Clinical trial NCT04737135: Myocardial FIbrosis in Repaired Tetralogy of FAllot- FIFA Study)
Official title: 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)
Study type: OBSERVATIONAL
Overall status: UNKNOWN
Conditions: Congenital Heart Disease
Congenital Heart Defect
Fallot Tetralogy
Keywords: Fallot Tetralogy
Myocardial fibrosis
T1-mapping
Collagen turnover biomarkers
Lead sponsor: Hospital Universitari Vall d'Hebron Research Institute
Sponsor class: OTHER
First posted: 2021-02-03
Enrollment: 224
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: PROBABILITY_SAMPLE
Study population: Patients aged 18 years or older with repaired tetralogy of Fallot or double outlet right ventricle Fallot type

Brief summary: 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: 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.

Eligibility criteria: 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.

Primary outcomes:
measure: 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).
timeFrame: 4 years
Secondary outcomes:
measure: Correlation between myocardial fibrosis biomarkers and prior cardiac events (near-miss sudden death, supraventricular arrhythmias, ventricular arrhythmias and heart failure admissions).
timeFrame: up to time of reparative surgery
measure: Correlation between myocardial fibrosis assessed by cardiac magnetic resonance (T1-mapping) and other cardiac magnetic resonance parameters (ventricular volumes, ejection fraction and strain).
timeFrame: Baseline
measure: Correlation between serum collagen turnover biomarkers and cardiac magnetic resonance parameters (ventricular volumes, ejection fraction and strain)
timeFrame: Baseline
measure: Correlation between myocardial fibrosis assessed by cardiac magnetic resonance (T1-mapping) and by serum collagen turnover biomarkers.
timeFrame: Baseline
Other outcomes:
Locations:
city: Barcelona
country: Spain
facility: Hospital Universitari Valle de Hebron
city: Barcelona
country: Spain
facility: Hospital Clínic i Provincial de Barcelona
city: Madrid
country: Spain
facility: Hospital General Universitario Gregorio Marañón
city: Madrid
country: Spain
facility: Hospital Universitario 12 de Octubre
city: Madrid
country: Spain
facility: Hospital Universitario La Paz
city: Sevilla
country: Spain
facility: Hospital Universitario Virgen del Rocío
city: Valencia
country: Spain
facility: Hospital Universitario y Politécnico La Fe
Study officials:
References:
citation: Oliver JM, Gallego P, Gonzalez AE, Garcia-Hamilton D, Avila P, Yotti R, Ferreira I, Fernandez-Aviles F. Risk factors for excess mortality in adults with congenital heart diseases. Eur Heart J. 2017 Apr 21;38(16):1233-1241. doi: 10.1093/eurheartj/ehw590.
citation: Stefanescu Schmidt AC, DeFaria Yeh D, Tabtabai S, Kennedy KF, Yeh RW, Bhatt AB. National Trends in Hospitalizations of Adults With Tetralogy of Fallot. Am J Cardiol. 2016 Sep 15;118(6):906-911. doi: 10.1016/j.amjcard.2016.06.034. Epub 2016 Jun 27.
citation: Puntmann VO, Peker E, Chandrashekhar Y, Nagel E. T1 Mapping in Characterizing Myocardial Disease: A Comprehensive Review. Circ Res. 2016 Jul 8;119(2):277-99. doi: 10.1161/CIRCRESAHA.116.307974.
citation: Chen CA, Tseng WY, Wang JK, Chen SY, Ni YH, Huang KC, Ho YL, Chang CI, Chiu IS, Su MY, Yu HY, Lin MT, Lu CW, Wu MH. Circulating biomarkers of collagen type I metabolism mark the right ventricular fibrosis and adverse markers of clinical outcome in adults with repaired tetralogy of Fallot. Int J Cardiol. 2013 Sep 10;167(6):2963-8. doi: 10.1016/j.ijcard.2012.08.059. Epub 2012 Sep 19.