Stress-Echography in Hereditary Haemorrhagic Telangiectasia Patient With Hepatic Involvement

NCT ID: NCT05954481

Last Updated: 2025-12-19

Basic Information

• Recruitment Status: COMPLETED
• Clinical Phase: NA
• Total Enrollment: 47 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2023-08-03
• Study Completion Date: 2025-01-14

Brief 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.

Conditions

Hereditary Hemorrhagic Telangiectasia; Rendu Osler Disease

Keywords

HHT; Stress echography; Pulmonary arterial pressures

Study Design

• Allocation Method: NON_RANDOMIZED
• Intervention Model: PARALLEL
• Primary Study Purpose: DIAGNOSTIC
• Blinding Strategy: NONE

Study Groups

HHT patients with hepatic involvement and high cardiac index

HHT patients with a dilated (diameter \> 6mm) or tortuous hepatic artery and an elevated cardiac index (\> 3.5 l/mn/m²)

Group Type: OTHER

Stress echocardiography

Intervention Type: OTHER

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

HHT patients with hepatic involvement and normal cardiac index

HHT patients with a dilated or tortuous hepatic artery and a normal cardiac index

Group Type: OTHER

Stress echocardiography

Intervention Type: OTHER

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

Interventions

Stress echocardiography

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

Intervention Type: OTHER

Eligibility Criteria

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

• 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)

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Hospices Civils de Lyon

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Cyrille BERGEROT, MD

Role: PRINCIPAL_INVESTIGATOR

Service d'Explorations fonctionnelles cardiovasculaires - Hôpital Louis Pradel - HCL

Locations

Service Explorations fonctionnelles cardiovasculaires - Hôpital Louis Pradel

Bron, , France

Service génétique - Hôpital Femme Mère Enfant

Bron, , France

Countries

France

References

Dr. Cyrille Bergerot Co-Authors: Sophie Dupuis-Girod, MD; Alexandre Guilhem, MD; Thomas Barret, MD; Quentin Barrier, MD; Anne Emmanuelle Fargeton; Helene Thibault, MD, PhD. Exercise Pulmonary Arterial Pressure during Exercise Stress Echocardiography in Hereditary Hemorrhagic Telangiectasia. International Journal of Cardiology.

Reference Type: RESULT

Other Identifiers

69HCL22_0487

Identifier Type: -

Identifier Source: org_study_id