A current approach to heart failure in Duchenne muscular dystrophy

Francesco Danilo Tiziano, Piergiorgio Bruno, Massimo Massetti, Marika Pane, Eugenio Maria Mercuri, Filippo Crea, Domenico D'Amario, Antonio Amodeo, Antonio Maria Leone, Gianluigi Perri, Giampaolo Niccoli, Italo Porto, Rachele Adorisio, Alessandra Ferlini, Josip Anä Borovac

Research output: Contribution to journalArticle

25 Citations (Scopus)

Abstract

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.
Original languageEnglish
Pages (from-to)1770-1779
Number of pages10
JournalHeart
Volume103
DOIs
Publication statusPublished - 2017

Keywords

  • Animals
  • Cardiology and Cardiovascular Medicine
  • Cardiomyopathy, Dilated
  • Disease Models, Animal
  • Disease Progression
  • Genetic Predisposition to Disease
  • Heart Failure
  • Heart Function Tests
  • Humans
  • Muscular Dystrophy, Duchenne
  • Mutation
  • Phenotype
  • Predictive Value of Tests
  • Prognosis
  • Risk Factors
  • cardiomyopathy, dilated
  • heart failure
  • muscular dystrophy, Duchenne
  • therapeutics

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