Time to Revisit—Cardiac Amyloidosis

Abstract

Amyloidosis" is a term that reminds us of our days in medical school because it was often tested in our pathology practical examinations and during general medicine examinations. However, most of us grew up even without making a diagnosis of cardiac amyloidosis (CA) in our clinical practice. The condition is now increasingly being recognized with advances in imaging and biochemical/ pathology laboratory testing. With the rise in the aging population, its incidence is expected to increase. More importantly, there is increasing evidence that it is an underrecognized entity, and the recent data has kindled hope for new therapeutic strategies.There are little data on CA in the Indian literature. As of early 1992, Talwar et al. 1 reported six cases of CA proven by endomyocardial biopsies in all cases. Only two of the six had suggestive features of echocardiography in their series. Raut et al. 2 could identify only six cases of CA from data spanning from 2008 to 2015. Certain features on echocardiography, late gadolinium enhancement (LGE) pattern on cardiac magnetic resonance (CMR), abdominal fat pad, and rectal biopsies were diagnostic in all. Mazumdar et al. 3 described a 56-year-old Indian patient who had syncope with heart failure. Echocardiogram suggested amyloidosis and fat pad histology confirmed amyloidosis. The study by Jain et al. 4 is published in this issue of IJCC. The authors showed that in a short span of 6 months, six patients were detected with echocardiographic features indicating CA. All of them could be confirmed as CA on bone marrow biopsy and other tests. The authors rightly concluded that a high degree of suspicion helps in early diagnosis.The transthyretin cardiac amyloidosis (ATTR-CA) and light-chain amyloidosis (AL-CA) are the two common types of CA. ATTR-CA is more common than it was previously appreciated. In a recent paper, Scully et al. 5 observed that one in seven patients undergoing TAVR had occult CA; the incidence of CA among degenerative aortic stenosis is approximately 16%.