Suspecting and diagnosing transthyretin amyloid cardiomyopathy (ATTR-CM) in India: An Indian expert consensus

Transthyretin cardiac amyloidosis (ATTR-CM) is a rare and under-recognized disorder characterized by the aggregation of transthyretin-derived insoluble amyloid fibrils in the myocardium. Heterogeneity of symptoms at presentation, makes its diagnosis often delayed. An expert panel gathered on a virtual platform across India to conduct a meeting for developing a guiding tool for ATTR-CM diagnosis. The panel recommended younger age (40 years) for suspecting ATTR-CM and thick-walled non-dilated hypokinetic ventricle was considered as one of the important red flags. Electrocardiogram (ECG) and echocardiography (ECHO) findings were recommended as primary tests to raise the suspicion while nuclear scintigraphy and hematological tests were recommended to confirm the diagnosis and rule out amyloid light-chain (AL) amyloidosis. Cardiac magnetic resonance (CMR) and biopsy were recommended in case of ambiguity in the presence of red flags. Considering the lack of expert guidelines in the Indian scenario, a standardized diagnostic algorithm was also proposed.

Keywords

Transthyretin cardiac amyloidosis (ATTRCM), Rare disorder, Amyloidosis, Cardiac dysfunction

Citation

Mohan Jagdish Chander, Dalal Jamshed, Chopra Vijay Kumar, Narasimhan Calambur, Kerkar Prafulla, Oomman Abraham, Fcsi Saumitra Ray, Sharma Anshu Rajnish, Dougall Pankaj, Simon Shelley, Drm Atul Verma, Radhakrishnan Vivek. Suspecting and diagnosing transthyretin amyloid cardiomyopathy (ATTR-CM) in India: An Indian expert consensus. Indian Heart Journal. 2022 Dec; 74(6): 441-449

URI

https://imsear.searo.who.int/handle/123456789/220941

Collections

Indian Heart Journal

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