BLOCKADE OF THE RENIN-ANGIOTENSIN-ALDOSTERONE SYSTEM IN PATIENTS WITH ARVD: A DOUBLE-BLIND MULTICENTRE PROSPECTIVE RANDOMIZED STUDY BRAVE

Arrhythmogenic right ventricular dysplasia

Right ventricular deterioration is defined by a decrease in RV longitudinal strain or/and increase of RV infundibulum diameter between baseline and 1 year of follow-up measured by echography. Ventricular arrhythmia is defined by the change in arrhythmia, defined as a number of occurrences of ventricular extrasystoles > 500 during a 24h-Holter ECG monitor test, between baseline and 1 year of follow-up

Ventricular arrhythmia is defined by number of ventricular extrasystoles during a 24h-Holter ECG monitor test, between baseline and 1 year of follow-up., Functional status is defined by presence/absence and number of palpitations, ventricular tachycardia, dyspnea, syncope, sudden death, thoracic pain, MACE, and hospital admissions owing to clinical deterioration during the 1 year of follow-up, LV function (LV diameters, LV volumes, LVEF, LVGLS, presence of aneurism, % of dyskinesia), size of RV (LVOT diameters - PLAX and SAX, end-diastolique area, end-systolique area, 3D volumes), RV function (fractional shortening, presence of aneurism , % dyskinesia, TAPSE, tissue Doppler velocity, 3D RVEF, lateral longitudinal strain, global longitudinal strain) on echography between baseline and 1 year , and evolution of QRS width and PR duration on ECG between baseline and 1 year, RV function (fractional shortening, presence of aneurism , % dyskinesia, TAPSE, tissue Doppler velocity, 3D RVEF, lateral longitudinal strain, global longitudinal strain) using echocardiography and arrhythmia burden using 24h Holter-ECG at 1 year of follow-up according to the genotype (No mutation versus, at least, one mutation on desmosome genes)., Quantification of fibrosis and inflammation (measured at baseline and 1-year of follow-up) by dosage of MMP9, TIMP1, TIMP2, IL6 and IL8, Ventricular arrhythmia is defined by number of ventricular extrasystoles during a 24h-Holter ECG monitor test, between baseline and 3 years of follow-up, Functional status is defined by presence/absence and number of palpitations, ventricular tachycardia, dyspnea, syncope, sudden death, thoracic pain, MACE, and hospital admissions owing to clinical deterioration during the 3 years of follow-up., Left Ventricle (LV) function (LV diameters, LV volumes, LVEF, LVGLS, presence of aneurism, % of dyskinesia), size of RV (LVOT diameters - PLAX and SAX, end-diastolique area, end-systolique area, 3D volumes), RV function (fractional shortening, presence of aneurism , % dyskinesia, TAPSE, tissue Doppler velocity, 3D RVEF, lateral longitudinal strain, global longitudinal strain) on echography between baseline and 3 years, and evolution of QRS width and PR duration on ECG between baseline and 3 year, RV function (fractional shortening, presence of aneurism , % dyskinesia, TAPSE, tissue Doppler velocity, 3D RVEF, lateral longitudinal strain, global longitudinal strain) using echocardiography and arrhythmia burden using 24h Holter-ECG at 3 years of follow-up according to the genotype (No mutation versus, at least, one mutation on desmosome genes)., Quantification of fibrosis and inflammation (measured at baseline and 3 years of follow-up) by dosage of MMP9, TIMP1, TIMP2, IL6 and IL8.

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