Stress-Echography in Hereditary Haemorrhagic Telangiectasia Patient With Hepatic Involvement

NCT05954481 | Completed

• 1 other identifier: 69HCL22_0487
• Study Type: interventional
• Target: 47
• Locations: 1 country
• Sites: 2

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

Outcome Measures

Primary Outcomes (1)

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

  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.

  Day 1

Study Arms (2)

HHT patients with hepatic involvement and high cardiac index

OTHER

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

Other: Stress echocardiography

HHT patients with hepatic involvement and normal cardiac index

OTHER

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

Other: Stress echocardiography

Interventions

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

HHT patients with hepatic involvement and high cardiac index; HHT patients with hepatic involvement and normal cardiac index

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• 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

You may not qualify if:

• 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)

Sponsors & Collaborators

• Hospices Civils de Lyon: lead

Study Sites (2)

Service Explorations fonctionnelles cardiovasculaires - Hôpital Louis Pradel

Bron, 69677, France

Location

Service génétique - Hôpital Femme Mère Enfant

Bron, 69677, France

Location

Related Publications (1)

• 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.

  RESULT

MeSH Terms

Conditions

Telangiectasia, Hereditary Hemorrhagic

Condition Hierarchy (Ancestors)

Hemostatic Disorders; Vascular Diseases; Cardiovascular Diseases; Telangiectasis; Hemorrhagic Disorders; Hematologic Diseases; Hemic and Lymphatic Diseases; Vascular Malformations; Cardiovascular Abnormalities; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Cyrille BERGEROT, MD

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

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NON RANDOMIZED
• Masking: NONE
• Purpose: DIAGNOSTIC
• Intervention Model: PARALLEL
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: July 13, 2023
• First Posted: July 20, 2023
• Study Start: August 3, 2023
• Primary Completion: January 14, 2025
• Study Completion: January 14, 2025
• Last Updated: December 19, 2025

Record last verified: 2025-12

Data Sharing

• IPD Sharing: Will not share

Locations

FR (2)