Pathobiology of cardiomyopathies: Experience at a Tertiary Care Center

Abstract

Background: Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with varied pathology. Pathology data from India are scarce. Methods: A retrospective, descriptive study of autopsies, as well as endomyocardial biopsy specimens, was done of patients with cardiomyopathy. The clinical and pathological features are described. Results: There were 32 patients with dilated cardiomyopathy. Two were pediatric, and two had arrhythmogenic right ventricular cardiomyopathy. Myocarditis was seen in 12 cases. In our endomyocardial biopsy data of 32 patients with restrictive cardiomyopathy (RCM), we found amyloid in 13 and idiopathic RCM in the remainder. Our genetic studies in cardiomyopathies suggest that the same genetic mutation may lead to different phenotypic manifestations with restrictive or hypertrophic cardiomyopathies in different families. Conclusions: This study gives insight into the pathology and etiology of some of the cardiomyopathies seen in India. They differ from the west, and now with the availability of genotyping and magnetic resonance imaging, more data should soon be available from more centers.

Keywords

• Arrhythmogenic right ventricular cardiomyopathy
• dilated cardiomyopathy
• hypertrophic cardiomyopathy
• restrictive cardiomyopathy