ECG Changes during Adult Life in Fabry Disease: Results from a Large Longitudinal Cohort Study
Mohamed El Sayed,
Pieter G. Postema,
Mareen Datema,
Laura van Dussen,
Jan A. Kors,
Cato C. ter Haar,
Hidde Bleijendaal,
Henrike Galenkamp,
Bert-Jan H. van den Born,
Carla E. M. Hollak,
Mirjam Langeveld
Affiliations
Mohamed El Sayed
Department of Internal Medicine, Division of Endocrinology and Metabolism, Amsterdam UMC Location University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Pieter G. Postema
Department of Cardiology, Heart Center, Amsterdam UMC Location University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Mareen Datema
Department of Internal Medicine, Division of Endocrinology and Metabolism, Amsterdam UMC Location University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Laura van Dussen
Department of Internal Medicine, Division of Endocrinology and Metabolism, Amsterdam UMC Location University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Jan A. Kors
Department of Medical Informatics, Erasmus MC, University Medical Center Rotterdam, 3015 GD Rotterdam, The Netherlands
Cato C. ter Haar
Department of Cardiology, Heart Center, Amsterdam UMC Location University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Hidde Bleijendaal
Department of Cardiology, Heart Center, Amsterdam UMC Location University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Henrike Galenkamp
Department of Public and Occupational Health, Amsterdam UMC, Location AMC, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Bert-Jan H. van den Born
Amsterdam Public Health, Health Behaviors and Chronic Diseases, 1105 AZ Amsterdam, The Netherlands
Carla E. M. Hollak
Department of Internal Medicine, Division of Endocrinology and Metabolism, Amsterdam UMC Location University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Mirjam Langeveld
Department of Internal Medicine, Division of Endocrinology and Metabolism, Amsterdam UMC Location University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
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 (p p 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.
