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Left ventricular hypertrabeculation/noncompaction in hereditary inclusion body myopathy
International Journal of Cardiology
|November 11, 2009
Summary
Left ventricular hypertrabeculation (LVHT), a rare heart condition, was unexpectedly found in a patient with hereditary inclusion body myopathy (IBM). This case suggests cardiac screening is vital for IBM patients.
Area of Science:
- Cardiology
- Neurology
- Genetics
Background:
- Hereditary inclusion body myopathy (IBM) is a progressive muscle disorder.
- Left ventricular hypertrabeculation (LVHT), or noncompaction, is a rare congenital cardiomyopathy.
- The association between IBM and LVHT has not been previously documented.
Observation:
- A 62-year-old male with symptoms suggestive of hereditary IBM presented with muscle stiffness, cramps, and elevated creatine kinase.
- Cardiac evaluation revealed hypertension, left anterior hemiblock, mild myocardial thickening, and isolated LVHT.
- Neurological examination and EMG were normal, but muscle biopsy confirmed hereditary IBM.
Findings:
- The patient was diagnosed with hereditary IBM and incidentally found to have LVHT.
- LVHT in this case was not associated with arrhythmias, systolic dysfunction, or embolic events.
- No specific neurological or cardiac therapy was required, and no progression was noted over 18 months.
Implications:
- This case highlights that LVHT can occur in patients with hereditary IBM.
- It underscores the importance of cardiac screening in individuals diagnosed with hereditary IBM.
- Cardiac involvement in IBM may present as myocardial damage or conduction abnormalities.
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