Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

Brian Feingold; William T Mahle; Scott Auerbach; Paula Clemens; Andrea A Domenighetti; John L Jefferies; Daniel P Judge; Ashwin K Lal; Larry W Markham; W James Parks; Takeshi Tsuda; Paul J Wang; Shi-Joon Yoo; American Heart Association Pediatric Heart Failure Committee of the Council on Cardiovascular Disease in the Young; Council on Clinical Cardiology; Council on Cardiovascular Radiology and Intervention; Council on Functional Genomics and Translational Biology; and Stroke Council

doi:10.1161/CIR.0000000000000526

Management of Cardiac Involvement Associated With Neuromuscular Diseases: A Scientific Statement From the American Heart Association

Circulation. 2017 Sep 26;136(13):e200-e231. doi: 10.1161/CIR.0000000000000526. Epub 2017 Aug 24.

Authors

Brian Feingold, William T Mahle, Scott Auerbach, Paula Clemens, Andrea A Domenighetti, John L Jefferies, Daniel P Judge, Ashwin K Lal, Larry W Markham, W James Parks, Takeshi Tsuda, Paul J Wang, Shi-Joon Yoo; American Heart Association Pediatric Heart Failure Committee of the Council on Cardiovascular Disease in the Young; Council on Clinical Cardiology; Council on Cardiovascular Radiology and Intervention; Council on Functional Genomics and Translational Biology; and Stroke Council

PMID: 28838934
DOI: 10.1161/CIR.0000000000000526

Abstract

For many neuromuscular diseases (NMDs), cardiac disease represents a major cause of morbidity and mortality. The management of cardiac disease in NMDs is made challenging by the broad clinical heterogeneity that exists among many NMDs and by limited knowledge about disease-specific cardiovascular pathogenesis and course-modifying interventions. The overlay of compromise in peripheral muscle function and other organ systems, such as the lungs, also makes the simple application of endorsed adult or pediatric heart failure guidelines to the NMD population problematic. In this statement, we provide background on several NMDs in which there is cardiac involvement, highlighting unique features of NMD-associated myocardial disease that require clinicians to tailor their approach to prevention and treatment of heart failure. Undoubtedly, further investigations are required to best inform future guidelines on NMD-specific cardiovascular health risks, treatments, and outcomes.

Keywords: AHA Scientific Statements; cardiomyopathy; muscular dystrophy; neuromuscular disease; pediatrics.

Publication types

Practice Guideline

MeSH terms

American Heart Association
Barth Syndrome / diagnosis
Barth Syndrome / genetics
Barth Syndrome / metabolism
Barth Syndrome / pathology
Cardiomyopathies / complications
Cardiomyopathies / diagnosis*
Cardiomyopathies / pathology
Friedreich Ataxia / diagnosis
Friedreich Ataxia / metabolism
Friedreich Ataxia / pathology
Humans
Muscular Diseases / diagnosis*
Muscular Diseases / metabolism
Muscular Diseases / pathology
Muscular Dystrophies, Limb-Girdle / diagnosis
Muscular Dystrophies, Limb-Girdle / metabolism
Muscular Dystrophies, Limb-Girdle / pathology
Muscular Dystrophy, Duchenne / diagnosis
Muscular Dystrophy, Duchenne / metabolism
Muscular Dystrophy, Duchenne / pathology
Muscular Dystrophy, Emery-Dreifuss / diagnosis
Muscular Dystrophy, Emery-Dreifuss / metabolism
Muscular Dystrophy, Emery-Dreifuss / pathology
Myopathies, Structural, Congenital / diagnosis
Myopathies, Structural, Congenital / genetics
Myopathies, Structural, Congenital / metabolism
Myopathies, Structural, Congenital / pathology
Myotonic Dystrophy / diagnosis
Myotonic Dystrophy / metabolism
Myotonic Dystrophy / pathology
Neuromuscular Diseases / complications
Neuromuscular Diseases / diagnosis*
Neuromuscular Diseases / pathology
Risk Factors
United States

Supplementary concepts

Myofibrillar Myopathy