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Left ventricular myocardial function in myotonic dystrophy

L Badano1, C Autore, P V Fragola

  • 1Servizio di Cardiologia, Ospedale di Nervi, Genoa, Italy.

The American Journal of Cardiology
|April 15, 1993
PubMed
Summary
This summary is machine-generated.

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This study found no evidence of preclinical left ventricular dysfunction in myotonic dystrophy patients without heart failure symptoms. Load-independent function indexes were similar between patients and controls, regardless of disease severity or conduction disturbances.

Area of Science:

  • Cardiology
  • Neuromuscular Diseases
  • Echocardiography

Background:

  • Myotonic dystrophy is an autosomal dominant disease impacting multiple organ systems, notably the heart.
  • Cardiac conduction abnormalities are common, but overt heart failure is rare.
  • Prevalence of preclinical left ventricular (LV) dysfunction in asymptomatic myotonic dystrophy patients remains controversial.

Purpose of the Study:

  • To compare load-independent LV function indexes in myotonic dystrophy patients and healthy controls.
  • To investigate if disease severity or conduction disturbances influence LV function.

Main Methods:

  • M-mode echocardiography used to measure LV function in 43 myotonic dystrophy patients and 35 controls.
  • Load-independent indexes: LV ejection fraction predicted for end-systolic stress and end-systolic stress/volume index ratio.

Related Experiment Videos

  • Blood pressure measured using a cuff sphygmomanometer.

    • Main Results:

    • No significant difference in predicted LV ejection fraction or stress/volume index ratio between patients and controls.
    • LV myocardial function was similar across different disease severity groups (mild vs. moderate/severe).
    • No significant difference in LV function was observed between patients with and without conduction disturbances.

    Conclusions:

    • Asymptomatic patients with myotonic dystrophy do not exhibit preclinical left ventricular dysfunction.
    • LV myocardial function is preserved in myotonic dystrophy, irrespective of disease severity or cardiac conduction status.