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Cardiac dysfunction in patients with chronic progressive external ophthalmoplegia
1First Department of Internal Medicine, School of Medicine, University of Tokushima, Japan.
Clinical Cardiology
|March 1, 1997
Summary
Left ventricular dysfunction is present in patients with chronic progressive external ophthalmoplegia (CPEO), a mitochondrial disorder. This finding suggests cardiac involvement warrants greater attention in CPEO patient management.
Area of Science:
- Cardiology
- Mitochondrial Medicine
- Genetics
Background:
- Chronic progressive external ophthalmoplegia (CPEO) is a mitochondrial disorder characterized by large mitochondrial DNA deletions.
- While mtDNA deletions are linked to dilated cardiomyopathy, cardiac involvement in CPEO was historically believed to be confined to the conduction system.
Purpose of the Study:
- To investigate and evaluate left ventricular function in patients diagnosed with CPEO.
Main Methods:
- Cardiac function assessment in five CPEO patients.
- Utilized carotid pulse recording and Doppler echocardiography for evaluation.
Main Results:
- Two patients exhibited increased pre-ejection period to ejection time ratio, left ventricular dilatation, and hypokinetic wall motion.
- Reduced left ventricular fractional shortening and circumferential shortening rates were observed.
- One patient died of congestive heart failure; remaining patients showed impaired diastolic filling patterns.
Conclusions:
- Left ventricular dysfunction is a potential complication in CPEO patients.
- Cardiac involvement in CPEO may extend beyond the conduction system.
- Increased clinical attention to cardiac function is recommended for managing CPEO patients.