Pubblicazioni

Autori:

Parisi, Vanda; Ditaranto, Raffaello; Taglieri, Nevio; Di Nicola, Federico; Baldassarre, Riccardo; Schiavo, Maria Alessandra; Nardi, Elena; Lillo, Rosa; Meucci, Maria Chiara; Cianci, Alessio; Re, Federica; Marchi, Giacomo; Monda, Emanuele; Capelli, Irene; Donadio, Vincenzo; Cappelli, Francesco; Salamon, Irene; Blanes, Juan-Ramón Gimeno; Girelli, Domenico; Pasquale, Ferdinando; Biffi, Mauro; Mignani, Renzo; Graziani, Francesca; Galiè, Nazzareno; Limongelli, Giuseppe; Olivotto, Iacopo; Biagini, Elena

Titolo:

Electrocardiogram evolution in Anderson-Fabry disease patients during follow-up in relation to specific treatment and cardiac disease progression

Anno:

2025

Tipologia prodotto:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Lingua:

Inglese

Referee:

No

Nome rivista:

International Journal of Cardiology

ISSN Rivista:

0167-5273

N° Volume:

431

Intervallo pagine:

1-10

Parole chiave:

Anderson-Fabry disease; Electrocardiogram (ECG); Evolution; Follow-up; Treatment status

Breve descrizione dei contenuti:

Aims: Electrocardiogram (ECG) analysis plays a central role in Anderson-Fabry disease (AFD) diagnosis and management. This study aimed to assess ECG evolution during follow-up in relation to specific treatment and disease progression. Methods: Retrospective study of a multicentric cohort of AFD patients with >= 2 ECG and echocardiographic data. Specific treatment status (enzyme replacement or chaperone) was defined as: chronic therapy (>= 1 year before the first ECG); therapy started during the follow-up; no therapy. Results: One-hundred-eighty-one AFD patients (median age 46 years, IQR 35-58; 36 % male; 67 % classic phenotype composed the study population: 24 % were on chronic therapy; 39 % started the therapy during follow-up; 37 % had no specific therapy. During a median follow-up of 62 months (IQR 33-83), significant ECG variations were: atrial fibrillation detection (p = 0.005), P(end)Q interval (p = 0.04), left atrial enlargement (p < 0.001), new right bundle branch block (RBBB, p < 0.001), QRS interval (p < 0.001), QRS fragmentation (p = 0.024), QTc (p = 0.001), and symmetric lateral negative T waves development (p = 0.016). P(end)Q interval showed a significant interaction with treatment status, significantly increasing in patients without or on chronic therapy but slightly reducing in patients who started the specific therapy during follow-up (p for interaction = 0.035). P(end)Q interval, new RBBB and pathologic QTc were associated with left ventricle wall thickness increase (p = 0.003, p = 0.014, p = 0.019, respectively). Conclusions: In this multicentric cohort of AFD patients, several ECG parameters showed significant changes during follow-up. Only P(end)Q interval showed a significant interaction with treatment status. Moreover, P(end)Q interval, new RBBB and pathologic QTc development were associated with cardiac hypertrophy progression.

Id prodotto:

146148

Handle IRIS:

11562/1163868

ultima modifica:

5 giugno 2025

Citazione bibliografica:

Parisi, Vanda; Ditaranto, Raffaello; Taglieri, Nevio; Di Nicola, Federico; Baldassarre, Riccardo; Schiavo, Maria Alessandra; Nardi, Elena; Lillo, Rosa; Meucci, Maria Chiara; Cianci, Alessio; Re, Federica; Marchi, Giacomo; Monda, Emanuele; Capelli, Irene; Donadio, Vincenzo; Cappelli, Francesco; Salamon, Irene; Blanes, Juan-Ramón Gimeno; Girelli, Domenico; Pasquale, Ferdinando; Biffi, Mauro; Mignani, Renzo; Graziani, Francesca; Galiè, Nazzareno; Limongelli, Giuseppe; Olivotto, Iacopo; Biagini, Elena, Electrocardiogram evolution in Anderson-Fabry disease patients during follow-up in relation to specific treatment and cardiac disease progression «International Journal of Cardiology» , vol. 431 , 2025 , pp. 1-10

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