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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.
Insights
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.
Abstract:
We report a case of hypertrophic cardiomyopathy (HCM) with apical left ventricular aneurysm, which is difficult to review because cases are so rare. A 54-year-old Japanese man was first found to have an electrocardiographic abnormality (T-wave inversion at rest) 19 years ago, and non-obstructive apical HCM without identifiable cause was diagnosed by echocardiography, left ventriculography, and clinical findings. After 19 years, he was admitted because of repeated episodes of palpitation and chest oppression at rest. Widespread left ventricular hypertrophy from the anteroseptal wall to the apex with an apical left ventricular aneurysm was detected by echocardiography, left ventriculography, and cardiac magnetic resonance imaging. Histologic examination of the hypertrophic apical myocardium surrounding the aneurysm showed that the myocardial tissue had been extensively replaced by fibrous tissue containing hypertrophic myocardial fibers, and uptakes of [123I]-metaiodobenzyl guanidine (MIBG) and [123I-] beta-methyliodophenyl pentadecanoic acid (BMIPP) in single-photon emission photography images were reduced despite high myocardial perfusion. On the other hand, histologic examination of the hypertrophic anterior wall revealed myocardial hypertrophy with disorganization; myocardial perfusion and the uptakes of MIBG and BMIPP were preserved. Abnormalities of myocardial fatty acid metabolism and sympathetic neuron activity with preserved perfusion flow and histologic changes such as fibrosis in the apical wall are indicative of apical myocardial injury or ischemia (infarction) without coronary artery stenosis; apical aneurysm may have occurred in severe apical HCM with cavity obliteration up to the midventricular level.