Perindopril preventive treatment on mortality in Duchenne muscular dystrophy: 10 years' follow-up

Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management

“…Respiratory interventions, in particular the institution of nocturnal ventilation, have had a major effect on survival in DMD, 65,66 and early indications are that prompt recognition and treatment of deterioration in cardiac status will also have a significant impact. 119,121 Further trials are awaited to determine the optimum timing to start cardioactive treatment. In the meantime, the recommendations presented here are consistent with previously published guidelines, 113,115 and most importantly reinforce the need for active engagement with a cardiologist at every stage of the condition.…”

“…Further studies are awaited to allow firm recommendations to be made. 108,[119][120][121][122][123] Signs or symptoms of abnormalities of cardiac rhythm should be promptly investigated with Holter or event monitor recording and should be treated. [124][125][126][127] Sinus tachycardia is common in DMD, but is also noted in systolic dysfunction.…”

Cardiac involvement in children with neuro-muscular disorders

“…Данные 10-летнего наблюдения указывают на более низкий уровень смертности среди пациентов, которым согласно рандомизированному отбору были изначально назначены ингибиторы ангиотензинпревращающего фермента (иАПФ) [27]. Увеличение выживаемости по сравнению с данными литературы отмечено в иссле-дованиях у пациентов с МДД и МДБ, получавших комбинированную терапию иАПФ и ß-блокаторами.…”

Cardiac Management of Ventilator-Assisted Individuals with Duchenne Muscular Dystrophy

“…16 Much of the existing data regarding prevalence and severity of cardiomyopathy was before ventilatory support was common for this group of patients, 7 and the literature regarding the pharmacological management of cardiomyopathy in DMD involves predominantly children and teenagers. 10,[12][13][14] Patients are now living into their 4th and 5th decades but to date there is little information about this group. One recent observational study, involving teenagers and young adults (mean age of 22 years), requiring ventilatory support reported a lower prevalence and progression of cardiomyopathy compared to other studies 17 raising a question regarding the common assumption that it was almost inevitable.…”

“…Pharmacological treatment, including angiotensin-converting enzyme (ACE) inhibitors with or without b blockers, for asymptomatic and symptomatic cardiomyopathy in DMD reduces both morbidity and mortality. 10,[12][13][14] 'Failure to see a cardiac specialist until late in the disease, after clinical manifestations of cardiac dysfunction are evident, have led to late treatment and poor outcomes'. 15 Regular assessment is thus necessary for early detection of asymptomatic cardiomyopathy in patients with DMD.…”