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ECG parameters to detect cardiac involvement in Fabry disease
Yu Wang1, Jie Kong1, Jun Wang2
1Division of Cardiology, Department of Medicine, The First Affiliated Hospital of Soochow University, Suzhou, China.
Insights
Electrocardiogram (ECG) parameters like QRS width and Tp-e/QT can assess cardiac involvement in Fabry disease. These ECG changes correlate with disease severity, aiding in treatment timing and monitoring.
Area of Science:
- Cardiology
- Genetics
- Biochemistry
Background:
- Fabry disease involves globotriaosylceramide accumulation, leading to cardiac dysfunction and mortality.
- Cardiac involvement is a major complication, impacting patient prognosis.
Purpose of the Study:
- Investigate electrocardiographic (ECG) changes in various Fabry disease stages.
- Evaluate ECG parameters for assessing cardiac involvement severity.
Main Methods:
- Recruited 62 Fabry disease patients and 45 healthy controls.
- Performed ECG, echocardiography, and cardiac magnetic resonance (CMR) assessments.
- Classified patients into four groups based on cardiac involvement severity.
Main Results:
- Tp-e/QT ratio decreased, and Sokolow-Lyon index increased with disease progression.
- QRS width and Tp-e/QT showed significant association with cardiac involvement severity.
Conclusions:
- QRS width and Tp-e/QT are valuable biomarkers for Fabry disease cardiac involvement.
- These ECG parameters can guide intervention timing and assess treatment response.
Background:
Cardiac 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.
Methods:
62 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.
Results:
Our 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.
Conclusions:
Our 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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