Epidemiology of cardiomyopathy – A clinical and genetic study of restrictive cardiomyopathy: The EPOCH-R study

Abstract

Introduction: Restrictive cardiomyopathy (RCM) is characterized by diastolic dysfunction, biatrial enlargement, and normal or near-normal systolic function. RCM is the rarest kind among cardiomyopathies with a severe outcome. Methods: Here, we present the clinical outcomes of thirty RCM patients recruited from a tertiary care unit of India, All India Institute of Medical Sciences, New Delhi. For clinical assessment, patients underwent electrocardiogram, echocardiography, and cardiac catheterization, and endomyocardial biopsy whenever required. Results: Out of 190 patients with cardiomyopathy, 100 had dilated cardiomyopathy, 60 had hypertrophic cardiomyopathy, and 30 had idiopathic RCM and were recruited for the study. Out of these thirty patients, 63.3% were males. A maximum number of patients were diagnosed in their second to third decade of life. Atrial fibrillation (73.3%) and ST-T abnormalities (76.6%) were common. Most of the patients showed the early age of onset with symptoms emerging in the first and second decades of life. Shortness of breath and fatigue were found to be common symptoms. No familial cases were found. Conclusion: RCM in India is a sporadic disease, rare, and occurs in the young. Prognosis of RCM is still worse than any other cardiomyopathy.