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

Gastroenterology. 1999 Jan;116(1):187-92. doi: 10.1016/s0016-5085(99)70243-x.


Background & aims: Hepatic involvement in hereditary hemorrhagic telangiectasia 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 manifestations. Liver transplantation and treatment of these complications are described and discussed in this article.

Methods: Three patients with hereditary hemorrhagic 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 output was estimated to range between 51% and 57.5% of cardiac output.

Results: Hyperdynamic circulation disappeared after liver transplantation in all patients. Results of computed tomography and right-sided heart catheterization performed 6 months later were normal. Follow-up periods currently are 65, 53, and 29 months, and each patient continues to be asymptomatic.

Conclusions: This report suggests that liver transplantation can be considered as an alternative and successful curative treatment that may prevent the irreversible evolution of cardiopulmonary disease.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Female
  • Heart Failure / complications
  • Heart Failure / prevention & control
  • Hemodynamics / physiology
  • Humans
  • Hypertension, Pulmonary / complications
  • Liver Circulation*
  • Liver Function Tests
  • Liver Transplantation*
  • Middle Aged
  • Respiratory Function Tests
  • Telangiectasia, Hereditary Hemorrhagic / complications
  • Telangiectasia, Hereditary Hemorrhagic / physiopathology*
  • Telangiectasia, Hereditary Hemorrhagic / surgery*
  • Tomography, X-Ray Computed