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Apical segmental dysfunction in hypertrophic cardiomyopathy: progression into end-stage heart failure with sudden
1Department of Internal Medicine, National Taiwan University Hospital, Taipei, Republic of China.
Insights
Apical segmental dysfunction in hypertrophic cardiomyopathy (HCM) is rare but linked to poor outcomes. This condition may predict end-stage heart failure and sudden cardiac death risk in HCM patients.
Area of Science:
- Cardiology
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
- Apical segmental dysfunction is an uncommon echocardiographic finding in HCM.
Observation:
- Two cases of HCM with apical segmental dysfunction presented with progressive apical dilatation.
- Both patients developed congestive heart failure and died suddenly.
- Intractable ventricular tachyarrhythmias were noted in one case during resuscitation.
Findings:
- Apical segmental dysfunction in HCM is associated with a poor clinical course.
- This subgroup of HCM patients faces a high incidence of malignant ventricular tachyarrhythmias.
- The condition may indicate a predisposition to end-stage heart failure.
Implications:
- Apical segmental dysfunction may serve as a prognostic marker in hypertrophic cardiomyopathy.
- Identifying this subgroup can guide risk stratification and management strategies.
- Further research is needed to clarify the long-term prognosis and therapeutic options.
Abstract:
Apical segmental dysfunction is an unusual finding in hypertrophic cardiomyopathy (HCM). It is characterized by a poor clinical course and a high incidence of malignant ventricular tachyarrhythmias. Long-term prognosis of patients with HCM and apical segmental dysfunction is still unclear. We report 2 cases of apical segmental dysfunction in HCM in whom progressive apical dilatation and congestive heart failure developed. Both patients died suddenly, and intractable ventricular tachyarrhythmias were documented in 1 case during resuscitation. This report provides further evidence that apical segmental dysfunction might predict a subgroup of patients with HCM who are likely to develop end-stage heart failure and are at high risk of sudden cardiac death.
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