Left thorascopic sympathectomy for refractory long QT syndrome in children

2011 ◽  
Vol 8 (5) ◽  
pp. 455-459 ◽  
Author(s):  
Steven W. Hwang ◽  
Jonathan G. Thomas ◽  
William E. Whitehead ◽  
Daniel J. Curry ◽  
Robert C. Dauser ◽  
...  
Keyword(s):  
Long Qt Syndrome ◽  
Pediatric Patients ◽  
Relevant Literature ◽  
Long Qt ◽  
Thoracic Sympathectomy ◽  
Life Threatening ◽  
Qt Syndrome ◽  
Congenital Long Qt Syndrome

Congenital long QT syndrome (LQTS) is a rare genetic ion transmembrane disorder that has been associated with multiple various genetic mutations including life-threatening cardiac arrhythmias and sudden death. Left thorascopic sympathectomy is an effective treatment for patients who are refractory to medical therapy or who need frequent epicardial internal cardiodefibrillator intervention. Although there is substantial literature about this therapy in adults, few reports detail the outcomes in children who undergo left thorascopic sympathectomies to treat LQTS. The authors report the successful use of a left thoracic sympathectomy for the treatment of an 11-year-old girl who had persistently symptomatic LQTS, even after implantation of an automatic cardioverter-defibrillator. The patient remained clinically stable without arrhythmias through 6 months' of follow-up. The authors also reviewed the relevant literature and found that it suggests that 77% of patients will have immediate resolution of their symptoms/arrhythmias after the procedure. When the outcome definition was broadened to include patients who had only 1 or 2 cardiac episodes in the follow-up period, 88% of cases were considered successful. The results of this case study and literature review suggest that left thorascopic sympathectomy is a safe and effective approach for treating pediatric patients with LQTS.

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 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 Long-term proarrhythmic pharmacotherapy among patients with congenital long QT syndrome and risk of arrhythmia and mortality

2019 ◽  
Vol 40 (37) ◽  
pp. 3110-3117 ◽  
Author(s):  
Peter E Weeke ◽  
Jesper S Kellemann ◽  
Camilla Bang Jespersen ◽  
Juliane Theilade ◽  
Jørgen K Kanters ◽  
...  
Keyword(s):  
Ventricular Arrhythmia ◽  
Drug Treatment ◽  
Long Qt Syndrome ◽  
Ventricular Arrhythmias ◽  
Torsades De Pointes ◽  
Long Qt ◽  
All Cause Mortality ◽  
Qt Syndrome ◽  
Congenital Long Qt Syndrome

Abstract Aims It is Class I recommendation that congenital long QT syndrome (cLQTS) patients should avoid drugs that can cause torsades de pointes (TdP). We determined use of TdP risk drugs after cLQTS diagnosis and associated risk of ventricular arrhythmia and all-cause mortality. Methods and results Congenital long QT syndrome patients (1995–2015) were identified from four inherited cardiac disease clinics in Denmark. Individual-level linkage of nation-wide registries was performed to determine TdP risk drugs usage (www.crediblemeds.org) and associated risk of ventricular arrhythmias and all-cause mortality. Risk analyses were performed using Cox-hazards analyses. During follow-up, 167/279 (60%) cLQTS patients were treated with a TdP risk drug after diagnosis. Most common TdP risk drugs were antibiotics (34.1%), proton-pump inhibitors (15.0%), antidepressants (12.0%), and antifungals (10.2%). Treatment with a TdP risk drug decreased 1 year after diagnosis compared with 1 year before (28.4% and 23.2%, respectively, P < 0.001). Five years after diagnosis, 33.5% were in treatment (P < 0.001). Risk factors for TdP risk drug treatment were age at diagnosis (5-year increment) [hazard ratio (HR) = 1.07, confidence interval (CI) 1.03–1.11] and previous TdP risk drug treatment (HR = 2.57, CI 1.83–3.61). During follow-up, nine patients were admitted with ventricular arrhythmia (three were in treatment with a TdP risk drug). Eight patients died (four were in treatment with a TdP risk drug). No significant association between TdP risk drug use and ventricular arrhythmias or all-cause mortality was found (P = 0.53 and P = 0.93, respectively), but events were few. Conclusion Torsades de pointes risk drug usage was common among cLQTS patients after time of diagnosis and increased over time. A critical need for more awareness in prescribing patterns for this high-risk patient group is needed.

scholarly journals Management of Long QT Syndrome in Women Before, During, and After Pregnancy

2021 ◽  
Vol 15 ◽  
Author(s):  
Caroline Taylor ◽  
Bruce S Stambler
Keyword(s):  
Long Qt Syndrome ◽  
Postpartum Period ◽  
Torsades De Pointes ◽  
Close Monitoring ◽  
Cardiac Events ◽  
Long Qt ◽  
Life Threatening ◽  
Β Blocker ◽  
Qt Syndrome ◽  
Congenital Long Qt Syndrome

Congenital long QT syndrome (LQTS) is a primary genetic and electrical disorder that increases risk for torsades de pointes, syncope, and sudden death. Post-pubertal women with LQTS require specialized multidisciplinary management before, during, and after pregnancy involving cardiology and obstetrics to reduce risk for cardiac events in themselves and their fetuses and babies. The risk of potentially life-threatening events is lower during pregnancy but increases significantly during the 9-month postpartum period. Treatment of women with LQTS with a preferred β-blocker at optimal doses along with close monitoring are indicated throughout pregnancy and during the high-risk postpartum period.

scholarly journals Genotype-specific QT correction for heart rate and the risk of life-threatening cardiac events in adolescents with congenital long-QT syndrome

Heart Rhythm ◽  
2011 ◽  
Vol 8 (8) ◽  
pp. 1207-1213 ◽  
Author(s):  
Alon Barsheshet ◽  
Derick R. Peterson ◽  
Arthur J. Moss ◽  
Peter J. Schwartz ◽  
Elizabeth S. Kaufman ◽  
...  
Keyword(s):  
Heart Rate ◽  
Long Qt Syndrome ◽  
Cardiac Events ◽  
Long Qt ◽  
Life Threatening ◽  
Qt Syndrome ◽  
Congenital Long Qt Syndrome
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