Abstract
Duchenne muscular dystrophy (DMD) cardiomyopathy (DCM) is characterized by a hypokinetic, dilated phenotype progressively increasing with age. Regular cardiac care is crucial in DMD care. Early recognition and prophylactic use of angiotensin converting enzyme inhibitors (ACEi) are the main stay therapeutic strategy to delay incidence of DMD-DCM. Pharmacological treatment to improve symptoms and left ventricle (LV) systolic function, have been widely implemented in the past years. Because of lack of DMD specific drugs, actual indications for established DCM include current treatment for heart failure (HF). This review focuses on current HF strategies to identify, characterize, and treat DMD-DCM.
Highlights
Dilated cardiomyopathy (DCM), arrhythmias, and congestive heart failure (HF) represent the most important life-limiting condition in Duchenne muscular dystrophy (DMD) [1–3].Routinely cardiovascular evaluation including echocardiography is recommended in the current 2018 DMD Care consideration sponsored by Centers of disease control and prevention [4]
We present the cardiologist perspective on current data regarding clinical management of DMD patients
The echocardiographic analysis on clinical course showed that the progression of the cardiomyopathy is slower in DMD when compared to Becker or other forms of cardiomyopathy
Summary
Dilated cardiomyopathy (DCM), arrhythmias, and congestive heart failure (HF) represent the most important life-limiting condition in Duchenne muscular dystrophy (DMD) [1–3]. Cardiovascular evaluation including echocardiography is recommended in the current 2018 DMD Care consideration sponsored by Centers of disease control and prevention [4]. HF treatments have evolved tremendously since 1980s and the armamentarium of adult HF specialists has been enriched with new drugs and the use of device (i.e., cardiac resynchronization therapy, intracardiac defibrillator, and ventricular assist device) before cardiac transplant. DMD patients are not usually candidate for heart transplantation because of the progressive skeletal myopathy, limited functional capacity [5], and shortage of donor availability. We present the cardiologist perspective on current data regarding clinical management of DMD patients
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