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Published on: June 14, 2016
Hypertrophic cardiomyopathy: an autopsy analysis of 14 cases
R S Phadke1, P Vaideeswar, B Mittal
1Department of Pathology, Seth G. S. Medical College and K. E. M. Hospital, Mumbai, India. phrahul25@hotmail.com
Insights
Hypertrophic cardiomyopathy (HCM) incidence in India was 13.9% among cardiomyopathies. Quantitative myofibre disarray is key for diagnosing HCM and ruling out other conditions.
Area of Science:
- Cardiology
- Pathology
Background:
- Hypertrophic cardiomyopathy (HCM) is a rare primary cardiomyopathy with limited data in Indian literature.
- This study addresses the scarcity of information on HCM within the Indian context.
Purpose of the Study:
- To determine the incidence of HCM in Indian patients.
- To analyze the clinicopathological characteristics of HCM.
Main Methods:
- A 15-year retrospective analysis of clinical and pathological data from a tertiary care center.
- Review of fourteen confirmed HCM cases, examining gross, microscopic, and clinical features.
Main Results:
- HCM accounted for 13.9% of primary cardiomyopathies (14 out of 101 autopsies), with a higher prevalence in males.
- Common symptoms included exertional dyspnea, angina, and palpitations; 50% showed obliterative small vessel disease.
- Significant myofibre disarray (>5%) was present in all cases, though only detectable in 40-50% of sections; Type IA was most common. Sudden death occurred in 6 patients, with cardiac failure being the leading cause of mortality.
Conclusions:
- Quantitative assessment of myofibre disarray is crucial for sensitive and specific HCM diagnosis.
- Endomyocardial biopsy is valuable for differentiating HCM from conditions with similar presentations.
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.
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