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Published on: April 25, 2014
Cardiomyopathy in Becker muscular dystrophy--does regional fibrosis mimic infarction?
Colin J Petrie1, Patrick B Mark, Henry J Dargie
1Department of Cardiology, Western Infirmary, Glasgow, UK. colinjamespetrie@yahoo.co.uk
Insights
Becker muscular dystrophy can cause severe heart failure. Cardiac MRI may show patterns mimicking heart attacks due to myocardial fibrosis, even without coronary artery disease.
Area of Science:
- Cardiology
- Genetics
- Biomedical Imaging
Background:
- Becker muscular dystrophy is an X-linked genetic disorder causing progressive muscle weakness.
- Cardiomyopathy is a common and serious complication of Becker muscular dystrophy.
- Congestive cardiac failure necessitates accurate diagnostic imaging for appropriate management.
Observation:
- A 39-year-old man with Becker muscular dystrophy presented with severe congestive cardiac failure.
- Cardiac magnetic resonance imaging (CMR) demonstrated subendocardial late gadolinium enhancement.
- This pattern of enhancement is typically associated with myocardial infarction.
Findings:
- The patient had no risk factors for atherosclerotic coronary artery disease.
- Coronary angiography revealed normal coronary arteries, ruling out obstructive coronary artery disease.
- The observed CMR findings are proposed to represent regional subendocardial myocardial fibrosis characteristic of Becker muscular dystrophy-associated cardiomyopathy.
Implications:
- Subendocardial fibrosis in Becker muscular dystrophy cardiomyopathy can mimic myocardial infarction on contrast-enhanced CMR.
- This distinction is crucial for accurate diagnosis and avoiding unnecessary invasive procedures.
- Understanding these imaging characteristics can improve the diagnostic pathway for patients with genetic myopathies.
Abstract:
We present a case of a 39-year-old man with Becker muscular dystrophy and severe congestive cardiac failure. Cardiac magnetic resonance imaging revealed subendocardial late gadolinium enhancement, similar to that seen in myocardial infarction. He had no risk factors for atherosclerotic coronary artery disease and coronary angiography was normal. We propose that regional subendocardial myocardial fibrosis which has been described histologically in the cardiomyopathy associated with Becker muscular dystrophy may resemble previous infarction at contrast enhanced cardiac magnetic resonance imaging.
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Myocarditis I: Introduction
Myocarditis II: Clinical Features and Diagnostic Tests
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