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Mechanical Control of Relaxation Using Intact Cardiac Trabeculae
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Left ventricular hypertrabeculation/noncompaction associated with coronary heart disease and myopathy
International Journal of Cardiology
|April 4, 2009
Summary
Left ventricular hypertrabeculation (LVHT), a rare heart condition, can be associated with coronary heart disease and metabolic myopathy. This case highlights LVHT potentially isolated to the left ventricular apex.
Area of Science:
- Cardiology
- Neurology
- Genetics
Background:
- Left ventricular hypertrabeculation (LVHT), also known as left ventricular noncompaction, is a rare congenital heart defect.
- Associations with coronary heart disease and metabolic myopathies are infrequently reported.
- This report details a rare case with potential implications for understanding LVHT etiology.
Observation:
- A 77-year-old male with extensive cardiovascular history presented with worsening heart failure.
- Transthoracic echocardiography revealed LVHT.
- Neurological examination and elevated creatine-kinase suggested an underlying metabolic myopathy.
Findings:
- Autopsy confirmed severe coronary heart disease and LVHT predominantly in the left ventricular apex.
- The patient exhibited symptoms consistent with a metabolic myopathy, including ptosis, hypacusis, and muscle weakness.
- Recurrently elevated creatine-kinase levels, with normal troponin, further supported myopathy.
Implications:
- LVHT may coexist with coronary heart disease and metabolic myopathies.
- LVHT might present with focal localization, specifically in the left ventricular apex.
- This case underscores the importance of a comprehensive diagnostic approach in patients with LVHT and multisystemic symptoms.
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