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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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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| 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) |
|
| HHT patients with hepatic involvement and normal cardiac index | Other | HHT patients with a dilated or tortuous hepatic artery and a normal cardiac index |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| 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 |
| Measure | Description | Time Frame |
|---|---|---|
| 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 |
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Inclusion Criteria:
Exclusion Criteria:
Secondary Exclusion Criteria verified on rest ultrasound
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| Name | Affiliation | Role |
|---|---|---|
| Cyrille BERGEROT, MD | Service d'Explorations fonctionnelles cardiovasculaires - Hôpital Louis Pradel - HCL | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Service Explorations fonctionnelles cardiovasculaires - Hôpital Louis Pradel | Bron | 69677 | France | |||
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| Result | 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. |
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| ID | Term |
|---|---|
| D013683 | Telangiectasia, Hereditary Hemorrhagic |
| ID | Term |
|---|---|
| D020141 | Hemostatic Disorders |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
| D013684 | Telangiectasis |
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| Service génétique - Hôpital Femme Mère Enfant |
| Bron |
| 69677 |
| France |
| D006474 |
| Hemorrhagic Disorders |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D054079 | Vascular Malformations |
| D018376 | Cardiovascular Abnormalities |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |