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the first purpose of the study is to determine the adaptative mechanisms of right ventricle (RV) to systemic afterload, and the mechanisms of RV failure, in patients with congenital heart disease and subaortic RV, using cardiac magnetic resonance imaging (CMR).The mechanisms are evaluated by measures of RV remodelling and RV wall stress using CMR. Second objectives are to evaluate these mechanisms using echography, arterial properties study and neurohormonal levels
Right ventricle (RV) in sub-aortic position is a rare situation, mainly in two congenital heart defects: congenitally corrected transposition of the great arteries and complete transposition of the great arteries palliated by atrial switch. In these patients, increase of afterload leads to hypertrophy and late RV dilatation. The stress exercised on RV walls could play a role in adaptative mechanisms to systemic afterload. Beyond a remodelling threshold, it could cause fibrosis damage and RV systolic failure. Magnetic resonance imaging, which has a major potential in estimation of RV remodelling, wall stress and fibrosis, could shed light on RV adaptation to systemic afterload and evolution towards failure. Systemic RV remodelling and function could also depend on the neuro-hormone secretion and mechanical arterial properties, that have a direct influence on patients afterload. The first purpose of the study is to determine the adaptative mechanisms of RV to systemic afterload, and the mechanisms of RV failure, in patients with congenital heart disease and subaortic RV, using cardiac magnetic resonance imaging (CMR).The mechanisms are evaluated by measures of RV remodelling and RV wall stress using CMR. Second objectives are to evaluate these mechanisms using echography, arterial properties study and neurohormonal levels
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| asymptomatic group | Sham Comparator | patients with systemic right ventricle who are asymptomatic, |
|
| symptomatic group | Sham Comparator | symptomatic group : patients with systemic right ventricle and heart failure signs and/or decrease exercise performance |
|
| control | Sham Comparator | healthy subject matched with patients of asymptomatic group |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| asymptomatic group | Other | 2 visits with MRI |
| |
| Measure | Description | Time Frame |
|---|---|---|
| remodelling indexes | 48 months | |
| wall stress index | 48 months | |
| fibrosis of systemic RV measured by MRI | 48 months |
| Measure | Description | Time Frame |
|---|---|---|
| echographic measures of RV function and geometry indexes | comparison between patients and healthy subjects and analysis of the evolution of the measured parameters in the 2 groups after one year of follow | 48 months |
| Arterial mechanical properties (stiffness aortic distensibility and arterial compliance) |
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Patients inclusion criteria:
Patients non-inclusion criteria:
healthy subjects inclusion criteria:
healthy subjects Exclusion criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Magalie Ladouceur, MD | Université Paris Descartes | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hôpital Européen Georges Pompidou | Paris | 75015 | France |
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| ID | Term |
|---|---|
| D006330 | Heart Defects, Congenital |
| ID | Term |
|---|---|
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D006331 | Heart Diseases |
| D000013 | Congenital Abnormalities |
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| symptomatic group |
| Other |
2 visits with MRI |
|
comparison between patients and healthy subjects and analysis of the evolution of the measured parameters in the 2 groups after one year of follow |
| 48 months |
| Rate of neurohormones of the renin-angiotensin-aldosterone system and neuropeptides | 48 months |
| Variability and reproducibility indices remodeling and wall stress MRI | inter and intra-observer variability and inter-examination MRI measures | 48 months |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |