TY - JOUR
T1 - Outcome by sex in patients with long qt syndrome with an implantable cardioverter defibrillator
AU - Younis, Arwa
AU - Aktas, Mehmet K.
AU - Rosero, Spencer
AU - Kutyifa, Valentina
AU - Polonsky, Bronislava
AU - McNitt, Scott
AU - Sotoodehnia, Nona
AU - Kudenchuk, Peter
AU - Rea, Thomas D.
AU - Arking, Dan E.
AU - Goldenberg, Ilan
AU - Zareba, Wojciech
N1 - Funding Information:
This study was supported by National Institutes of Health Grant HL123483 and by American Heart Association Grant 19SFRN34830063.
Publisher Copyright:
© 2020 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley. T.
PY - 2020
Y1 - 2020
N2 - BACKGROUND: Sex differences in outcome have been reported in patients with congenital long QT syndrome. We aimed to report on the incidence of time-dependent life-threatening events in male and female patients with long QT syndrome with an implantable cardioverter defibrillator (ICD). METHODS AND RESULTS: A total of 60 patients with long QT syndrome received an ICD for primary or secondary prevention indi-cations. Life-threatening events were evaluated from the date of ICD implant and included ICD shocks for ventricular tachycar-dia, ventricular fibrillation, or death. ICDs were implanted in 219 women (mean age 38±13 years), 46 girls (12±5 years), 55 men (43±17 years), and 40 boys (11±4 years). Mean follow-up post-ICD implantation was 14±6 years for females and 12±6 years for males. At 15 years of follow-up, the cumulative probability of life-threatening events was 27% in females and 34% in males (log-rank P=0.26 for the overall difference). In the multivariable Cox model, sex was not associated with significant differences in risk first appropriate ICD shock (hazard ratio, 0.83 female versus male; 95% CI, 0.52–1.34; P=0.47). Results were similar when stratified by age and by genotype: long QT syndrome type 1 (LQT1), long QT syndrome type 2 (LQT2), and long QT syndrome type 3 (LQT3). Incidence of inappropriate ICD shocks was higher in males versus females (4.2 versus 2.7 episodes per 100 patient-years; P=0.018), predominantly attributed to atrial fibrillation. The first shock did not terminate ventricular tachycardia/ ventricular fibrillation in 48% of females and 62% of males (P=0.25). CONCLUSIONS: In patients with long QT syndrome with an ICD, the risk and rate of life-threatening events did not significantly differ between males and females regardless of ICD indications or genotype. In a substantial proportion of patients with long QT syndrome, first shock did not terminate ventricular tachycardia/ventricular fibrillation.
AB - BACKGROUND: Sex differences in outcome have been reported in patients with congenital long QT syndrome. We aimed to report on the incidence of time-dependent life-threatening events in male and female patients with long QT syndrome with an implantable cardioverter defibrillator (ICD). METHODS AND RESULTS: A total of 60 patients with long QT syndrome received an ICD for primary or secondary prevention indi-cations. Life-threatening events were evaluated from the date of ICD implant and included ICD shocks for ventricular tachycar-dia, ventricular fibrillation, or death. ICDs were implanted in 219 women (mean age 38±13 years), 46 girls (12±5 years), 55 men (43±17 years), and 40 boys (11±4 years). Mean follow-up post-ICD implantation was 14±6 years for females and 12±6 years for males. At 15 years of follow-up, the cumulative probability of life-threatening events was 27% in females and 34% in males (log-rank P=0.26 for the overall difference). In the multivariable Cox model, sex was not associated with significant differences in risk first appropriate ICD shock (hazard ratio, 0.83 female versus male; 95% CI, 0.52–1.34; P=0.47). Results were similar when stratified by age and by genotype: long QT syndrome type 1 (LQT1), long QT syndrome type 2 (LQT2), and long QT syndrome type 3 (LQT3). Incidence of inappropriate ICD shocks was higher in males versus females (4.2 versus 2.7 episodes per 100 patient-years; P=0.018), predominantly attributed to atrial fibrillation. The first shock did not terminate ventricular tachycardia/ ventricular fibrillation in 48% of females and 62% of males (P=0.25). CONCLUSIONS: In patients with long QT syndrome with an ICD, the risk and rate of life-threatening events did not significantly differ between males and females regardless of ICD indications or genotype. In a substantial proportion of patients with long QT syndrome, first shock did not terminate ventricular tachycardia/ventricular fibrillation.
KW - Female
KW - Implantable cardioverter defibrillator
KW - Life threatening events
KW - Long QT syndrome
KW - Sex
UR - http://www.scopus.com/inward/record.url?scp=85092654848&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85092654848&partnerID=8YFLogxK
U2 - 10.1161/JAHA.120.016398
DO - 10.1161/JAHA.120.016398
M3 - Article
C2 - 33021160
AN - SCOPUS:85092654848
VL - 9
JO - Journal of the American Heart Association
JF - Journal of the American Heart Association
SN - 2047-9980
IS - 19
M1 - e016398
ER -