Academic Journal
ECG as a storytelling of cardiac involvement evolution in Anderson Fabry disease
| العنوان: | ECG as a storytelling of cardiac involvement evolution in Anderson Fabry disease |
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| المؤلفون: | Chiti, C, Ditaranto, R, Barlocco, F, Lillo, R, Re, F, Marchi, G, Parisi, V, Ferrara, V, Baldassarre, R, Di Nicola, F, Gimeno Blanes, J R, Graziani, F, Galie', N, Olivotto, I, Biagini, E |
| المصدر: | European Heart Journal ; volume 43, issue Supplement_2 ; ISSN 0195-668X 1522-9645 |
| بيانات النشر: | Oxford University Press (OUP) |
| سنة النشر: | 2022 |
| الوصف: | Background Cardiac involvement in Anderson-Fabry disease (AFD) is related to a progressive glycosphingolipid storage over time and is characterized by left ventricular hypertrophy (LVH), conduction abnormalities and myocardial fibrosis. ECG is useful for early recognition of AFD, however evidence is limited on the association between ECG alterations and disease stage. Purpose To assess the relationship between ECG characteristics and progressive cardiac involvement, from the pre-hypertrophic phase to phenotypes with increasing degree of LVH. Methods In a multicenter cohort, 183 AFD patients (40% male, age 47±12 years, 60% affected by “classical AFD”) underwent ECG and transthoracic echocardiography. Patients were divided into 4 groups according to the different degree of LV thickness measured in parasternal short axis view: group A ≤9 mm (N=46, 25%), group B 10–14 mm (N=77, 42%), group C 15–19 mm (N=45, 25%) and group D ≥20 mm (N=15, 8%). Patients with pacemaker and under 18 years of age were excluded. Results A normal ECG was present in 89% in group A, 59% in group B, 11% in group C and it was absent in group D. Short PR (<120 ms) was more frequent in group A, whereas with LVH increasing, median PR interval duration significantly prolonged among the 4 groups (136 [125–150]vs 141 [130–160] vs 160 [130–180] vs 170 [130–180] ms, p=0.002 respectively). Median P-wave duration was shorter in group A and B compared to group C and D (80 m vs 100 ms, p<0.001), while both QRS and QTc gradually increased. Median Sokolow-Lyon voltage criteria statistically augmented among the groups (22 [18–26] vs 27 [20–33] vs 32 [25–45] vs 35 [18–40] mm, p<0.001 respectively), along with right ventricular hypertrophy (0%, 1%, 11%, 8%, p=0.02). Right bundle branch block (RBBB) had a higher frequency in advanced stages (0%, 34%, 34%, 40%, p<0.001), with a prevalence of complete RBBB of 46% in group D. Similarly, left anterior fascicular block (0%, 7%, 18%, 46%, p<0.001) and QRS fragmentation (2%, ... |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| DOI: | 10.1093/eurheartj/ehac544.1777 |
| الاتاحة: | https://doi.org/10.1093/eurheartj/ehac544.1777 https://academic.oup.com/eurheartj/article-pdf/43/Supplement_2/ehac544.1777/46364633/ehac544.1777.pdf |
| Rights: | https://academic.oup.com/pages/standard-publication-reuse-rights |
| رقم الانضمام: | edsbas.284F137C |
| قاعدة البيانات: | BASE |
| DOI: | 10.1093/eurheartj/ehac544.1777 |
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