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Multicenter Study
. 2021;8(4):495-502.
doi: 10.3233/JND-200620.

Improved Cardiac Outcomes by Early Treatment with Angiotensin-Converting Enzyme Inhibitors in Becker Muscular Dystrophy

Caroline Stalens  1   2 , Leslie Motté  3 , Anthony Béhin  4 , Rabah Ben Yaou  4 , France Leturcq  5 , Guillaume Bassez  4 , Pascal Laforêt  6 , Bertrand Fontaine  4 , Stéphane Ederhy  7 , Marion Masingue  4 , Malika Saadi  3 , Sarah Leonard Louis  4 , Nawal Berber  4 , Tanya Stojkovic  4 , Denis Duboc  3   8 , Karim Wahbi  3   8   9
Affiliations
Multicenter Study

Improved Cardiac Outcomes by Early Treatment with Angiotensin-Converting Enzyme Inhibitors in Becker Muscular Dystrophy

Caroline Stalens et al. J Neuromuscul Dis. 2021.

Abstract

Background: The latest practice guidelines from the American College of Cardiology/American Heart Association recommend the prescription of an ACE-i for patients presenting with non-ischemic cardiomyopathy when left ventricular ejection fraction (LVEF) falls below 40%.

Objective: To determine if the initiation of treatment with an angiotensin-converting enzyme inhibitor (ACE-i) earlier than recommended by practice guidelines issued by professional societies improves the long-term cardiac outcomes of patients presenting with Becker muscular dystrophy (MD) cardiomyopathy.

Methods: From a multicenter registry of Becker MD, we selected retrospectively patients presenting between January 1990 and April 2019 with a LVEF ≥40 and ≤49%. We used a propensity score analysis to compare the risk of a) hospitalization for management of heart failure (HF), and b) a decrease in LVEF to <35% in patients who received an ACE-i when LVEF fell below 40% (conventional treatment), versus below 50% (early treatment).

Results: From the 183 patients entered in our registry, we identified 85 whose LVEF was between 40 and 49%, 51 of whom received early and 34 received conventional ACE-i treatment. Among patients with early versus conventional treatments, 2 (3.9%) versus 4 (11.8%) were hospitalized for management of HF [hazard ratio (HR) 0.151; 95% confidence interval (CI) 0.028 to 0.822; p = 0.029], and 9 (17.6%) versus 10 (29.4%) had a decrease in LVEF below 35% (HR 0.290; 95% CI 0.121 to 0.694; p = 0.005).

Conclusions: The long-term cardiac outcome of patients presenting with Becker MD was significantly better when treatment with ACE-i was introduced after a decrease in LVEF below 50%, instead of below 40% as recommended in the current practice guidelines issued by professional societies.

Keywords: Becker muscular dystrophy; angiotensin-converting enzyme inhibitor; heart failure; inherited myopathy; non-ischemic cardiomyopathy.

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Conflict of interest statement

The authors have no conflict of interest to disclose.

Figures

Fig. 1
Fig. 1
Extraction of the study sample. ACE-i = angiotensin-converting enzyme inhibitor; LVEF = left ventricular ejection fraction.
Fig. 2
Fig. 2
Hospitalization for management of heart failure and decrease in left ventricular ejection fraction to <35% during follow-up. IPTW = inverse probability of treatment weighting; ACE-i = angiotensin-converting enzyme inhibitor.
See this image and copyright information in PMC

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