Liver transplantation resolves the hyperdynamic circulation in hereditary hemorrhagic telangiectasia with hepatic involvement
| Autor: | Jacques Delaye, Isabelle Mechet, François Mion, Pierre Paliard, Francesco Bianco, Danielle Gille, Henri Plauchu, Olivier Boillot, Jean-Paul Viale |
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| Rok vydání: | 1998 |
| Předmět: |
Adult
medicine.medical_specialty medicine.medical_treatment Hypertension Pulmonary Liver transplantation Gastroenterology Liver Function Tests Internal medicine medicine Humans Telangiectasia Cardiopulmonary disease Heart Failure Hepatology medicine.diagnostic_test business.industry Hemodynamics Middle Aged medicine.disease Surgery Liver Transplantation Respiratory Function Tests Transplantation Heart failure Heart catheterization Hyperdynamic circulation Female Telangiectasia Hereditary Hemorrhagic medicine.symptom business Liver function tests Tomography X-Ray Computed Liver Circulation |
| Zdroj: | Gastroenterology. 116(1) |
| ISSN: | 0016-5085 |
| Popis: | 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. GASTROENTEROLOGY 1999;116:187-192 |
| Databáze: | OpenAIRE |
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