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Related Experiment Videos

Left ventricular diastolic function in congenital myotonic dystrophy.

F A Bu'Lock1, M Sood, J V De Giovanni

  • 1Department of Cardiology, Birmingham Children's Hospital, UK.

Archives of Disease in Childhood
|May 18, 1999
PubMed
Summary

Young patients with myotonic dystrophy exhibit diastolic dysfunction and electrophysiological abnormalities. Further research is needed to understand the prognostic impact of these cardiac findings in myotonic dystrophy.

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Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Myotonic dystrophy (DM) is a multisystem disorder.
  • Cardiac involvement is a significant cause of morbidity and mortality in DM.
  • Left ventricular (LV) function and conduction abnormalities are common in DM.

Purpose of the Study:

  • To evaluate left ventricular (LV) function and conduction abnormalities in pediatric patients with myotonic dystrophy (DM).
  • To compare echocardiographic parameters with age-matched controls.

Main Methods:

  • Twelve patients with DM underwent electrocardiography and echocardiography.
  • Echocardiographic parameters were compared to body surface area (BSA)-matched normal values.

Main Results:

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  • Mildly reduced fractional shortening and mitral valve prolapse were observed in some patients.
  • Diastolic dysfunction was evident, with prolonged isovolumic relaxation time (IVRT) and early filling duration.
  • Reduced heart rate and common conduction abnormalities were noted, but without clear correlation to diastolic dysfunction.

Conclusions:

  • Pediatric patients with myotonic dystrophy demonstrate myocardial diastolic dysfunction and electrophysiological abnormalities.
  • The long-term prognostic significance of these cardiac findings in myotonic dystrophy warrants further investigation.