Hereditary Hemorrhagic TelangiectasiaROSENCT05954481
Stress-Echography in Hereditary Haemorrhagic Telangiectasia Patient With Hepatic Involvement
A study testing Stress echocardiography in people with Hereditary Hemorrhagic Telangiectasia and Rendu Osler Disease, run by Hospices Civils de Lyon at 2 sites in France.
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. Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Hereditary Hemorrhagic Telangiectasia, Rendu Osler Disease
- What is being tested?
- Stress echocardiography
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 47 participants
- Where
- 2 sitesFrance
- Study dates
- 3 Aug 2023to 14 Jan 2025
- Phase
- Interventional
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Who may be able to join
- 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
Who cannot join
- 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)
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- 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)
Trial locations and contacts
2 sites in 1 country.
France2 sites
Service Explorations fonctionnelles cardiovasculaires - Hôpital Louis Pradel
Bron, 69677
Service génétique - Hôpital Femme Mère Enfant
Bron, 69677
About this study
Official title: Evaluation of the Value of Measuring Pulmonary Arterial Pressures During Exercise in Hereditary Haemorrhagic Telangiectasia Patients With Hepatic Involvement - Pilot Study
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.
- Being tested
- Stress echocardiography
- Keywords
- HHT, Stress echography, Pulmonary arterial pressures
- Medical subject headings
- Telangiectasia, Hereditary Hemorrhagic
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Stress echocardiographyOther
After a rest trans thoracic echocardiography (usual follow-up), eligible patients will have a stress ultrasound echocardiography on a dedicated cycle ergometer
| Study group | Role | What they receive |
|---|---|---|
| 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) Receives: 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 Receives: Other: Stress echocardiography |
What is being measured
The primary outcome is the main question the study is designed to answer.
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.
Measured: Day 1
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted20 Jul 2023
- Study start3 Aug 2023
- Primary completion14 Jan 2025
- Study completion14 Jan 2025
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (1)Papers the sponsor has linked to this study.
- Other trials for Hereditary Hemorrhagic TelangiectasiaStudies of the same condition, open and under way.
- More trials run by Hospices Civils de LyonThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase N/A
- Primary purpose
- Diagnosis
- Allocation
- Non-randomised
- Intervention model
- Parallel groups
- Masking
- None (open label)
- Enrolment
- 47 participants (actual)
Sponsor and investigators
- Lead sponsor
- Hospices Civils de Lyon
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Cyrille BERGEROT, MD, Principal investigator, Service d'Explorations fonctionnelles cardiovasculaires - Hôpital Louis Pradel - HCL
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT05954481
- Sponsor's protocol ID
- 69HCL22_0487
- Acronym
- ROSE
- Status last verified
- Dec 2025
- First submitted
- 13 Jul 2023
- Last updated
- 19 Dec 2025
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Publications
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.