Hypertrophic cardiomyopathy: an autopsy analysis of 14 cases.

BACKGROUND

Hypertrophic cardiomyopathy (HCM) is one of the less common forms of primary cardiomyopathies. There is little data available on HCM in Indian literature.

AIMS: To assess the incidence and analyse the clinicopathological features of HCM.

SETTINGS: Analysis of data of 15 years from a tertiary care centre.

METHODS AND MATERIAL: The clinical and pathological data in fourteen cases of HCM with respect to their gross and microscopic features and clinical presentation were reviewed.

RESULTS

Incidence of HCM amongst the autopsied primary cardiomyopathies (N = 101) was 13.9% (n=14). Males were affected more. Common presenting symptoms were exertional dyspnoea, angina and palpitations. Concentric and asymmetric hypertrophy was equally seen. Obliterative small vessel disease was noted in 50% of the cases. Although significant myofibre disarray (>5%) was seen in all fourteen cases, it could be demonstrated in only 40- 50% of an average of twenty sections studied. Type IA myofibre disarray was the commonest. Six of the fourteen patients died suddenly. Cardiac failure was the commonest cause of death.

CONCLUSIONS

Myofibre disarray is a highly sensitive and specific marker for HCM only when considered in a quantitative rather than a qualitative fashion. In this context, the rationale for performing endomyocardial biopsy is to rule out mimics of HCM.