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

Abstract

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.

• Authors: O Boillot, F Bianco, J P Viale, F Mion, I Mechet, D Gille, J Delaye, P Paliard, H Plauchu
• Journal: Gastroenterology (Gastroenterology) Vol. 116 Issue 1 Pg. 187-92 (Jan 1999) ISSN: 0016-5085 [Print] United States
• PMID: 9869617 (Publication Type: Case Reports, Journal Article)

Topics

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