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.