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Long term survival of patients with repaired tetralogy of Fallot is excellent (about 85% at 35 year-old). However these patients are exposed to residual pulmonary stenosis (PS) and/or pulmonary regurgitation (PR). It is well established that these lesions can lead to irreversible sequelae such as right ventricle dilatation and dysfunction. Pulmonary valve replacement technique was developed to avoid long term right ventricular dysfunction. Pulmonary valve replacement indications are based upon the presence of symptoms at exercise and/or morphological or functional parameters such as severe pulmonary regurgitation with right ventricle dilatation/dysfunction. The best timing of such intervention is still underdebate with the main aim of having the right balance between avoiding long term sequelae of PR or PS and being the latter possible to push ahead the need for new intervention. Recent publication showed that myocardial diffuse fibrosis can contribute to irreversible alteration of myocardial contractility. Quantification of diffuse fibrosis by magnetic resonance imaging is feasible and could help the physician to best determine the right timing for PVR in this population of patients. Cardiac function assessment at rest and during exercise is possible using MR and our centre has developed a program for cardiac exercise during MRI. This could help to detect infra clinic abnormality and to analyse myocardial adaptation during exercise.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| RV and LV ejection fraction assesment | Experimental | assesment RV and LV ejection fraction after PVR measured by MRI |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| MRI | Other |
|
|
| Measure | Description | Time Frame |
|---|---|---|
| determination of the quantity of diffuse myocardial fibrosis | determine if the quantity of diffuse myocardial fibrosis measured by cardiac magnetic resonnance (CMR) compare to baseline measure is correlated with decrease of RV volume and increase in RV and LV ejection fraction after PVR | 2.5 years |
| Measure | Description | Time Frame |
|---|---|---|
| determination of contractile reserve | determine if contractile reserve measured by cardiac magnetic resonnance (CMR) compare to baseline measure is correlated with decrease of RV volume and increase in RV and LV ejection fraction after PVR | 2.5 years |
| Measure | Description | Time Frame |
|---|---|---|
| determnation of variation fibrosis biomarkers | determine if the fibrosis biomarkers dosage compare to baseline measure is correlated with decrease of RV volume and increase in RV and LV ejection fraction after PVR | 2.5 years |
Inclusion Criteria:
- older than 14 year-old, with repaired tetralogy of Fallot requiring pulmonary valve
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Beatrice BONELLO, MD | Assistance Publique Hopitaux De Marseille | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Assistance Publique Hopitaux de Marseille | Marseille | 13005 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 30596647 | Result | Habert P, Bentatou Z, Aldebert P, Finas M, Bartoli A, Bal L, Lalande A, Rapacchi S, Guye M, Kober F, Bernard M, Jacquier A. Exercise stress CMR reveals reduced aortic distensibility and impaired right-ventricular adaptation to exercise in patients with repaired tetralogy of Fallot. PLoS One. 2018 Dec 31;13(12):e0208749. doi: 10.1371/journal.pone.0208749. eCollection 2018. |
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| ID | Term |
|---|---|
| D013771 | Tetralogy of Fallot |
| ID | Term |
|---|---|
| D006330 | Heart Defects, Congenital |
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D006331 | Heart Diseases |
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| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |