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Sequential changes in cardiac structure and function in patients with Duchenne type muscular dystrophy: a
1Second Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan.
American Heart Journal
|June 18, 1998
Summary
Heart failure accelerates cardiac impairment in Duchenne muscular dystrophy (DMD) patients. Two-dimensional echocardiography aids early diagnosis of left ventricular dysfunction in DMD, guiding treatment.
Area of Science:
- Cardiology
- Neuromuscular Disorders
Background:
- Duchenne muscular dystrophy (DMD) often leads to respiratory failure, but some patients experience fatal heart failure.
- Cardiac impairment progression varies significantly among DMD patients.
Purpose of the Study:
- To investigate the long-term progression of cardiac impairment in Duchenne muscular dystrophy patients.
- To assess the utility of two-dimensional echocardiography in monitoring DMD-related cardiac changes.
Main Methods:
- Longitudinal follow-up of 74 DMD patients over 4 years using two-dimensional echocardiography.
- Classification of patients into four groups based on the Swinyard-Dever system.
- Echocardiographic evaluation of 22 additional DMD patients within one year of death.
Main Results:
- Progressive left ventricular expansion and regional wall motion abnormalities (posterior wall, apex) observed over 4 years.
- Myocardial dysfunction correlated with disease severity (Swinyard-Dever stage) in most patients.
- Severe left ventricular dilation and circumferential wall motion impairment noted in patients who died of heart failure.
Conclusions:
- Accelerated myocardial impairment is characteristic of DMD patients succumbing to heart failure.
- Two-dimensional echocardiography is valuable for early detection of left ventricular dysfunction in DMD.
- Echocardiographic findings provide crucial information for managing cardiac complications in DMD.