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Hypertrophic cardiomyopathy with apical left ventricular aneurysm
Y Akutsu1, A Shinozuka, T Y Huang
1Third Department of Internal Medicine, Showa University School of Medicine, Tokyo, Japan.
Japanese Circulation Journal
|April 29, 1998
Summary
This case study details a rare instance of hypertrophic cardiomyopathy (HCM) with an apical left ventricular aneurysm. The findings suggest apical myocardial injury or infarction in severe HCM, leading to aneurysm formation.
Area of Science:
- Cardiology
- Pathology
Background:
- Hypertrophic cardiomyopathy (HCM) is a complex cardiac condition.
- Apical left ventricular aneurysms are exceptionally rare complications of HCM.
Observation:
- A 54-year-old man with a 19-year history of non-obstructive apical HCM presented with palpitations and chest oppression.
- Advanced imaging revealed widespread left ventricular hypertrophy and an apical aneurysm.
- Histological analysis showed myocardial fibrosis and reduced MIBG/BMIPP uptake in the apex, despite preserved perfusion.
Findings:
- The apical myocardium exhibited extensive fibrosis and hypertrophic fibers, indicating myocardial injury or infarction.
- The anterior wall showed hypertrophy with disorganization but preserved perfusion and metabolic markers.
- Reduced MIBG and BMIPP uptake suggest impaired fatty acid metabolism and sympathetic denervation in the apical region.
Implications:
- Apical aneurysm formation in HCM may result from severe apical injury or infarction.
- These findings highlight the potential for myocardial damage and altered metabolism in specific HCM phenotypes.
- Understanding these mechanisms is crucial for diagnosing and managing rare HCM complications.