Liver transplantation resolves the hyperdynamic circulation in hereditary hemorrhagic telangiectasia with hepatic involvement

Background & Aims: Hepatic involvement in hereditary hemorrhagic telangiect asia is common but often asymptomatic. However, in some cases, the vascular lesions that involve the liver may lead to high-output cardiac failure and pulmonary hypertension that is predominant over hepatobiliary manifestatio ns. Liver transplantation and treatment of these complications are describe d and discussed in this article. Methods: Three patients with hereditary he morrhagic telangiectasia and hepatic involvement received transplants. They had pulmonary hypertension and chronic right-sided heart failure caused by disseminated intrahepatic telangiectasias with shunts between the hepatic artery and hepatic veins or portal vein. Left-to-right intrahepatic shunt o utput was estimated to range between 51% and 57.5% of cardiac output. Resul ts: Hyperdynamic circulation disappeared after liver transplantation in all patients. Results of computed tomography and right-sided heart catheteriza tion performed 6 months later were normal. Follow-up periods currently are 65, 53, and 29 months, and each patient continues to be asymptomatic. Concl usions: This report suggests that liver transplantation can be considered a s an alternative and successful curative treatment that may prevent the irr eversible evolution of cardiopulmonary disease.