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Article

Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis

1
Departement of Clinical Medicine and Surgery, Federico II University Hospital, 80131 Naples, Italy
2
Mediterranea Cardiocentro, 80122 Naples, Italy
3
Department of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, Italy
4
Department of Public Health, Nephrology Unit, Federico II University Hospital, 80131 Naples, Italy
*
Author to whom correspondence should be addressed.
J. Clin. Med. 2020, 9(9), 2741; https://doi.org/10.3390/jcm9092741
Received: 21 July 2020 / Revised: 20 August 2020 / Accepted: 21 August 2020 / Published: 25 August 2020
(This article belongs to the Special Issue Clinical and Research of Genetic Cardiomyopathies)
Background: Anderson-Fabry disease (AFD) can induce both central nervous system white matter lesions (WMLs) and cardiac abnormalities including left atrial (LA) dysfunction. We sought to evaluate the possible interrelations of LA structure and function impairment with the presence of WMLs in AFD patients. Methods 22 AFD patients and 22 controls, matched for age and sex, underwent an echo-Doppler exam including quantification of peak atrial longitudinal strain (PALS). AFD patients underwent also a 3-T brain magnetic resonance imaging with a visual quantification of WMLs by Fazekas’ score (FS) on 3D FLAIR images. Results AFD patients had significantly higher left ventricular (LV) mass index (LVMi) and relative wall thickness, and lower PALS compared to controls. Among AFD patients, 9 showed a FS = 0, and 13 a FS > 1. AFD patients with FS ≥ 1 showed lower PALS (29.4 ± 6.7 vs. 37.2 ± 3.9%, p = 0.003) than those with FS = 0, without difference in LA volume index and LVMi. In AFD patients, FS was inversely related to PALS (r = −0.49, p < 0.0001), even after adjusting for LVMi (r = −0.43, p < 0.05). Conclusions In the absence of significant alterations in LA size, AFD patients had lower PALS compared to controls. The inverse association between PALS and presence of WMLs indicates a possible parallel early involvement of heart and brain. View Full-Text
Keywords: Anderson Fabry disease; echocardiography; left atrial strain; white matter lesions; fazekas’ score Anderson Fabry disease; echocardiography; left atrial strain; white matter lesions; fazekas’ score
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MDPI and ACS Style

Esposito, R.; Russo, C.; Santoro, C.; Cocozza, S.; Riccio, E.; Sorrentino, R.; Pontillo, G.; Luciano, F.; Imbriaco, M.; Brunetti, A.; Pisani, A. Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis. J. Clin. Med. 2020, 9, 2741. https://doi.org/10.3390/jcm9092741

AMA Style

Esposito R, Russo C, Santoro C, Cocozza S, Riccio E, Sorrentino R, Pontillo G, Luciano F, Imbriaco M, Brunetti A, Pisani A. Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis. Journal of Clinical Medicine. 2020; 9(9):2741. https://doi.org/10.3390/jcm9092741

Chicago/Turabian Style

Esposito, Roberta, Camilla Russo, Ciro Santoro, Sirio Cocozza, Eleonora Riccio, Regina Sorrentino, Giuseppe Pontillo, Federica Luciano, Massimo Imbriaco, Arturo Brunetti, and Antonio Pisani. 2020. "Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis" Journal of Clinical Medicine 9, no. 9: 2741. https://doi.org/10.3390/jcm9092741

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