%0 Journal Article
%T Concomitant Transthyretin Amyloidosis and Severe Aortic Stenosis in Elderly Indian Population: A Pilot Study.
%A Singal AK
%A Bansal R
%A Singh A
%A Dorbala S
%A Sharma G
%A Gupta K
%A Saxena A
%A Bhargava B
%A Karthikeyan G
%A Ramakrishnan S
%A Bisoi AK
%A Hote MP
%A Rajashekar P
%A Chowdhury UK
%A Devagourou V
%A Patel C
%A Ray R
%A Arawa SK
%A Mishra S
%J JACC CardioOncol
%V 3
%N 4
%D Oct 2021
%M 34746852
%F 8.422
%R 10.1016/j.jaccao.2021.08.008
%X <B>BACKGROUND: </B>Prevalence of both degenerative severe aortic stenosis (AS) and transthyretin cardiac amyloidosis (ATTR-CA) increases with age. Dual disease (AS+myocardial ATTR-CA) occurs in significant proportion of patients undergoing surgical aortic valve replacement (SAVR).<BR><B>OBJECTIVE: </B>This study aimed to determine the prevalence of ATTR-CA in severe AS in the Indian population, identify noninvasive predictors of its diagnosis, and understand its impact on prognosis.<BR><B>METHODS: </B>Symptomatic severe AS patients aged ≥65 years undergoing SAVR were enrolled. ATTR-CA diagnosis was based on preoperative 99m-technetium pyrophosphate (PYP) scan and intraoperatively obtained basal interventricular septum biopsy for myocardial ATTR-CA, and excised native aortic valve for isolated valvular ATTR-CA. Primary amyloidosis was excluded by serum/urine protein electrophoresis with serum immunofixation.<BR><B>RESULTS: </B>SAVR was performed in 46 AS patients (age 70 ± 5 years, 70% men). PYP scan was performed for 32 patients, with significant PYP uptake in 3 (n = 3 of 32, 9.4%), suggestive of myocardial ATTR-CA. On histopathological examination, none of the interventricular septum biopsy specimens had amyloid deposits, whereas 33 (71.7%) native aortic valves showed amyloid deposits, of which 19 (57.6%) had transthyretin deposition suggestive of isolated valvular amyloidosis. Noninvasive markers of dual disease included low myocardial contraction fraction (median [interquartile range], 28.8% [23.8% to 39.1%] vs 15.3% [9.3% to 16.1%]; P = 0.006), deceleration time (215 [144 to 236] ms vs 88 [60 to 106] ms; P = 0.009) and global longitudinal strain (-18.7% [-21.1% to -16.9%] vs -14.2% [-17.0% to -9.7%]; P = 0.030). At 1-year follow-up, 2 patients died (4.3%); 1 each in myocardial ATTR-CA negative and positive groups (3.4% vs 33.3%; P = 0.477).<BR><B>CONCLUSIONS: </B>Dual disease is not uncommon in India. Isolated valvular amyloidosis in severe AS is much more common.