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Tp-e interval and Tp-e/QT ratio and their association with left ventricular diastolic dysfunction in Fabry disease
Mustafa Yenerçağ1, Uğur Arslan1
1Department of Cardiology, University of Health Sciences Samsun Training and Research Hospital, Samsun, Turkey.
Insights
Early-stage Fabry disease patients show prolonged repolarization parameters, including the Tp-e interval and Tp-e/QT ratios. These findings correlate with left ventricular diastolic dysfunction, suggesting subclinical cardiac involvement.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Fabry disease, a genetic lysosomal storage disorder, can lead to early diastolic dysfunction and cardiovascular complications.
- Left ventricular hypertrophy (LVH) may develop later, but early subclinical cardiac changes are critical to identify.
- Repolarization parameters on electrocardiography (ECG) may offer insights into early cardiac involvement.
Purpose of the Study:
- To evaluate ECG repolarization parameters in early-stage Fabry disease patients without LVH.
- To investigate the relationship between these repolarization parameters and left ventricular diastolic dysfunction.
Main Methods:
- A cross-sectional study comparing 23 newly diagnosed Fabry patients with 20 healthy controls.
- Fabry disease diagnosis confirmed by leukocyte enzyme activity and genetic analysis.
- ECG parameters (Tp-e, QT, QTc, Tp-e/QT, Tp-e/QTc) and echocardiographic diastolic function (E/e') were assessed.
Main Results:
- Fabry patients exhibited significantly prolonged Tp-e interval, Tp-e/QT, and Tp-e/QTc ratios compared to controls (p < 0.001).
- A significant positive correlation was observed between Tp-e interval and E/e' ratio (r=0.626, p=0.003).
- A significant positive correlation was also found between Tp-e/QTc ratio and E/e' ratio (r=0.578, p=0.008) in Fabry patients.
Conclusions:
- Prolonged repolarization parameters (Tp-e interval, Tp-e/QT, Tp-e/QTc) are evident in pre-hypertrophic Fabry patients.
- These repolarization abnormalities correlate significantly with left ventricular diastolic dysfunction.
- Findings suggest early, subclinical cardiac involvement in Fabry disease, detectable through ECG repolarization analysis.
Introduction:
In the early stage of the Fabry disease, diastolic dysfunction can occur before left ventricular hypertrophy (LVH). Cardiovascular complications, most of which are seen as malignant arrhythmias and heart failure, are the leading causes of death in these patients. The aim of the present study was to assess repolarization parameters of Fabry patients in early stage (without LVH) and to show the relationship between these parameters and left ventricular diastolic dysfunction.
Methods:
This cross-sectional single center study was carried out with newly diagnosed 23 Fabry patients and 20 healthy individuals, between April 2016 and September 2019. Diagnosis of Fabry disease was based on a measurement of enzyme activity in leukocytes and was confirmed by genetic analysis. Basic, demographic and clinical features were reviewed. The risk of ventricular arrhythmia was evaluated by calculating the electrocardiographic, the Tp-e and QT interval, corrected QT (QTc), Tp-e/QT and Tp-e/QTc ratios. Left ventricular systolic and diastolic dysfunctions were evaluated using echocardiography.
Results:
Tp-e interval (86.9 ± 6.2 vs. 73.8 ± 6.3 ms; p < 0.001), Tp-e/QT ratio (0.23 ± 0.008 vs. 0.21 ± 0.01; p < 0.001) and Tp-e/QTc ratio (0.21 ± 0.007vs. 0.18 ± 0.017; p < 0.001) were significantly higher in Fabry patients than the control group. There was a significant positive correlation between Tp-e interval and E/e' ratio (r = 0.626, p = 0.003) and Tp-e/QTc ratio and E/e' ratio (r = 0.578, p = 0.008) in Fabry patients.
Conclusion:
Our study showed that the Tp-e interval, Tp-e/QT ratio, and Tp-e/QTc ratio, which are evaluated electrocardiographically in patients with pre-hypertrophic Fabry patients, are prolonged compared to normal healthy individuals. The most significant finding was the positive correlation found between repolarization parameters and LV diastolic dysfunction. These results may be indicative of an early subclinical cardiac involvement in Fabry patients, considering the diastolic dysfunction severity.
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