Advanced echocardiographic assessment in wild-type and hereditary transthyretin cardiac amyloidosis: the role of myocardial work and left atrial strain

Abstract

Abstract Introduction Transthyretin cardiac amyloidosis (ATTR-CA) is a progressive infiltrative cardiomyopathy caused by extracellular deposition of insoluble transthyretin (TTR) amyloid fibrils in the myocardium. ATTR-CA can be classified as wild type ATTR-CA (ATTRwt-CA), caused by age-related misfolding of TTR, and hereditary ATTR-CA (ATTRv-CA), an autosomal dominant disease in which gene mutations lead to changes in TTR protein. Speckle tracking echocardiography and myocardial work (MW) analysis can better characterize left ventricular and left atrial (LA) function in ATTRwt-CA and in ATTRv-CA. Aims to investigate differences in myocardial work and left atrial strain parameters between patients affected by ATTRwt-CA and patients affected by ATTRv-CA. Material and methods 40 male patients affected by ATTR-CA were prospectively enrolled, distinguishing between 20 patients with ATTRwt-CA (76 ± 7 yrs) and 20 patients with ATTRv-CA (74 ± 6 yrs) (1 pts with Val30Met mutation, 6 pts with Val122Ile mutation; 8 pts with Glu89Gln mutation; 5 pts with Phe64Leu mutation). All underwent a transthoracic echcoardiogram to calculate global longitudinal strain (GLS), MW parameters and LA strain. Results No difference was found between the two groups (ATTRwt-CA vs ATTRv-CA) for what regards age (76 ± 7 vs 74 ± 6 yrs, p = 0,06), BMI (27,2 ± 2,2 vs 26,6 kg/mq; p = 0,3), BSA (1,8 ± 0,2 vs 1,7 ± 0,1 mq; p = 0,1) and left ventricle mass index (LVMi) (183,41 ± 45,2 vs 148,5 ± 47,3 g/mq; p = 0,07). In ATTRwt-CA patients, ejection fraction (EF) is lower, but without any statistical significance (50 ± 6,9 vs 54 ± 6,4 %, p = 0,06). On the other hand, in ATTRwt-CA patients GLS is significantly higher than in ATTRv-CA patients (-9,8 ± 3,2 vs -15,0 ± 2,5 %; p < 0,0001). Global work index (GWI) (853,6 ± 367,6 vs 1431,6 ± 318,3 mmHg%; p < 0,0001), global constructive work (GCW) (1169 ± 303 vs 1726,5 ± 310,3 mmHg %; p < 0,0001) and global work efficiency (GWE) (83,2 ± 9,4 vs 89 ± 5,3 %; p = 0,04) are significantly reduced in ATTRwt-CA in comparison with ATTRv-CA. For what regards atrial function, in ATTwt-CA patients LA reservoir (9,7 ± 3,7 vs 17,6 ± 7,5 %; p = 0,004) and LA ejection fraction (LAFE) (28,9 ± 13,6 vs 46,2 ± 8,9; p = 0,02) are reduced, while LA booster is higher (-2,5 ± 3,1 vs -10 ± 4,7 %; p < 0, 0001). Conclusion In patients with ATTR-CA advanced echocardiographic assessment can better differentiate left atrial and left ventricular involvement in ATTRwt-CA versus ATTRv-CA.TablesGraphs