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Prognosis in hypertrophic cardiomyopathy observed in a large clinic population
M J Kofflard1, D J Waldstein, J Vos
1Department of Cardiology, Thoraxcenter, Academic Hospital, Dijkzigt, Erasmus University, Rotterdam, The Netherlands.
Insights
Hypertrophic cardiomyopathy (HC) has a better prognosis than previously thought. This study found a 1% annual cardiac mortality rate, significantly lower than earlier estimates.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Previous estimates of annual cardiac mortality in hypertrophic cardiomyopathy (HC) range from 2-4%, largely based on retrospective data from specialized centers.
- There is a need for prospective data to accurately assess cardiac mortality in a broader HC patient population.
Purpose of the Study:
- To prospectively evaluate the annual cardiac mortality rate in a cohort of 113 patients diagnosed with hypertrophic cardiomyopathy (HC).
- To assess cardiac mortality risk in subgroups traditionally considered high-risk for sudden cardiac death.
Main Methods:
- A prospective study of 113 patients with hypertrophic cardiomyopathy (HC).
- Data collection included patient demographics, clinical history, and cardiac events during follow-up.
- Cardiac mortality was calculated as an annual rate.
Main Results:
- The mean age at diagnosis was 37 +/- 16 years.
- Over the follow-up period, 11 cardiac deaths and 2 non-cardiac deaths occurred.
- The overall annual cardiac mortality rate was 1% (95% CI: 0.2-1.8%).
- No significant difference in cardiac death risk was observed in high-risk subgroups (young age, family history, syncope, ventricular tachycardia, or prior surgery).
Conclusions:
- Hypertrophic cardiomyopathy (HC) appears to have a more benign prognosis than previously reported, with an annual cardiac mortality of 1%.
- This prospective finding suggests HC mortality may be 2 to 4 times lower than suggested by retrospective studies.
- Risk stratification for sudden death in HC may require re-evaluation based on these findings.
Abstract:
Overall annual cardiac mortality in hypertrophic cardiomyopathy (HC) has been reported to be between 2 and 4%, although these numbers are primarily from retrospective studies of patients referred to large research institutions. A clinic population of 113 patients with HC was prospectively studied to assess cardiac mortality in the overall group and in selected subgroups commonly thought to be at high risk for sudden death. The mean age at diagnosis was 37 +/- 16 years. During follow-up, there were 11 cardiac and 2 noncardiac deaths. The annual cardiac mortality was 1% (95% confidence interval 0.2-1.8%). Because of the small number of deaths, relative risk for cardiac death was not significantly different in the presence of young age (< or = 30 years), family history of HC and sudden death, history of syncope or previous cardiac arrest, or both, ventricular tachycardia on 24-hour Holter monitoring, or septal myotomy/myectomy for refractory symptoms and outflow tract obstruction. It is concluded that HC has a relatively benign prognosis (1% annual cardiac mortality) that is 2 to 4 times less than that previously reported.