Cardioprotective medication in Duchenne muscular dystrophy: a single-centre cohort study

Duchenne’s muscular dystrophy (DMD) is a neuromuscular disorder characterised by progressive muscle wasting impacting mobility, ventilation and cardiac function. Associated neuromuscular cardiomyopathy remains a major cause of morbidity and mortality. We investigated the effects of cardioprotective medications (ACE-inhibitors(ACE-I), beta-blockers) on clinical outcomes in DMD patients.This was a retrospective cohort study (reference:2021/12469) of DMD patients at a tertiary centre between 1993-2001 screening the electronic records for demographics, comorbidities, medication, disease specific features, echocardiography, hospitalisations, and ventilator use.68 patients were identified aged 27.4(6.6) years, of which 52 were still alive. There was a difference in body mass index between survivors and deceased patients (23.8(5.9) vs 19.9(3.8)kg/m2; p=0.03). Home mechanical ventilation (HMV) was required in 90% of patients, 85% had DMD associated cardiomyopathy. About 2/3 of all hospitalisations during the observation period were secondary to cardiopulmonary causes. The left ventricular ejection fraction (LVEF) at initial presentation was 44.8(10.6)% and declined by 3.3(95%CI 0.4 to -7)% over the follow up period (p=0.002). 61 patients were established on ACE-i for 75.9(35.1)%, and 62 were on beta-blockers for 73.6(33.5)% of the follow up period. There was a significant LVEF decline in those taking ACE-i for limited periods compared to those permanently on ACE-i (p=0.002); a ...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: 01.01 - Clinical problems - no related to asthma or COPD Source Type: research