Cardiac amyloidosis in India: A clinicopathological study

Background: Cardiac amyloidosis (CA) has been rarely reported from India. We aim to describe the experience of diagnosing and treating cardiac amyloid over three decades from a tertiary care center in India. Materials and Methods: Medical records were screened starting from 1987 up to 2019 and patients with histopathological diagnosis of CA were identified. The demographics, clinical presentation, investigations available, prognosis, and outcomes were retrospectively reviewed and analyzed. Results: A total of 40 patients were enrolled, out of which 26 (65%) were males. Median age at presentation was 51 years with a range of 33–65 years. Fatigue, weakness, and shortness of breath were the most common presenting symptoms. Clinical features of systemic amyloidosis including periorbital ecchymosis and macroglossia were present in 7.5% and 10% patients, respectively. Median survival from diagnosis was 2.1 years. Conclusions: Cardiac amyloid is a rarely diagnosed cause of restrictive cardiomyopathy in India and usually fatal. The patients in India are younger than the West. Endomyocardial biopsy is usually required to make a diagnosis of cardiac involvement though magnetic resonance imaging and nuclear imaging may raise a suspicion of cardiac amyloid.