Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis
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 pat...
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MDPI AG
2020-08-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/9/9/2741 |
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| author | Roberta Esposito Camilla Russo Ciro Santoro Sirio Cocozza Eleonora Riccio Regina Sorrentino Giuseppe Pontillo Federica Luciano Massimo Imbriaco Arturo Brunetti Antonio Pisani |
| author_facet | Roberta Esposito Camilla Russo Ciro Santoro Sirio Cocozza Eleonora Riccio Regina Sorrentino Giuseppe Pontillo Federica Luciano Massimo Imbriaco Arturo Brunetti Antonio Pisani |
| author_sort | Roberta Esposito |
| collection | DOAJ |
| description | 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%, <i>p</i> = 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, <i>p</i> < 0.0001), even after adjusting for LVMi (r = −0.43, <i>p</i> < 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. |
| first_indexed | 2024-03-10T16:51:10Z |
| format | Article |
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| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-10T16:51:10Z |
| publishDate | 2020-08-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-c4732e205ed446999bde3768d012493e2023-11-20T11:18:09ZengMDPI AGJournal of Clinical Medicine2077-03832020-08-0199274110.3390/jcm9092741Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at DiagnosisRoberta Esposito0Camilla Russo1Ciro Santoro2Sirio Cocozza3Eleonora Riccio4Regina Sorrentino5Giuseppe Pontillo6Federica Luciano7Massimo Imbriaco8Arturo Brunetti9Antonio Pisani10Departement of Clinical Medicine and Surgery, Federico II University Hospital, 80131 Naples, ItalyDepartment of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, ItalyDepartment of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, ItalyDepartment of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, ItalyDepartment of Public Health, Nephrology Unit, Federico II University Hospital, 80131 Naples, ItalyDepartment of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, ItalyDepartment of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, ItalyDepartment of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, ItalyDepartment of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, ItalyDepartment of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, ItalyDepartment of Public Health, Nephrology Unit, Federico II University Hospital, 80131 Naples, ItalyBackground: 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%, <i>p</i> = 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, <i>p</i> < 0.0001), even after adjusting for LVMi (r = −0.43, <i>p</i> < 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.https://www.mdpi.com/2077-0383/9/9/2741Anderson Fabry diseaseechocardiographyleft atrial strainwhite matter lesionsfazekas’ score |
| spellingShingle | Roberta Esposito Camilla Russo Ciro Santoro Sirio Cocozza Eleonora Riccio Regina Sorrentino Giuseppe Pontillo Federica Luciano Massimo Imbriaco Arturo Brunetti Antonio Pisani Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis Journal of Clinical Medicine Anderson Fabry disease echocardiography left atrial strain white matter lesions fazekas’ score |
| title | Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis |
| title_full | Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis |
| title_fullStr | Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis |
| title_full_unstemmed | Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis |
| title_short | Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis |
| title_sort | association between left atrial deformation and brain involvement in patients with anderson fabry disease at diagnosis |
| topic | Anderson Fabry disease echocardiography left atrial strain white matter lesions fazekas’ score |
| url | https://www.mdpi.com/2077-0383/9/9/2741 |
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