Background: Patients with hypertrophic cardiomyopathy (HCM) associated with a deletion of lysine 183 (K183del) in the cardiac troponin I (cTnI) gene suffer sudden cardiac death at all ages. However, the correlation between QT variables and sudden cardiac death in these patients remains uncertain.
Hypothesis: We evaluated the correlation between QT variables and sudden cardiac death and/or ventricular tach-yarrhythmia (SCD/VT) in patients with HCM associated with the cTnI mutation.
Methods: We analyzed 10 probands with HCM associated with the cTnI gene K183del and their family members. The subjects were divided into three groups: Group A (n = 7), mutation carriers with SCD/VT; Group B (n = 16), mutation carriers without SCD/VT; Group C (n = 24), no mutation carriers. QT intervals were corrected using Bazett's formula.
Results: Maximum QTc and corrected QT dispersion were significantly longer in Groups A and B than in Group C. However, there were no differences in either parameter between Groups A and B. On the contrary, the peak-to-end interval of T wave/QT interval in V5 (Tpe) in Group A was significantly longer than that in Groups B and C. Logistic regression analysis revealed that Tpe was a good clinical predictor for SCD/VT in patients with HCM in this study.
Conclusions: These results suggest that Tpe rather than QT dispersion may be one of the best predictors for SCD/VT in patients with HCM associated with the K183del mutation in the cTnI gene.