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J. Clin. Med. 2018, 7(9), 291; https://doi.org/10.3390/jcm7090291

Dystrophin Cardiomyopathies: Clinical Management, Molecular Pathogenesis and Evolution towards Precision Medicine

1
Dipartimento di Scienze Cardiovascolari e Toraciche, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Largo A. Gemelli 8, 00100 Rome, Italy
2
Centro Cardiologico Monzino-IRCCS, Unit of Vascular Biology and Regenerative Medicine, Via Carlo Parea 4, 20138 Milan, Italy
3
Department of Paediatric Cardiology and Cardiac Surgery, Bambino Gesù Hospital (OPBG), Piazza S. Onofrio 4, 00165 Rome, Italy
4
Department of Clinical Sciences and Community Health (DISCCO), University of Milan, Via Francesco Sforza 35, 20122 Milan, Italy
5
Department of Pathophysiology, University of Split School of Medicine, Soltanska 2, 21000 Split, Croatia
These authors had equal contributions.
*
Author to whom correspondence should be addressed.
Received: 3 August 2018 / Revised: 2 September 2018 / Accepted: 14 September 2018 / Published: 19 September 2018
(This article belongs to the Special Issue Hereditary Neuromuscular Diseases)
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Abstract

Duchenne’s muscular dystrophy is an X-linked neuromuscular disease that manifests as muscle atrophy and cardiomyopathy in young boys. However, a considerable percentage of carrier females are often diagnosed with cardiomyopathy at an advanced stage. Existing therapy is not disease-specific and has limited effect, thus many patients and symptomatic carrier females prematurely die due to heart failure. Early detection is one of the major challenges that muscular dystrophy patients, carrier females, family members and, research and medical teams face in the complex course of dystrophic cardiomyopathy management. Despite the widespread adoption of advanced imaging modalities such as cardiac magnetic resonance, there is much scope for refining the diagnosis and treatment of dystrophic cardiomyopathy. This comprehensive review will focus on the pertinent clinical aspects of cardiac disease in muscular dystrophy while also providing a detailed consideration of the known and developing concepts in the pathophysiology of muscular dystrophy and forthcoming therapeutic options. View Full-Text
Keywords: cardiomyopathy; dilated; cardiomyopathy in muscular dystrophy; Duchenne muscular dystrophy; dystrophin; heart failure; personalized medicine cardiomyopathy; dilated; cardiomyopathy in muscular dystrophy; Duchenne muscular dystrophy; dystrophin; heart failure; personalized medicine
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D’Amario, D.; Gowran, A.; Canonico, F.; Castiglioni, E.; Rovina, D.; Santoro, R.; Spinelli, P.; Adorisio, R.; Amodeo, A.; Perrucci, G.L.; Borovac, J.A.; Pompilio, G.; Crea, F. Dystrophin Cardiomyopathies: Clinical Management, Molecular Pathogenesis and Evolution towards Precision Medicine. J. Clin. Med. 2018, 7, 291.

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