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Therapy Insight: cardiovascular complications associated with muscular dystrophies

Nature Clinical Practice Cardiovascular Medicine volume 2, pages 301–308 (2005)Cite this article

Abstract

The muscular dystrophies are commonly associated with cardiovascular complications, including cardiomyopathy and cardiac arrhythmias. These complications are caused by intrinsic defects in cardiomyocyte and cardiac conduction system function, and by the presence of severe skeletal muscle disease, which also contributes to cardiac dysfunction. Unlike the skeletal muscle degenerative process, for which treatment options are currently limited, therapy is available for the cardiovascular complications that accompany muscular dystrophy. New therapies for skeletal muscle degeneration are moving into clinical trials and, ultimately, into clinical practice. These therapies are expected to also improve the cardiac function, longevity and wellbeing of muscular dystrophy patients.

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Figure 1: Membrane-associated proteins in skeletal myofibers.
Figure 2: Muscle regeneration.

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Acknowledgements

EMM is supported by the Muscular Dystrophy Association, the NIH and the Burroughs Wellcome Fund.

Author information

Authors and Affiliations

  1. Departments of Medicine and Human Genetics,

    Elizabeth M McNally & Heather MacLeod

  2. Department of Medicine, at the University of Chicago, IL, USA

    Elizabeth M McNally & Heather MacLeod

Authors
  1. Elizabeth M McNally
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  2. Heather MacLeod
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Corresponding author

Correspondence to Elizabeth M McNally.

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The authors declare no competing financial interests.

Glossary

DYSTROPHIN–GLYCOPROTEIN COMPLEX

A macromolecular complex important for membrane stability and interactions with the extracellular matrix

SARCOGLYCAN

The sarcoglycan subunits α, β, γ and δ are part of the dystrophin–glycoprotein complex

TELETHONIN

Mutations in telethonin lead to muscular dystrophy; together with titin, telethonin is thought to be important for passive stretch in cardiomyocytes

TITIN

A giant protein that spans the length of the sarcomere and is thought to be important for elastic recoil of muscle

MYOTILIN

A protein found at the Z band; mutations in myotilin lead to muscular dystrophy

FUKUTIN RELATED PROTEIN

A protein thought to have glyosylation functions that are important for membrane-matrix interactions

KYPHOSCOLIOSIS

A combination of backward (kyphosis) and lateral (scoliosis) curvature of the spinal column

HEREGULIN

An extracellular matrix protein that is involved in neuromuscular junction function

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McNally, E., MacLeod, H. Therapy Insight: cardiovascular complications associated with muscular dystrophies. Nat Rev Cardiol 2, 301–308 (2005). https://doi.org/10.1038/ncpcardio0213

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