Document Detail

Hereditary hemorrhagic telangiectasia, liver vascular malformations and cardiac consequences.
MedLine Citation:
PMID:  23312966     Owner:  NLM     Status:  Publisher    
BACKGROUND: HHT patients with liver vascular malformations (VMs) may develop high-output cardiac failure requiring liver transplant in few cases. OBJECTIVE: Our aim is to show that echocardiography is a good tool to evaluate the severity of hepatic vascular malformations in HHT and can improve medical management in HHT patients. METHODS: The study is a cross-sectional study of cardiac parameters in HHT patients with dyspnea in a single referral center. All HHT patients with dyspnea, consecutively seen at HHT reference center in Lyon between May 2007 and November 2009 were included and had hepatic vascular Ultrasound and Echocardiography. Echocardiographic measures included cardiac output (CO) and index (CI), left ventricle (LV) filling pressures, and pulmonary artery pressure. Then, patients were classified in 4 groups according to the severity: group 1 (normal values), group 2 with isolated high CI, group 3 with high CI and increased LV-filling pressures and group 4 with increased LV-filling pressures and pulmonary hypertension. RESULTS: Fifty-two HHT-patients were analyzed. Eight patients were in group 1, 25 in group 2, 6 in group 3 and 13 in group 4. Age, NYHA class dyspnea, edema, atrial fibrillation, hepatic artery diameter, and BNP (brain natriuretic peptide) levels significantly increased from groups 1 to 4 as well as left atrial area, and presence of mitral regurgitation. Patients with associated pulmonary VMs (n=11) did not show any clinical or echocardiographic differences. CONCLUSION: Performing echocardiography in HHT patients with dyspnea allowed us to better understand the physiological processes of high-CO failure complicating liver vascular malformations and may improve follow-up of patients and treatment decisions.
Isabelle Ginon; Evelyne Decullier; Gerard Finet; Jean-Francois Cordier; Denis Marion; Jean-Christophe Saurin; Sophie Dupuis-Girod
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-1-8
Journal Detail:
Title:  European journal of internal medicine     Volume:  -     ISSN:  1879-0828     ISO Abbreviation:  Eur. J. Intern. Med.     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-1-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9003220     Medline TA:  Eur J Intern Med     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
Hospices Civils de Lyon, Service d'Explorations Cardiologiques, Centre Hospitalier Lyon Sud, Pierre-Bénite, France. Electronic address:
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