication to isolated LT. His preoperative left ventricular function was normal, and he had mild to moderate right ventricular dysfunction. He underwent CLLT, which consisted of sequential double-lung transplantation under cardiopulmonary bypass followed by standard LT under venovenous bypass. Re-exploration of the chest cavity was necessary because of bleeding, and respiratory failure developed; however, the patient recovered, was discharged home on day 26, and remained well 1 year after CLLT with the standard immunosuppressants (similar to those used for heart and lung transplantation). For PPHTN, combined thoracic organ and liver transplantation has been reported in only 10 patients. Six of these patients, including our case, underwent CLLT, whereas 4 patients underwent CHLLT. Notably, 2 of the 6 CLLT patients expired within 24 hours of transplantation because of acute right heart failure. CHLLT should be considered for patients with refractory PPHTN. The assessment of preoperative cardiac function is a vital part of the decision to include heart transplantation in CLLT.