Hypertrophic cardiomyopathy: an autopsy analysis of 14 cases - PubMed (original) (raw)

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.