Becker muscular dystrophy (BMD) is the result of a partial dystrophin deficiency. Approximately 40% of adults with BMD present dilated cardiomyopathy, including 15% of under 20-year-olds and 55% of over 40-year-olds. International cardiological guidelines recommend the prescription of angiotensin converting enzyme (ACE) inhibitors when the left ventricular ejection fraction (LVEF) drops below 40%.
A retrospective French study set up by Karim Wahbi, Professor of Cardiology at the Cochin Hospital and the Institute of Myology (Paris), relating to a cohort of adult patients with BMD, has just recently demonstrated the benefit of using the ACE inhibitors well before this threshold is reached.
Earlier ACE inhibitor-based treatment improves cardiac status
The BMD-Heart register encompasses 183 patients with BMD, followed up in 4 Paris-based centres (Cochin Hospital, the Institute of Myology, Pitié-Salpêtrière Hospital in Paris and the Raymond Poincaré Hospital in Garches).
The study relates to 85 patients with BMD registered in this register, all over 18 years of age, with an LVEF of between 40% and 49% at the beginning of the follow up:
- 34 of these patients (group 1) received an ACE inhibitor treatment once LVEF had dropped to 40%;
- for the other 51 patients (group 2), the treatment was initiated earlier, for LVEF lower than 50%.
The clinical follow-up data show that 3.9% of patients treated earlier were hospitalised for heart failure (HF), whereas a 3 times greater percentage were hospitalised for HF in the group treated later (11.8% versus 3.9%).
Additionally, LVEF fell much more frequently and more quickly beneath the 35% threshold in the group treated later compared to the group treated earlier, with a mean age difference of three years.
These results indicate that the early initiation of an angiotensin converting enzyme inhibitor treatment in patients with Becker muscular dystrophy preserves the heart better.
