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Stress-Echography in Hereditary Haemorrhagic Telangiectasia Patient With Hepatic Involvement — ROSE  

Hereditary Hemorrhagic Telangiectasia Clinical Trial

Official title:
Evaluation of the Value of Measuring Pulmonary Arterial Pressures During Exercise in Hereditary Haemorrhagic Telangiectasia Patients With Hepatic Involvement - Pilot Study

Clinical Trial Summary

The hepatic involvement of HHT (Hereditary Haemorrhagic Telangiectasia) disease is characterised by the formation of arterio-sus-hepatic shunts which lead to dilatation of the hepatic artery and may result in high output heart failure. This evolves silently for long-standing period from left ventricular cavities dilatation to advanced heart failure with post-capillary pulmonary hypertension (PH) (more rarely pre-capillary), and its evolution is poorly understood. The specific treatment options for HHT disease are either the use of anti-angiogenic therapy (bevacizumab) or liver transplantation. As rest echocardiography can only detect advanced cases or heart failure with rest PH, the investigators speculate that exercise echocardiography can provide additional information in patients without rest PH. The hypothesis is that an exaggerated pulmonary pressure increase during exercise may precede the occurrence of rest PH in the course of the disease. It could identify patients with substantial heart failure at an earlier stage and may facilitate the access to liver transplantation. These parameters have never been studied in this context and it seems interesting to evaluate them in this pilot study. The investigators hypothesise that HHT (Hereditary Haemorrhagic Telangiectasia) patients with hepatic involvement and cardiac high output will have significantly greater and/or earlier elevation of exercise pulmonary arterial pressures than those with normal cardiac output.

Clinical Trial Description

n/a

Study Design

Related Conditions & MeSH terms

  • Hereditary Hemorrhagic Telangiectasia
  • Telangiectasia, Hereditary Hemorrhagic
  • Telangiectasis
Clinical Trial Details
NCT number NCT05954481
Study type Interventional
Source Hospices Civils de Lyon
Contact Cyrille BERGEROT, Dr
Phone 04 72 11 93 61
Email cyrille.bergerot@chu-lyon.fr
Status Recruiting
Phase N/A
Start date August 3, 2023
Completion date August 2024
View Details
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