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Cardiac MRI in a patient with coincident left ventricular non-compaction and hypertrophic cardiomyopathy
Zahra Alizadeh-Sani1, Shabnam Madadi, Anita Sadeghpour
1Shaheed Rajaei Cardiovascular, Medical and Research Center, Tehran University of Medical Sciences, Tehran, Iran.
Insights
Left ventricular non-compaction cardiomyopathy is a rare congenital heart condition. Echocardiography and cardiac MRI are key diagnostic tools, with biopsy revealing hypertrophy and fibrosis in this case.
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Left ventricular non-compaction cardiomyopathy (LVNC) is a rare congenital cardiomyopathy affecting all ages.
- Diagnosis relies heavily on echocardiography due to insufficient clinical manifestations.
- Echocardiography identifies ventricular non-compaction and prognostic factors.
Observation:
- A 47-year-old woman with prior dilated cardiomyopathy of unknown cause presented with symptoms.
- Echocardiography revealed mild left ventricular enlargement and severe systolic dysfunction (EF 20-25%).
- Cardiac MRI suggested non-compaction left ventricle with hypertrophic cardiomyopathy.
Findings:
- Right ventricular endomyocardial biopsy was performed.
- Biopsy showed moderate cardiac myocyte hypertrophy and foci of myocytolysis.
- Moderate interstitial fibrosis was present, with no infiltrative deposition.
Implications:
- This case highlights the diagnostic utility of combined imaging modalities (echocardiography, MRI) in LVNC.
- Endomyocardial biopsy provides crucial histopathological details for diagnosis and understanding disease mechanisms.
- Further research is needed to elucidate the etiology and refine management strategies for LVNC.
Abstract:
Left ventricular non-compaction cardiomyopathy is a rare congenital cardiomyopathy that affects both children and adults. Since the clinical manifestations are not sufficient to establish diagnosis, echocardiography is the diagnostic tool that makes it possible to document ventricular non-compaction and establish prognostic factors. We report a 47-year-old woman with a history of dilated cardiomyopathy with unknown etiology. Echocardiography showed mild left ventricular enlargement with severe systolic dysfunction (EF = 20-25%). According to cardiac magnetic resonance imaging findings non-compaction left ventricle with hypertrophic cardiomyopathy was considered, and right ventricular septal biopsy was recommended. Right ventricular endomyocardial biopsy showed moderate hypertrophy of cardiac myocytes with foci of myocytolysis and moderate interstitial fibrosis. No evidence of infiltrative deposition was seen.
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