Heart Rate Variability and QT Changes in Children with Hemophilia

Background: Hemophilia-A is a sex-related hereditary bleeding disorder, like the general populations, mortality from cardiovascular diseases is currently responsible for one-third of deaths in hemophilia patients. Method: A case controlled study was carried out on fifty children; twenty five male with hemophilia-A compared to twenty five healthy male children.12 lead surface ECG, 24 hour holter monitoring and basic echocardiography assessment were done for all participating children. Results: ECG recording revealed that increased heart rate (b/min), QTc/msec, the maximum and QT dispersion in the hemophilia children vs. the control, increased QT dispersion by 24 hour holter in hemophilia children. Reduced time domain parameters for HRV (ms) in hemophilia children were compared to the healthy controls. A significant difference was observed between the diseased and healthy group in the number and frequency of the subject with increased QTc dispersion more than 70 ms detected by ECG, but not by holter. Conclusion: There was an increase in QT dispersion in children with hemophilia that put them at risk for developing dysrhythmia, sympathy-vagal imbalance detected by the presence of depressed HRV, and also exposed them to cardiac morbidities and sudden cardiac death.