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Morphologic features of exertional versus nonexertional sudden death in patients with hypertrophic cardiomyopathy
Fabio Tavora1, Nathaniel Cresswell, Ling Li
1Armed Forces Institute of Pathology, Washington, DC, USA.
Insights
Sudden deaths from hypertrophic cardiomyopathy (HC) occurred at a younger age during exertion. Decreased heart weight and male gender were associated with exertional sudden death in HC patients.
Area of Science:
- Cardiovascular Pathology
- Sudden Cardiac Death
- Hypertrophic Cardiomyopathy
Background:
- Hypertrophic cardiomyopathy (HC) is a leading cause of sudden cardiac death, particularly in young individuals.
- Pathologic features differentiating exertional versus nonexertional sudden deaths in HC are not well-established.
- Understanding these differences is crucial for risk stratification and prevention strategies.
Purpose of the Study:
- To investigate and compare the pathologic features of hypertrophic cardiomyopathy in sudden deaths related to exertion versus nonexertional events.
- To identify specific pathological characteristics associated with exertional sudden death in HC.
- To correlate autopsy findings with clinical data for a comprehensive analysis.
Main Methods:
- Analysis of gross measurements and histologic findings from 103 autopsy cases of hypertrophic cardiomyopathy.
- Comparison of pathologic features between 71 sudden deaths, categorized as exertional or nonexertional.
- Multivariate analysis to determine significant associations with exertional sudden death.
Main Results:
- Sudden deaths due to exertion occurred at a significantly younger age (27 years) compared to nonexertional deaths (40 years).
- Exertional deaths were more prevalent in women, while nonexertional deaths occurred more frequently at rest.
- Decreased mean heart weight and male gender were significantly associated with exertional sudden death in HC.
Conclusions:
- No specific pathologic features definitively identify hypertrophic cardiomyopathy patients at risk for exertional sudden death.
- Relatively decreased heart weight is a strong indicator for exertional sudden death in HC.
- Cardiologists should consider HC in athletes with mild cardiomegaly, especially young men, even without significant asymmetry.
Abstract:
Pathologic features that characterize hypertrophic cardiomyopathy (HC) in exertional versus nonexertional sudden deaths have not been extensively studied. We performed gross measurements and histologic analysis on 103 autopsy cases of HC and correlated these with clinical findings. Pathologic features of the 71 sudden deaths were compared between exertional and nonexertional deaths. Age at death was significantly younger in exertional (27 +/- 13 years) versus nonexertional sudden deaths (40 +/- 16 years, p = 0.0003). Exertional deaths were more likely in women (35 of 37) versus sudden deaths at rest (21 of 34, p = 0.0002). There was no significant difference in the incidence of syncope in the exertional sudden deaths (14%) compared to the nonexertional sudden deaths (9%, p = 0.5) or in the rate of a previous diagnosis of HC (21% vs 21%, respectively). Mean heart weight was significantly decreased in exertional sudden deaths versus nonexertional sudden deaths. There was no difference in the frequency of left ventricular outflow tract plaque (54% exertional vs 46% nonexertional, p = 0.06). By multivariate analysis, including all categories of HC, only decreased heart weight (p = 0.02) and male gender (p = 0.002) were significantly associated with exertional sudden death. In conclusion, there are no pathologic features that would identify patients with HC at risk for exertional death. Because relatively decreased heart weight is strongly associated with exertional death, and because a large proportion of exertional deaths with HC are not associated with significant asymmetry, cardiologists should be careful in excluding the diagnosis of HC in athletes with even mild degrees of cardiomegaly, especially young men.
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