Tp-e interval and Tp-e/QT ratio and their association with left ventricular diastolic dysfunction in Fabry disease without left ventricular hypertrophy

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.