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Article Dans Une Revue European Heart Journal Année : 2021

Association between prophylactic angiotensin-converting enzyme inhibitors and overall survival in Duchenne muscular dystrophy—analysis of registry data

1 CRESS (U1153 / UMR_A 1125) - Centre for Research in Epidemiology and Statistics | Centre de Recherche Épidémiologie et Statistiques
2 Hôpital Necker - Enfants Malades [AP-HP]
3 END-ICAP - Handicap neuromusculaire : Physiopathologie, Biothérapie et Pharmacologies appliquées
4 MMG - Marseille medical genetics - Centre de génétique médicale de Marseille
5 Département de génétique médicale [Hôpital de la Timone - APHM]
6 Clinique de pneumologie [CHRU Lille]
7 PhyMedExp - Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046]
8 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
9 CHU Trousseau [APHP]
10 URePSSS - Unité de Recherche Pluridisciplinaire Sport, Santé, Société (URePSSS) - ULR 7369 - ULR 4488
11 Neurologie, maladies neuro-musculaires [Hôpital de la Timone - APHM]
12 CHU Pitié-Salpêtrière [AP-HP]
13 Hôpital Cochin [AP-HP]
14 Centre de recherche en Myologie – U974 SU-INSERM
15 FILNEMUS - Filière Neuromusculaire
16 2I - Infection et inflammation
17 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
18 UM - Université de Montpellier
19 CHV - Centre Hospitalier de Versailles André Mignot
20 INM - Institut des Neurosciences de Montpellier
21 MASCOT (UMR_S_942 / U942) - Marqueurs cardiovasculaires en situation de stress
22 CIC 1429 - Centre d’Investigation Clinique 1429 [Garches]
23 Service de cardiologie Pédiatrique [Marseille]
24 Hôpital Raymond Poincaré [AP-HP]
25 PARCC (UMR_S 970/ U970) - Paris-Centre de Recherche Cardiovasculaire
Djillali Annane
Damien Bonnet
Guillaume Bassez

Résumé

Aims: To estimate the effect of prophylactic angiotensin-converting enzyme inhibitors (ACEi) on survival in Duchenne muscular dystrophy (DMD). Methods and results: We analysed the data from the French multicentre DMD Heart Registry (ClinicalTrials.gov: NCT03443115). We estimated the association between the prophylactic prescription of ACEi and event-free survival in 668 patients aged 8 to 13 years, with normal left ventricular function, using (i) a Cox model with intervention as a time-dependent covariate, (ii) a propensity-based analysis comparing ACEi treatment vs. no treatment, and (iii) a set of sensitivity analyses. The study outcomes were overall survival and hospitalizations for heart failure (HF) or acute respiratory failure. Among the 668 patients included in the DMD Heart Registry, 576 (mean age 6.1 ± 2.8 years) were eligible for this study, of whom 390 were treated with ACEi prophylactically. Death occurred in 53 patients (13.5%) who were and 60 patients (32.3%) who were not treated prophylactically with ACEi, respectively. In a Cox model with intervention as a time-dependent variable, the hazard ratio (HR) associated with ACEi treatment was 0.49 [95% confidence interval (CI) 0.34-0.72] and 0.47 (95% CI 0.31-0.17) for overall mortality after adjustment for baseline variables. In the propensity-based analysis, 278 patients were included in the treatment group and 834 in the control group, with 18.5% and 30.4% 12-year estimated probability of death, respectively. ACEi were associated with a lower risk of death (HR 0.39; 95% CI 0.17-0.92) and hospitalization for HF (HR 0.16; 95% CI 0.04-0.62). All other sensitivity analyses yielded similar results. Conclusion: Prophylactic ACEi treatment in DMD was associated with a significantly higher overall survival and lower rates of hospitalization for HF.
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Dates et versions

hal-03179750 , version 1 (25-03-2021)

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Raphaël Porcher, Isabelle Desguerre, Helge Amthor, Brigitte Chabrol, Frédérique Audic, et al.. Association between prophylactic angiotensin-converting enzyme inhibitors and overall survival in Duchenne muscular dystrophy—analysis of registry data. European Heart Journal, 2021, ⟨10.1093/eurheartj/ehab054⟩. ⟨hal-03179750⟩
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