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), PendQ 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).
Electrocardiogram evolution in Anderson-Fabry disease patients during follow-up in relation to specific treatment and cardiac disease progression / Parisi V., Ditaranto R., Taglieri N., Di Nicola F., Baldassarre R., Schiavo M.A., Nardi E., Lillo R., Meucci M.C., Cianci A., Re F., Marchi G., Monda E., Capelli I., Donadio V., Cappelli F., Salamon I., Blanes J.-R.G., Girelli D., Pasquale F., et al.. - In: INTERNATIONAL JOURNAL OF CARDIOLOGY. - ISSN 0167-5273. - ELETTRONICO. - 431:(2025), pp. 133219.0-133219.0. [10.1016/j.ijcard.2025.133219]
Electrocardiogram evolution in Anderson-Fabry disease patients during follow-up in relation to specific treatment and cardiac disease progression
Schiavo M. A.;Lillo R.;Marchi G.;Capelli I.;Cappelli F.;Pasquale F.;Biffi M.;Mignani R.;Graziani F.;Limongelli G.;Olivotto I.;
2025
Abstract
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), PendQ 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).
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