Cardiac amyloidosis in India: Experience from a single tertiary care center

Abstract PURPOSE: Cardiac amyloidosis (CA) is perceived as a rare disease. Identifying certain red flag signs in patients helps in early diagnosis of CA. Epidemiology of CA in India is not well known. We performed this study to determine various demographic, clinical, and imaging parameters of patients diagnosed with CA in a tertiary care center in India. METHODS: We prospectively screened 48 patients between January 2021 and December 2023 with one or more red flags signs of CA. Their demographic, clinical, and imaging parameters were analyzed. RESULTS: Among 48 patients with one or more red flag signs, CA was diagnosed in 23 patients (47.91%). Seventeen patients were found to have AL CA and 6 had ATTR CA. The mean age of patients was 62.2 years with slight male preponderance ( n = 13, 56.5%). All the patients presented with heart failure with the majority in New York Heart Association Class III ( n = 11, 47.8%). The most common electrocardiogram abnormality was low-voltage QRS complexes in limb leads (56.5% of patients). Common echocardiographic abnormalities were left ventricular hypertrophy (100%), biatrial enlargement (100%), and reduced global longitudinal strain with relative apical sparing (cherry on top appearance) (74%). Tc99-PYP bone scintigraphy was done in 16 patients. It showed grade 2 uptake in all ATTR CA patients and 6 out of 11 in AL CA patients.Cardiovascular magnetic resonance was done in 5 AL CA patients. The most typical finding was diffuse subendocardial late gadolinium enhancement. CONCLUSIONS: There are significant lacunae in epidemiologic, diagnostic, and prognostic data of CA, especially in India. CA is not as uncommon, as it was perceived earlier. High index of suspicion with the use of novel echocardiographic parameters can identify patients who require further testing to confirm CA. Early diagnosis of CA helps in the early initiation of disease-modifying therapy which improves survival.