ArticleBMC cardiovascular disorders2026
ECG parameters to detect cardiac involvement in Fabry disease.
Article in BMC cardiovascular disorders, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundCardiac involvement of the disease is a leading cause of death and disability in Fabry disease characterized by pathological accumulation of globotriaosylceramide (Gb3) and lyso-globotriaosylceramide (lyso-Gb3) in multiple organs. In this study, we sought to investigate the electrocardiographic (ECG) changes in different clinical stages and evaluate the value of these parameters in assessing cardiac involvement.
methods62 patients with Fabry disease and 45 healthy controls were recruited in this study. ECG assessment, echocardiographic assessment and cardiac magnetic resonance (CMR) were recorded at rest in the same day. We divided Fabry disease patients into 4 groups according to echocardiographic assessment and CMR: Pre-detectable group, Non-hypertrophic group, Hypertrophic and pre-fibrotic group as well as Hypertrophic and fibrotic group.
resultsOur results showed that Tp-e/QT was significantly decreased and Sokolow-Lyon index was markedly increased following the development of disease. QRS width and Tp-e/QT was associated with the severity of cardiac involvement in Fabry disease.
conclusionsOur results demonstrated that QRS width and Tp-e/QT were associated with the severity of cardiac involvement in patients with Fabry disease and contributed to define the optimal intervention timepoint and assess the severity of cardiac involvement and response to disease-specific therapies.
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