Clinical profile of patients with cardiac amyloidosis in India

Limited data exists on patients with cardiac amyloidosis (CA) in India, due to underdiagnosis and late presentation. We present single centre data from 13 patients over a 4 year period with a median age of 65 years. A majority presented with symptomatic heart failure (69%) and eight patients had confirmed AL amyloidosis. At the end of the follow up period, 46% patients died, with 30% of the overall cohort dead within six months. Among the survivors, 71% continue to have NYHA grade III/IV symptoms. A suggested algorithm for earlier diagnosis in resource constrained settings is also presented. Histopathological diagnosis of amyloidosis was obtained by abdominal fat pad biopsy in 4 (31%) patients, renal biopsy in 3 (23%) and bone marrow and gastrointestinal biopsies in two (15.3%) patients each. Bone marrow examination was available for 10 (77%) patients, of which five (38%) had concomitant multiple myeloma. Free light chain levels were available for eight patients, with median concentration of kappa chains 38 mg/dl (IQR, 27.5 to 175.5) and lambda 373 mg/dl (Range, 149–425). The median dFLC at diagnosis was 385 (IQR, 324 to 630).