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

Rachele Adorisio; Erica Mencarelli; Nicoletta Cantarutti; Camilla Calvieri; Liliana Amato; Marianna Cicenia; Massimo Silvetti; Adele D'Amico; Maria Grandinetti; Fabrizio Drago; Antonio Amodeo

doi:10.3390/jcm9103186

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

J Clin Med. 2020 Oct 1;9(10):3186. doi: 10.3390/jcm9103186.

Affiliations

¹ Heart Failure Clinic-Heart Failure, Heart Transplant, Mechanical Circulatory Support Unit, Department of Pediatric Cardiology and Cardiac Surgery, Heart and Lung Transplant, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
² Pediatric Cardiology and Cardiac Arrhythmias/Syncope Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
³ Neuromuscolar Disease, Genetic and Rare Disease Research Area, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
⁴ Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A, Gemelli IRCCS, 20097 Rome, Italy.

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.

Publication types

Review