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 tachyarrhythmia (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.
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 tachyarrhythmia (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.
Authors: Annabella Braschi; Vincenzo C Francavilla; Maurizio G Abrignani; Lorenzo Todaro; Giuseppe Francavilla Journal: Ann Noninvasive Electrocardiol Date: 2012-04 Impact factor: 1.468
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