Right Atrial Strain In Anderson-Fabry Disease

Rosa Lillo ^1,2 Alessio Cianci ² Maria Chiara Meucci ¹ Giulia Iannaccone ^1,2 Claudio Di Brango ² Filippo Tusa ² Mario Marsilia ² Gaetano Antonio Lanza ^1,2 Antonella LOMBARDO ^1,2 Francesco Burzotta ^1,2 FRANCESCA GRAZIANI ^1*

• ¹ Department of Cardiovascular Sciences, Agostino Gemelli University Polyclinic (IRCCS), Rome, Italy
• ² Department of Cardiovascular and Pneumological Sciences, Catholic University of the Sacred Heart, Rome, Roma, Italy

Background: Nowadays limited data are available on right atrium (RA) morpho-functional remodelling in Fabry disease (FD).Purpose: To investigate RA structural and functional remodelling in FD vs healthy controls (HC) using 2D speckle tracking echocardiography (STE) and to explore if differences exist in FD patients with and without left ventricular hypertrophy (LVH).We prospectively enrolled FD patients and controls matched for age, sex and cardiovascular risk factors. FD patients were divided in two groups, according to the presence/absence of LVH (LVH+: left ventricular wall thickness > 12 mm). All patients underwent standard echocardiography and STE analysis investigating the mechanics of all cardiac chambers, including RA reservoir, contractile and conduit strain.Results: A total of 64 patients with FD (50% males; mean age 50 ± 17 years; 51.5 % LVH+) and 64 controls were included. Focusing on right chambers, RA and right ventricular (RV) dimensions were similar between FD and controls, no differences were found for TAPSE (p=0.073) and RVFAC (p=0.461) while RV systolic Tissue Doppler velocity was reduced in the former (p=0.041). STE revealed impaired strain values in all cardiac chambers in FD vs HC: left ventricular global longitudinal strain (LV-GLS, p<0.001), left atrial (LA) reservoir (p=0.001), conduit (p=0.012) and contractile strain (p<0.001), RV-GLS and RV free wall strain (p<0.001).Similarly, all RA strain phases were significantly reduced in FD vs controls (RA reservoir 27.4 ± 11.1 vs 41.9 ± 8.3%, p<0.001; RA contractile 9.9 ± 5.1 vs 18.0 ± 4.9%, p<0.001; RA conduit 19.1 ± 8.1 vs 24.1 ± 8.1%, p=0.001). When comparing FD LVH-vs controls, RA reservoir and contractile strains were significantly reduced in the formers (p<0.001). In multivariable linear regression analyses, LA reservoir (p=0.010) and LV-GLS (p=0.044) emerged as independent correlates of RA mechanics, after adjustment for RA dimensions, RV systolic function parameters and hypertrophy, LV maximal wall thickness.In FD impaired RA strains is a common finding. RA reservoir and contractile strains are reduced in FD patients even before LVH ensues, as compared to controls. LA reservoir strain and LV-GLS showed an independent correlation with RA reservoir strain.