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A current approach to heart failure in Duchenne muscular dystrophy

Heart (British Cardiac Society), 2017-11, Vol.103 (22), p.1770-1779 [Peer Reviewed Journal]

Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted. ;Copyright: 2017 © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted. ;ISSN: 1355-6037 ;EISSN: 1468-201X ;DOI: 10.1136/heartjnl-2017-311269 ;PMID: 28668906

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Title:
A current approach to heart failure in Duchenne muscular dystrophy
Author: D’Amario, Domenico ; Amodeo, Antonio ; Adorisio, Rachele ; Tiziano, Francesco Danilo ; Leone, Antonio Maria ; Perri, Gianluigi ; Bruno, Piergiorgio ; Massetti, Massimo ; Ferlini, Alessandra ; Pane, Marika ; Niccoli, Giampaolo ; Porto, Italo ; D’Angelo, Gianluca A ; Borovac, Josip Anđelo ; Mercuri, Eugenio ; Crea, Filippo
Subjects: Age ; Animals ; Biopsy ; Cardiomyopathy ; Cardiomyopathy, Dilated - diagnosis ; Cardiomyopathy, Dilated - genetics ; Cardiomyopathy, Dilated - physiopathology ; Cardiomyopathy, Dilated - therapy ; Disease Models, Animal ; Disease Progression ; Enzymes ; Genetic Predisposition to Disease ; Heart failure ; Heart Failure - diagnosis ; Heart Failure - genetics ; Heart Failure - physiopathology ; Heart Failure - therapy ; Heart Function Tests ; Humans ; Kinases ; Muscular dystrophy ; Muscular Dystrophy, Duchenne - diagnosis ; Muscular Dystrophy, Duchenne - genetics ; Muscular Dystrophy, Duchenne - physiopathology ; Muscular Dystrophy, Duchenne - therapy ; Mutation ; Phenotype ; Predictive Value of Tests ; Prognosis ; Proteins ; Risk Factors ; Rodents ; Signal transduction ; Transplants & implants
Is Part Of: Heart (British Cardiac Society), 2017-11, Vol.103 (22), p.1770-1779
Description: Duchenne muscular dystrophy (DMD) is a genetic, progressive neuromuscular condition that is marked by the long-term muscle deterioration with significant implications of pulmonary and cardiac dysfunction. As such, end-stage heart failure (HF) in DMD is increasingly becoming the main cause of death in this population. The early detection of cardiomyopathy is often challenging, due to a long subclinical phase of ventricular dysfunction and difficulties in assessment of cardiovascular symptomatology in these patients who usually loose ambulation during the early adolescence. However, an early diagnosis of cardiovascular disease in patients with DMD is decisive since it allows a timely initiation of cardioprotective therapies that can mitigate HF symptoms and delay detrimental heart muscle remodelling. Echocardiography and ECG are standardly used for screening and detection of cardiovascular abnormalities in these patients, although these tools are not always adequate to detect an early, clinically asymptomatic phases of disease progression. In this regard, cardiovascular magnetic resonance (CMR) with late gadolinium enhancement is emerging as a promising method for the detection of early cardiac involvement in patients with DMD. The early detection of cardiac dysfunction allows the therapeutic institution of various classes of drugs such as corticosteroids, beta-blockers, ACE inhibitors, antimineralocorticoid diuretics and novel pharmacological and surgical solutions in the multimodal and multidisciplinary care for this group of patients. This review will focus on these challenges and available options for HF in patients with DMD.
Publisher: England: BMJ Publishing Group LTD
Language: English
Identifier: ISSN: 1355-6037
EISSN: 1468-201X
DOI: 10.1136/heartjnl-2017-311269
PMID: 28668906
Source: MEDLINE
ProQuest Central

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A current approach to heart failure in Duchenne muscular dystrophy

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