Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society
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Review Case Reports
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. ⋯ 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.