Myocardial FIbrosis in Repaired Tetralogy of FAllot- FIFA Study)

Study Overview
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Brief Summary
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This study aims to study the correlation between biomarkers of myocardial fibrosis (extracellular volume fraction calculated by cardiac magnetic resonance imaging (MRI) (T1-mapping) and levels of molecular biomarkers of fibrosis) and adverse events in a population of patients with repaired tetralogy of Fallot.

Detailed Description
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The main causes of mortality in adults with repaired tetralogy of Fallot (TF) are sudden death and heart failure. Myocardial fibrosis has been linked to the appearance of arrhythmias and ventricular dysfunction in other patient populations, but this association is poorly studied in patients with TF, perhaps because research in congenital heart disease (CHD) requires multicenter studies, difficult to carry out. Interstitial myocardial fibrosis assessed by molecular and imaging biomarkers is associated with adverse events in patients with repaired Fallot tetralogy.

Official Title
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Interstitial Myocardial Fibrosis in Repaired Tetralogy of Fallot: Assessment by Molecular and Imaging Biomarkers and Association With Adverse Events ( Myocardial FIbrosis in Repaired Tetralogy of FAllot- FIFA Study)

Conditions
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Congenital Heart Disease, Congenital Heart Defect, Fallot Tetralogy

Intervention / Treatment
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* Other: Non intervention


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients aged 18 years or older with repaired tetralogy of Fallot or double outlet right ventricle Fallot type Exclusion Criteria: Patients with pathologies that may interfere with the determination of the extracellular volume of myocardium (ischemic heart disease, storage diseases). Patients with pathologies that affect collagen metabolism (liver cirrhosis, stage ≥4 renal insufficiency, pulmonary fibrosis, metabolic bone disease, connective tissue diseases, active neoplasms, active treatment with corticosteroids and bone fractures or surgery in the previous 6 months). Pregnancy. Denial of informed consent. Patients with claustrophobia and pacemakers or defibrillators.

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patients<br>Patients with Tetralogy of Fallot | Other: Non intervention<br>* Patients without intervention<br>* Other names: Patients without intervention;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Correlation between myocardial fibrosis biomarkers and a composite of cardiac adverse events (cardiovascular death, sudden cardiac death, near-miss sudden death, supraventricular arrhythmias, ventricular arrhythmias, heart failure). | Myocardial fibrosis biomarkers: Cardiac Magnetic Resonance T1-mapping (extracellular volume fraction) and serum collagen turnover biomarkers (C-terminal propeptide of type I procollagen, C-terminal Telopeptide of type I Collagen, Matrix Metalloproteinase 1 and Tissue Inhibitor of Metalloproteinases-1) | 4 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Correlation between myocardial fibrosis biomarkers and prior cardiac events (near-miss sudden death, supraventricular arrhythmias, ventricular arrhythmias and heart failure admissions). | Myocardial fibrosis biomarkers: Cardiac Magnetic Resonance T1-mapping (extracellular volume fraction) and serum collagen turnover biomarkers (C-terminal propeptide of type I procollagen, C-terminal Telopeptide of type I Collagen, Matrix Metalloproteinase 1 and Tissue Inhibitor of Metalloproteinases-1) | up to time of reparative surgery | 
| Correlation between myocardial fibrosis assessed by cardiac magnetic resonance (T1-mapping) and other cardiac magnetic resonance parameters (ventricular volumes, ejection fraction and strain). | Correlation of myocardial fibrosis assessed by cardiac magnetic resonance T1-mapping (extracellular volume fraction) and other cardiac magnetic resonance parameters (ventricular volumes, ejection fraction and strain). | Baseline | 
| Correlation between serum collagen turnover biomarkers and cardiac magnetic resonance parameters (ventricular volumes, ejection fraction and strain) | Correlation between serum collagen turnover biomarkers (C-terminal propeptide of type I procollagen, C-terminal Telopeptide of type I Collagen, Matrix Metalloproteinase 1 and Tissue Inhibitor of Metalloproteinases-1) and cardiac magnetic resonance parameters (ventricular volumes, ejection fraction and strain). | Baseline | 
| Correlation between myocardial fibrosis assessed by cardiac magnetic resonance (T1-mapping) and by serum collagen turnover biomarkers. | Correlation of myocardial fibrosis assessed by cardiac magnetic resonance T1-mapping (extracellular volume fraction) and by serum collagen turnover biomarkers (serum collagen turnover biomarkers (C-terminal propeptide of type I procollagen, C-terminal Telopeptide of type I Collagen, Matrix Metalloproteinase 1 and Tissue Inhibitor of Metalloproteinases-1). | Baseline | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Fallot Tetralogy, Myocardial fibrosis, T1-mapping, Collagen turnover biomarkers