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Review
. 2020 Oct 1;9(10):3186.
doi: 10.3390/jcm9103186.

Duchenne Dilated Cardiomyopathy: Cardiac Management from Prevention to Advanced Cardiovascular Therapies

Rachele Adorisio  1 , Erica Mencarelli  1 , Nicoletta Cantarutti  2 , Camilla Calvieri  2 , Liliana Amato  1 , Marianna Cicenia  2 , Massimo Silvetti  2 , Adele D'Amico  3 , Maria Grandinetti  1   4 , Fabrizio Drago  2 , Antonio Amodeo  1
Affiliations
Review

Duchenne Dilated Cardiomyopathy: Cardiac Management from Prevention to Advanced Cardiovascular Therapies

Rachele Adorisio et al. J Clin Med. .

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.

Keywords: dilated cardiomyopathy; duchenne muscular distrophy; heart failure.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Clinical features of Duchenne muscular distrophy cardiomyopathy (DMD-DCM). Panel (A): typical EKG with sinus tachycardia and tall R waves. Panel (B): parasternal long axis view of left ventricle (LV). Arrows indicate the presence of posterior wall aneurysm. Panel (C): cardiac magnetic resonance: short axis view of the LV. Presence of a transmural late gadolinium enhancement pattern located at the infero-lateral wall (Courtesy of Dr. A. Secinaro).
Figure 2
Figure 2
Progression of DMD-DCM. According to clinical stage of the DMD-DCM, different strategy might be considered. ACE: Angiotensin Converting Enzyme; CRT: Cardiac Resyncronization Therapy; ICD: IntraCardiac Defibrillator; VAD: Ventricular Assist Device.
See this image and copyright information in PMC

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