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Myostatin is elevated in congenital heart disease and after mechanical unloading

Lawrence T Bish1, Isaac George, Simon Maybaum

  • 1Department of Physiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, United States of America. bish@mail.med.upenn.edu

Plos One
|September 21, 2011
PubMed

Insights

Myostatin levels increase with worsening heart function in congenital heart disease (CHD). This suggests the myostatin/IGF-1 ratio may be a target for treating heart failure (HF) in these patients.

Area of Science:

  • Cardiovascular Research
  • Molecular Biology
  • Pediatric Cardiology

Background:

  • Myostatin negatively regulates skeletal muscle mass and is elevated in adult heart failure (HF).
  • The role of myostatin in congenital heart disease (CHD) has not been previously investigated.
  • Understanding myostatin's role in CHD is crucial for developing new therapeutic strategies.

Purpose of the Study:

  • To investigate myostatin and IGF-1 expression in pediatric patients with congenital heart disease (CHD).
  • To determine the relationship between myostatin/IGF-1 ratio and the severity of ventricular dysfunction in CHD.
  • To explore potential therapeutic targets for heart failure in CHD.

Main Methods:

  • Western blot analysis was used to measure myostatin and IGF-1 expression in cardiac tissue.
  • Myocardial biopsies were collected from four patient cohorts: adult normal, pediatric normal (RVOT), pediatric heart transplant (OHT), and pediatric BiVAD support.
  • Expression levels were correlated with the degree of myocardial dysfunction and biventricular support.

Main Results:

  • Myostatin expression was significantly elevated in pediatric patients with worsening heart failure (OHT and BiVAD groups) compared to controls.
  • A more than twofold increase in myostatin was observed in decompensated biventricular failure (BiVAD) compared to OHT and RVOT groups.
  • An elevated myostatin/IGF-1 ratio was strongly associated with increased ventricular dysfunction.

Conclusions:

  • Myostatin expression is upregulated in congenital heart disease (CHD) and correlates with disease severity.
  • The myostatin/IGF-1 ratio increases as ventricular function deteriorates in CHD patients.
  • Further research is warranted to evaluate the therapeutic potential of modulating the myostatin/IGF-1 ratio in heart failure.
Abstract

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