Stress-Echography in Hereditary Haemorrhagic Telangiectasia Patient With Hepatic Involvement

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.

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Key information

Conditions

Hereditary Hemorrhagic Telangiectasia Cardiovascular Abnormalities Cardiovascular Diseases Congenital Abnormalities Congenital, Hereditary, and Neonatal Diseases and Abnormalities Hematologic Diseases Hemic and Lymphatic Diseases Hemorrhagic Disorders Hemostatic Disorders Rendu Osler Disease Telangiectasia, Hereditary Hemorrhagic Telangiectasis Vascular Diseases Vascular Malformations

Age range

18 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Not applicable

Primary location

Service Explorations fonctionnelles cardiovasculaires - Hôpital Louis Pradel, Bron, France

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

HHT patient > 18 yo
Hepatic involvement (hepatic artery diameter > 6mm)
Patient having received the information and signed the informed consent form
Patient affiliated to a social security scheme or beneficiaries of a similar scheme

Exclusion criteria

Haemoglobin < 90 g/L
Active Infection
Atrial Fibrillation permanent or persistent
Known cardiopathy
Pregnant or breastfeeding woman (by questioning)
Adult subject to a legal protection measure (guardianship)
Participation in another clinical trial that may interfere with the proposed trial (investigator judgment)
Patient physically unable to pedal
Patient with Pulmonary arteriovenous malformations awaiting embolization
Patient treated with beta-blockers

Secondary Exclusion Criteria verified on rest ultrasound

PAH (Pulmonary Arterial Hypertension) at rest (Vmax > 2.8 m/sec)
Inability to obtain a stream of IT at rest
Patient with atrial fibrillation (AF)

Treatment and study plan

Stress echocardiography

Other

After a rest trans thoracic echocardiography (usual follow-up), eligible patients will have a stress ultrasound echocardiography on a dedicated cycle ergometer

Primary outcomes

Comparison of exercise pulmonary artery systolic pressure in patients with HHT and liver involvement.

Time frame: Day 1

The systolic pulmonary artery pressure (PAPS) during exercise will be compared between the two groups of patients. PAPS will be estimated by the maximum velocity (Vmax) of tricuspid insufficiency flow at peak exercise on cardiac ultrasound.

Sponsors and collaborators

Lead sponsor

Hospices Civils de Lyon

Other

Registry information

Official study title

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

Acronym: ROSE

Important dates

Study start: 2023
Primary completion: 2025
Study completion: 2025
First posted: Jul 20, 2023
Registry last updated: Dec 19, 2025

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

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This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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