In:
Diagnostics, MDPI AG, Vol. 13, No. 3 ( 2023-01-18), p. 354-
Abstract:
Background: Fabry disease (FD) is an X-linked, lysosomal storage disorder leading to severe cardiomyopathy in a significant proportion of patients. To identify ECG markers that reflect early cardiac involvement and disease progression, we conducted a long term retrospective study in a large cohort of FD patients. Methods: A total of 1995 ECGs from 133 patients with classical FD (64% females, 80% treated with enzyme replacement therapy), spanning 20 years of follow-up, were compared to ECGs from 3893 apparently healthy individuals. Generalized linear mixed models were used to evaluate the effect of age, FD and sex on: P-wave duration, PR-interval, QRS-duration, QTc, Cornell index, spatial QRS-T angle and frontal QRS-axis. Regression slopes and absolute values for each parameter were compared between FD patients and control subjects. Results: At a younger age ( 〈 40 years), the Cornell index was higher and frontal QRS-axis more negative in FD patients compared to controls (p 〈 0.05). For the other ECG parameters, the rate of change, more than the absolute value, was greater in FD patients compared to controls (p 〈 0.05). From the fifth decade (men) or sixth (women) onwards, absolute values for P-wave duration, QRS-duration, QTc and spatial QRS-T angle were longer and higher in FD patients compared to control subjects. Conclusions: ECG abnormalities indicative of FD are age and sex dependent. Tracking the rate of change in ECG parameters could be a good way to detect disease progression, guiding treatment initiation. Moreover, monitoring ECG changes in FD can be used to evaluate the effectiveness of treatment.
Type of Medium:
Online Resource
ISSN:
2075-4418
DOI:
10.3390/diagnostics13030354
Language:
English
Publisher:
MDPI AG
Publication Date:
2023
detail.hit.zdb_id:
2662336-5
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