TRIGGER ABLATION IN LONG QT TYPE 2 PATIENT

2018 ◽  
Vol 1 (46) ◽  
pp. 28-30
Author(s):  
Agnieszka Wojdyła-Hordyńska ◽  
Grzegorz Hordyński ◽  
Patrycja Pruszkowska-Skrzep ◽  
Oskar Kowalski
Keyword(s):  
Ventricular Tachycardia ◽  
Torsade De Pointes ◽  
Polymorphic Ventricular Tachycardia ◽  
Atrial Pacing ◽  
Long Qt ◽  
Premature Ventricular Contractions ◽  
Cardioverter Defibrillator ◽  
Lqt Syndrome

Implantable cardioverter-defibrillator (ICD) is an indicated therapy for Long QT (LQT) patients after syncope or cardiac arrest survival. Premature ventricular contractions (PVCs) triggers ablation is also a known, yet, still rare, method aiming at ventricular tachycardia or torsade de pointes treatment in LQT syndrome. We present a 21-year-old female with no previous medical history admitted after syncope during cycling. Family history revealed sudden cardiac death in a 36-year-old father of the patient. Genetic test presented a puntiform mutation of KCNH2 gene. Beta-blocker and life vest therapy were introduced. Three months later the patient was admitted due to presyncopy and documented polymorphic ventricular tachycardia initiated by premature ventricular contractions. The clinical PVC triggers ablation was performed and cardioverter-defibrillator implanted. The PVC in the left aortic cusp was successfully ablated, and an ICD was implanted to utilise an atrial pacing. During the next 12-months follow up no VTs were recorded.

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.

Video-Assisted Modified Left Sympathectomy for Long-QT Syndrome

2000 ◽  
Vol 8 (1) ◽  
pp. 52-53 ◽  
Author(s):  
Guo Xing Weng ◽  
Hang Ding ◽  
Juan Qi ◽  
Chun Xuan Xu
Keyword(s):  
Ventricular Tachycardia ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Torsade De Pointes ◽  
Horner’S Syndrome ◽  
Horner's Syndrome ◽  
Long Qt ◽  
Video Assisted ◽  
Qt Syndrome

A 22-year-old female suffering from idiopathic long-QT syndrome complicated by frequent syncope, torsade-de-pointes-type ventricular tachycardia, and asthma, was successfully treated by video-assisted extensive left second and third thoracic sympathetic ganglionectomy, instead of left stellate and first thoracic ganglio-nectomy, to avoid postoperative Horner's syndrome. The QT interval was significantly shortened from 0.6 to 0.43 seconds four days after the surgery. It remained at 0.43 seconds during a 3-month follow-up with no recurrence of tachycardia or syncope.

Polymorphic ventricular tachycardia, ischaemic ventricular fibrillation, and torsade de pointes: importance of the QT and the coupling interval in the differential diagnosis

2021 ◽  
Author(s):  
Raphael Rosso ◽  
Aviram Hochstadt ◽  
Dana Viskin ◽  
Ehud Chorin ◽  
Arie Lorin Schwartz ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Fibrillation ◽  
Qt Interval ◽  
Torsade De Pointes ◽  
Polymorphic Ventricular Tachycardia ◽  
Ectopic Beat ◽  
Long Qt ◽  
Drug Induced ◽  
Long Qt Interval ◽  
Coupling Interval

Abstract Aims Distinctive types of polymorphic ventricular tachycardia (VT) respond differently to different forms of therapy. We therefore performed the present study to define the electrocardiographic characteristics of different forms of polymorphic VT. Methods and results We studied 190 patients for whom the onset of 305 polymorphic VT events was available. The study group included 87 patients with coronary artery disease who had spontaneous polymorphic VT triggered by short-coupled extrasystoles in the absence of myocardial ischaemia. This group included 32 patients who had a long QT interval but nevertheless had their polymorphic VT triggered by ectopic beats with short coupling interval, a subcategory termed ‘pseudo-torsade de pointes] (TdP). For comparison, we included 50 patients who had ventricular fibrillation (VF) during acute myocardial infarction (‘ischaemic VF’ group) and 53 patients with drug-induced TdP (‘true TdP’ group). The QT of patients with pseudo-TdP was (by definition) longer than that of patients with polymorphic VT and normal QT (QTc 491.4 ± 25.2 ms vs. 447.3 ± 55.6 ms, P < 0.001). However, their QT was significantly shorter than that of patients with true TdP (QTc 564.6 ± 75.6 ms, P < 0.001). Importantly, the coupling interval of the ectopic beat triggering the arrhythmia was just as short during pseudo-TdP as during polymorphic VT with normal QT (359.1 ± 38.1 ms vs. 356.6 ± 39.4 ms, P = 0.467) but was much shorter than during true TdP (581.2 ± 95.3 ms, P < 0.001). Conclusions The coupling interval helps discriminate between polymorphic VT that occurs despite a long QT interval (pseudo-TdP) and polymorphic arrhythmias striking because of a long QT (true TdP).

Abstract 15884: Life-threatening Arrhythmias With Autosomal Recessive TECRL Variants

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Gregory Webster ◽  
Elhadi H Aburawi ◽  
Marie Chaix ◽  
Stephanie Chandler ◽  
Roger Foo ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Sudden Death ◽  
Long Qt Syndrome ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Long Qt ◽  
Long Term Follow Up ◽  
Qt Syndrome

Introduction: Sudden death and aborted sudden death have been observed in patients with biallelic variants in TECRL . Phenotypes have only begun to be described and no data are available on medical therapy after long-term follow-up. Methods: An international, multicenter retrospective review was conducted. We report new cases associated with TECRL variants and long-term follow-up from previously published cases. Results: We present 10 cases and 37 asymptomatic heterozygous carriers. Median age at onset of cardiac symptoms was 8 years (range 1-22 years) and cases were followed for an average of 10.3 years (SD 8.3), right censored by death in 3 cases. All patients on metoprolol, bisoprolol or atenolol were transitioned to nadolol or propranolol due to failure of therapy. Phenotypes typical of both long QT syndrome and catecholaminergic polymorphic ventricular tachycardia were observed. We also observed divergent phenotypes in some cases despite identical homozygous variants. None of 37 heterozygous family members had a cardiac phenotype. Conclusions: Patients with biallelic pathogenic TECRL variants present with variable cardiac arrhythmia phenotypes, including those typical of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia. Nadolol and propranolol may be superior beta-blockers in this setting. No cardiac disease or sudden death was present in patients with a heterozygous genotype.

scholarly journals Paediatric/young versus adult patients with long QT syndrome or catecholaminergic polymorphic ventricular tachycardia

2021 ◽  
Author(s):  
Sharen Lee ◽  
Jiandong Zhou ◽  
Kamalan Jeevaratnam ◽  
Wing Tak Wong ◽  
Ian Chi Kei Wong ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Long Qt Syndrome ◽  
Primary Outcome ◽  
Public Hospitals ◽  
Adult Patients ◽  
Polymorphic Ventricular Tachycardia ◽  
Qtc Interval ◽  
Long Qt ◽  
Old Patients ◽  
Qt Syndrome

AbstractIntroductionLong QT syndrome (LQTS) and catecholaminergic ventricular tachycardia (CPVT) are less prevalent cardiac ion channelopathies than Brugada syndrome in Asia. The present study compared paediatric/young and adult patients with these conditions.MethodsThis was a territory-wide retrospective cohort study of consecutive patients diagnosed with LQTS and CPVT attending public hospitals in Hong Kong. The primary outcome was spontaneous ventricular tachycardia/ventricular fibrillation (VT/VF).ResultsA total of 142 LQTS (mean onset age= 27±23 years old) and 16 CPVT (mean presentation age=11±4 years old) patients were included. For LQTS, arrhythmias other than VT/VF (HR=4.67, 95% confidence interval=[1.53-14.3], p=0.007), initial VT/VF (HR=3.25 [1.29-8.16], p=0.012) and Schwartz score (HR=1.90 [1.11-3.26], p=0.020) were predictive of the primary outcome for the overall cohort, whilst arrhythmias other than VT/VF (HR=5.41 [1.36-21.4], p=0.016) and Schwartz score (HR=4.67 [1.48-14.7], p=0.009) were predictive for the adult subgroup (>25 years old; n=58). All CPVT patients presented before the age of 25 but no significant predictors of VT/VF were identified. A random survival forest model identified initial VT/VF, Schwartz score, initial QTc interval, family history of LQTS, initially asymptomatic, and arrhythmias other than VT/VF as the most important variables for risk prediction in LQTS, and initial VT/VF/sudden cardiac death, palpitations, QTc, initially symptomatic and heart rate in CPVT.ConclusionClinical and ECG presentation vary between the pediatric/young and adult LQTS population. All CPVT patients presented before the age of 25. Machine learning models achieved more accurate VT/VF prediction.

