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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
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Cardiac involvement over 10 years in myotonic and Becker muscular dystrophy and mitochondrial disorder

Josef Finsterer1, Claudia Stöllberger, Gerhard Blazek

  • 1Krankenanstalt Rudolfstiftung, Vienna, Austria. duarte@aonmail.at

International Journal of Cardiology
|January 30, 2007
PubMed

Insights

Cardiac involvement (CI) is definite in all myotonic dystrophy type 1 (MD), Becker muscular dystrophy (BMD), and mitochondrial myopathy (MMP) patients. While CI progresses significantly only in BMD patients over 10 years, early cardiac investigation is crucial for all.

Area of Science:

  • Cardiology
  • Neurology
  • Genetics

Background:

  • Long-term cardiac involvement (CI) outcomes in myotonic dystrophy type 1 (MD), Becker muscular dystrophy (BMD), and mitochondrial myopathy (MMP) are not well understood.
  • This study investigates the 10-year progression of CI in these patient groups.

Purpose of the Study:

  • To assess the progression of definite, possible, or absent cardiac involvement (CI) over 10 years.
  • Evaluated MD, BMD, and MMP patients using clinical history, physical examination, ECG, Holter monitoring, and echocardiography.

Main Methods:

  • 13 MD, 5 BMD, and 9 MMP patients were initially included.
  • Outcome measures included muscular disability score, CI classification, and abnormality scores.
  • Follow-up was conducted over 10 years, with final analysis on 11 MD, 3 BMD, and 4 MMP patients due to mortality and withdrawals.

Main Results:

  • Cardiac involvement (CI) was definite in most patients at baseline and 10 years.
  • ECG and echocardiography were the most frequently abnormal investigations.
  • Mean abnormality scores increased in MD and BMD patients, but decreased in MMP patients. 100% of BMD patients required cardiac medication.

Conclusions:

  • Cardiac involvement (CI) becomes definite in all MD, BMD, and MMP patients.
  • Significant CI progression was observed primarily in Becker muscular dystrophy (BMD) patients within 10 years.
  • Early cardiological evaluation and treatment are recommended upon neurological diagnosis for MD, BMD, and MMP patients, especially with symptomatic CI or severe abnormalities.
Abstract

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