scholarly journals Outcome by Sex in Patients With Long QT Syndrome With an Implantable Cardioverter Defibrillator

2020 ◽  
Vol 9 (19) ◽  
Author(s):  
Arwa Younis ◽  
Mehmet K. Aktas ◽  
Spencer Rosero ◽  
Valentina Kutyifa ◽  
Bronislava Polonsky ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Fibrillation ◽  
Implantable Cardioverter Defibrillator ◽  
Long Qt Syndrome ◽  
Syndrome Type ◽  
Long Qt ◽  
Cardioverter Defibrillator ◽  
Life Threatening ◽  
Qt Syndrome

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 indications. Life‐threatening events were evaluated from the date of ICD implant and included ICD shocks for ventricular tachycardia, 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.

scholarly journals Pregnancy in long QT syndrome with implantable cardioverter-defibrillator. A case report and review of the literature

2014 ◽  
Vol 20 (1) ◽  
pp. 1-4
Author(s):  
Sandra Kutkiene˙ ◽  
Lina Gumbiene ◽  
Juratę Aganauskiene ◽  
Rima Steponenienea ◽  
Germanas Marinskisa ◽  
...  
Keyword(s):  
Implantable Cardioverter Defibrillator ◽  
Long Qt Syndrome ◽  
Ventricular Arrhythmias ◽  
Long Qt ◽  
Cardioverter Defibrillator ◽  
Pregnancy And Delivery ◽  
Life Threatening ◽  
Qt Syndrome ◽  
Congenital Long Qt Syndrome

Summary Implantable cardioverters-defibrillators have decreasedmorbidity and mortality as well as improved quality of life in patients with life-threatening cardiac arrhythmias and allowed an increasing number of young women to reach their reproductive years. New questions and tasks arise for medical professionals as to organize appropriate management of these patients, because little is known regarding the risk and outcomes of such pregnancies. The aim of this report is to describe our centre’s first experience of pregnancy and delivery management in patient with an implantable cardioverter-defibrillator as primary prevention of ventricular arrhythmias in congenital long QT syndrome.

scholarly journals Subcutaneous and transvenous implantable cardioverter defibrillator in high-risk long-QT syndrome type 3 associated with Val411Met mutation in SCN5A

2020 ◽  
Vol 22 (5) ◽  
pp. 238-241
Author(s):  
Yasuhiro Yokoyama ◽  
Takeshi Aiba ◽  
Nobuhiko Ueda ◽  
Kenzaburo Nakajima ◽  
Tsukasa Kamakura ◽  
...  
Keyword(s):  
High Risk ◽  
Implantable Cardioverter Defibrillator ◽  
Long Qt Syndrome ◽  
Syndrome Type ◽  
Long Qt ◽  
Cardioverter Defibrillator ◽  
Qt Syndrome ◽  
Type 3

Abstract 3094: Implantable Cardioverter Defibrillator Therapy for Congenital Long QT Syndrome: a Single Center Experience

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Justin M Horner ◽  
Masayoshi Kinoshita ◽  
Carla Haglund ◽  
Paul Friedman ◽  
Michael J Ackerman
Keyword(s):  
Secondary Prevention ◽  
Implantable Cardioverter Defibrillator ◽  
Long Qt Syndrome ◽  
Long Qt ◽  
Similar Frequency ◽  
Cardioverter Defibrillator ◽  
Appropriate Therapy ◽  
Qt Syndrome ◽  
To Receive

Background: Long QT syndrome (LQTS) is a potentially lethal yet highly treatable channelopathy underscored by marked heterogeneity that necessitates evidence-based and individualized therapeutic approaches. One such therapy is the implantable cardioverter defibrillator (ICD). Here, we sought to determine the percentage of patients (pts) with LQTS that received an ICD in our LQTS clinic and its short term outcomes. Methods: An IRB-approved, retrospective analysis of the electronic medical records of 167 LQT1, 113 LQT2, and 29 LQT3 pts evaluated in our LQTS Clinic with subset analysis of those implanted with an ICD in our institution from January 2000 to June 2007. Results: Forty-eight pts (15% of total, 33 females, 14 LQT1, 20 LQT2, and 14 LQT3) received an ICD. The average age at implant was 30 ± 12 years (range 8 – 52 years). Nearly two-thirds received an ICD as primary prevention. The QTc exceeded 500 ms in 18 (37.5%). With an average follow-up of 42 ± 32 months, 9 (19%) have had an appropriate, VF-terminating therapy including 3 with LQT1 and 6 with LQT2. Notably, 5 of the 6 LQT2 females have already received an appropriate ICD therapy whereas none of the LQT3 pts have received one. Secondary prevention indications (p = 0.006), LQT2 genotype (p = 0.03), QTc > 500 ms (p = 0.001), and surprisingly a negative family history (p = 0.0005) predicted those ICD pts most likely to receive an appropriate therapy. Importantly, none of the non-ICD treated pts (> 250) have died a LQT-related death during the same follow-up. Conversely, 9 pts have experienced an inappropriate shock including 6 with LQT3 and males were more likely to receive inappropriate therapies (p = 0.002). Conclusions: The majority of pts with LQTS can be treated effectively without an ICD. In this study, potentially life-saving therapies have been rendered so far at a 5– 6%/year rate. Inappropriate shocks occurred with similar frequency. Secondary prevention, genotype, and QTc predicted those ICD recipients most likely to receive appropriate therapy. Although LQT3 status is commonly associated with an ICD recommendation, so far none of the LQT3 positive ICD recipients have needed their device. Instead, the greatest “save” rate has occurred among the LQT2 positive females deemed at high risk.

scholarly journals Primary prevention with the implantable cardioverter-defibrillator in high-risk long-QT syndrome patients

EP Europace ◽  
2018 ◽  
Vol 21 (2) ◽  
pp. 339-346 ◽  
Author(s):  
Yitschak Biton ◽  
Spencer Rosero ◽  
Arthur J Moss ◽  
Ilan Goldenberg ◽  
Valentina Kutyifa ◽  
...  
Keyword(s):  
Primary Prevention ◽  
High Risk ◽  
Implantable Cardioverter Defibrillator ◽  
Long Qt Syndrome ◽  
Long Qt ◽  
Cardioverter Defibrillator ◽  
Qt Syndrome

Inappropriate shock delivery by an implantable cardioverter-defibrillator due to electrical interference with a refrigerator in a 4-year-old child

2021 ◽  
pp. 1-3
Author(s):  
Sezen Gulumser Sisko ◽  
Hasan Candas Kafali ◽  
Yakup Ergul
Keyword(s):  
Ventricular Fibrillation ◽  
Implantable Cardioverter Defibrillator ◽  
Inappropriate Shock ◽  
Long Qt ◽  
Shock Delivery ◽  
Cardioverter Defibrillator ◽  
Qt Syndrome ◽  
Implantable Cardioverter Defibrillator Shock ◽  
Defibrillator Shock ◽  
Electrical Interference

Abstract We report a patient with long QT syndrome who received an inappropriate implantable cardioverter-defibrillator shock due to electrical interference from a refrigerator. This electrical interference was mistakenly detected as an episode of ventricular fibrillation and ended with an inappropriate delivery of shock without any warning symptoms before.

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