scholarly journals Cardioverter-Defibrillator Implantation to Treat Cardiac Fibroma-Induced Ventricular Tachycardia in a 70-Year-Old Woman

2014 ◽  
Vol 41 (3) ◽  
pp. 329-331 ◽  
Author(s):  
Fayaz Ahmad Hakim ◽  
Anil Pandit ◽  
Farouk Mookadam ◽  
Sylvia Mamby
Keyword(s):  
Ventricular Tachycardia ◽  
Implantable Cardioverter Defibrillator ◽  
Clinical Symptoms ◽  
Surgical Excision ◽  
Orthotopic Heart Transplantation ◽  
Implantable Cardioverter Defibrillator Therapy ◽  
Cardiac Fibroma ◽  
Outflow Obstruction ◽  
Cardioverter Defibrillator

Benign cardiac fibroma is rarely reported in adults. Its clinical symptoms are related to outflow obstruction or dysrhythmias. We present the case of a 70-year-old woman who had a syncopal episode from ventricular tachycardia caused by cardiac fibroma. Because of unfavorable tumor anatomy, the patient was not a candidate for surgical excision, and she declined orthotopic heart transplantation. To prevent sudden cardiac death, we placed an implantable cardioverter-defibrillator, and the patient remained well throughout the 2-year follow-up period. To our knowledge, this is the first report of implantable cardioverter-defibrillator therapy to treat an adult patient's unresectable cardiac fibroma.

1175Structural progression increases the risk of ventricular arrhythmias in patients with arrhythmogenic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Chivulescu ◽  
Ø.H Lie ◽  
H Skulstad ◽  
B A Popescu ◽  
R O Jurcut ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Arrhythmias ◽  
Arrhythmogenic Cardiomyopathy ◽  
Cardioverter Defibrillator ◽  
Structural Progression ◽  
Life Threatening ◽  
Arrhythmic Event ◽  
History Of ◽  
Structural Disease

Abstract Background Arrhythmogenic cardiomyopathy (AC) is an inheritable cardiomyopathy with incomplete penetrance, variable phenotype severity and poorly described disease progression. It is characterized by high risk of life-threatening ventricular arrhythmias and sudden cardiac death in young individuals. Risk stratification and selection of patients presenting without history of life-threatening arrhythmic events for cardioverter-defibrillator implantation in primary prevention remains challenging. Purpose We aimed to assess the impact of disease progression on arrhythmic outcomes in AC patients. Methods We included consecutive AC probands and mutation-positive family members with at least one complete follow-up evaluation. Echocardiographic and electrical parameters were defined according to the 2010 Revised Task Force criteria at inclusion and at last follow-up. Structural progression was defined as development of new echocardiographic diagnostic criteria. Electrical progression was defined as the development of new diagnostic depolarization, repolarization and/or premature ventricular complex count criteria during follow-up. Non-sustained ventricular tachycardia or ventricular tachycardia occurring during follow-up defined incident ventricular arrhythmic events. Results We included a total of 144 patients (48% female, 47% probands, 40±16 years old). At inclusion, 54 patients (37%) had a history of arrhythmic events, 30 patients (21%) had overt structural disease and 114 (79%) had no or minor structural disease. During 7.0 (IQR: 4.5 to 9.4) years of follow-up, 49 patients (43%) with no or minor structural disease at inclusion developed new structural criteria being defined as progressors. Among 80 participants with no or minor structural disease and no arrhythmic history at inclusion, a first arrhythmic event occurred in 14 (17%). The incidence of arrhythmic events was higher in progressors (11/27, 41%) than in non-progressors (3/53, 6%) (p<0.001) (Figure). Structural progression was associated with higher risk of first arrhythmic events during follow-up when adjusted for sex, age at inclusion and follow-up duration, independent of electrical progression (7.6, 95% CI [1.5, 37.2], P=0.01). Incident arrhythmic events distribution Conclusion Almost half of patients without overt structural cardiac disease at genetic diagnosis develop new structural criteria during 7 years follow-up and 17% experienced their first ventricular arrhythmic event. Structural progression was independently associated with ventricular arrhythmic events during follow-up. These findings highlight the increased risk of arrhythmias when structural abnormalities are detected. Their finding may initiate the evaluation for primary prevention cardioverter-defibrillator implantation.

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