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Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm: a case report
K Harada1, T Shimizu, Y Sugishita
1Department of Cardiovascular Medicine, Graduate School of Medicine, University of Tokyo, Japan. harada-2im@h.u-tokyo.ac.jp
Insights
This case study highlights a rare combination of hypertrophic cardiomyopathy, midventricular obstruction, and apical aneurysm in an elderly woman. Myocardial ischemia is suggested as a potential cause for the apical aneurysm.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Pathophysiology
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
- Midventricular obstruction and apical aneurysms are rare complications of HCM.
Observation:
- A 71-year-old woman presented with a history of abnormal electrocardiograms, dizziness, and low blood pressure.
- Holter monitoring revealed significant ventricular arrhythmias, including nonsustained ventricular tachycardia.
- Doppler echocardiography, MRI, and angiography confirmed midventricular hypertrophy, apical aneurysm, and aberrant coronary artery compression.
Findings:
- Hemodynamic study showed low cardiac output and a significant intraventricular pressure gradient.
- Stress thallium-201 imaging indicated severe persistent defect in the left ventricular apex.
- The findings suggest myocardial ischemia may contribute to apical aneurysm formation in HCM.
Implications:
- This case underscores the complex interplay of structural abnormalities and potential ischemia in HCM.
- Understanding these rare presentations is crucial for accurate diagnosis and management of hypertrophic cardiomyopathy.
- Further research into the role of ischemia in HCM complications is warranted.
Abstract:
A 71-year-old woman had hypertrophic cardiomyopathy associated with midventricular obstruction and an apical aneurysm in the left ventricle. She had had abnormal electrocardiograms for more than 30 years and for the past year had been suffering from occasional attacks of dizziness and low systemic blood pressure. Holter 24-h electrocardiographic monitoring revealed ventricular paroxysmal contractions (676/day) with nonsustained ventricular tachycardia. Doppler echocardiography revealed paradoxical jet flow from the apical aneurysm to the left ventricular outflow during early diastole. Magnetic resonance imaging depicted midventricular hypertrophy and a dyskinetic thin apical wall, which were confirmed by angiography. Coronary angiograms showed no narrowing of the major extramural coronary arteries, but there was compression of aberrant coronary arteries apparently feeding the hypertrophic portion of the left ventricular wall. Stress thallium-201 myocardial imaging showed a persistent severe defect in the left ventricular apex. A hemodynamic study revealed low cardiac output and an intraventricular pressure gradient (approximately 90 mmHg) between the left ventricular apical high-pressure chamber and the subaortic low-pressure chamber. The present case represents a rare combination of hypertrophic cardiomyopathy, midventricular obstruction, and an apical aneurysm in an elderly woman. Myocardial ischemia may have played an important role in the genesis of the apical aneurysm.
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