In Duchenne muscular dystrophy (DMD), heart involvement leads to heart failure. Angiotensin-converting enzyme (ACE) inhibitors are routinely prescribed at around 10 years of age for prevention. A French retrospective study initiated by Prof. Karim Wahbi, Professor of Cardiology at the Cochin Hospital and the Institute of Myology (Paris), relating to a cohort of boys with DMD presenting normal left-ventricular function, has just demonstrated the efficacy of the ACE inhibitors on increasing life expectancy and reducing hospitalisation rates.
The ACE inhibitors reduce the risk of death and hospitalisation
This study relates to data for 576 patients with DMD, aged 8 to 13 years, included in the French DMD-Heart multicentre Registry, and who were diagnosed between January 1986 and October 2018. Eight neuromuscular consultation services in France participated (Lille, Marseille, Montpellier, Garches, Trousseau, Necker, Cochin and Pitié Salpêtrière in Paris).
Among the patients followed up, 390 were treated early and over the long term with ACE inhibitors and 186 were not.
- Analysing the data using the Cox model allows one to take into account the variability of the patients’ situations (period treated with ACE inhibitors, age, heart function, etc.) in analysing the survival data. It shows that the risk of death decreased 2.5 fold in patients taking ACE inhibitors: the study identified 53 deaths among the 390 treated patients versus 60 among the 186 untreated patients.
- Another type of analysis was performed to investigate a relationship between age-related data, laboratory data, clinical data and data related to patient care (ventilation/no ventilation, arthrodesis/no arthrodesis, etc.) on the one hand, and treatment or non-treatment with ACE inhibitors and period of said treatment on the other. It confirms that preventive treatment with ACE inhibitors reduces the risk of death and hospitalisation due to heart failure. On the other hand, this treatment does not reduce the risk of hospitalisation for acute respiratory failure.
