scholarly journals Evaluation of ventricular tachycardia inducibility after implementation of a standardized programmed ventricular stimulation protocol in patients with repaired Tetralogy of Fallot

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
L Herrador Galindo ◽  
J Francisco Pascual ◽  
A Santos Ortega ◽  
J Perez Rodon ◽  
B Benito ◽  
...  
Keyword(s):  
Risk Factors ◽  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Tetralogy Of Fallot ◽  
Cardiac Death ◽  
Clinical Information ◽  
Icd Implantation ◽  
Repaired Tetralogy Of Fallot ◽  
Drive Trains

Abstract Introduction The electrophysiologic (EP) evaluation with programmed electrical stimulation (PVS) is generally recommended in patients with repaired Tetralogy of Fallot and additional risk factors for sudden cardiac death. Nevertheless, different PVS protocols have been described. The aim of our study was to evaluate the differences in ventricular tachycardia (VT) inducibility of patients with TOF after the implementation of a standard PVS protocol in the EP laboratory of a Congenital Heart Disease reference center. Methods All patients with repaired TOF who underwent an EP study with PVS between January 2001 and October 2020 were included. The new standardized PVS protocol was performed in 2 ventricular sites (apex and outflow tract) with 3 drive trains (cycle lengths 400, 500 and 600ms) and up to 3 extrastimuli. In absence of VT induction, the protocol was repeated under isoprenaline infusion. This new protocol was implemented since January 2012. Non protocolized PVS studies before 2012 were defined as “Non-standardized”. Baseline clinical information about symptoms and previous arrhythmias was recorded as well as electrocardiogram, echocardiogram and cardiac MRI parameters. Finally, the follow-up events (ICD implantation, sudden cardiac death, global mortality, arrythmias and ICD therapies) were also retrospective recorded. Results A total of 154 EP studies with PVS were performed in 128 patients with repaired TOF. 31 of them were performed before the 1st January 2012 (non-standardized PVS) and 112 were performed with the new standardized protocol. The median follow-up was 6,5 years. Both groups had similar baseline characteristics except LVEF and RVEF, that were lower in the “Non-standardized PVS” group. There were no differences between the ventricular tachycardia inducibility of both protocols (22,3% vs 33,3%; p=0,162). The risk factors for VT inducibility were the QRS length (184,46ms vs 169,34 ms; p=0,038), the RVEF (36,25% vs 43,79; p=0,0007), the presence of ventricular ectopia (VE) (38,5% vs 20,0%; p=0,024) and previous VT (35,9% vs 13,9%; p=0,003). VT induction during EP study was related with ICD implantation (71,8% vs 21,7%, p≤0,001), VT (30,8% vs 20%, p<0,001) and all kind of arrythmias (VT, non-sustained VT, VE and auricular flutter) (41% vs 21,7%, p=0,005) during follow-up. A total of 6 deaths (1 in the group with induced VT and 5 in the group with non-induced VT) were recorded. Conclusions The implementation of a standardized and more complete PVS protocol in patients with repaired TOF has not shown differences in the experience of our center. The risk factors for VT inducibility were the QRS length, the RVEF, the presence of ventricular ectopia and previous VT, which have also been reported as risk factors for sudden cardiac death in previous studies. The presence of VT induction entailed more ICD implantation and more arrythmias at follow-up. FUNDunding Acknowledgement Type of funding sources: None.

5204Primary prevention of sudden cardiac death in patients with tetralogy of Fallot with implantable cardioverter defibrillator: insights from the DAI-T4F study

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
V Waldmann ◽  
A Bouzeman ◽  
F Bessiere ◽  
F Labombarda ◽  
M Ladouceur ◽  
...  
Keyword(s):  
Risk Factors ◽  
Ventricular Tachycardia ◽  
Primary Prevention ◽  
Sudden Cardiac Death ◽  
Tetralogy Of Fallot ◽  
Cardiac Death ◽  
Cardioverter Defibrillator ◽  
Programmed Ventricular Stimulation ◽  
Ventricular Stimulation

Abstract Background Ventricular arrhythmias and sudden death are feared late complications in patients with tetralogy of Fallot. Selection of candidates for primary prevention implantable cardioverter defibrillator (ICD) remains challenging in this population. Non-sustained ventricular tachycardia (NSVT), altered left ventricular ejection fraction (LVEF), positive programmed ventricular stimulation, and enlarged QRS are currently used for risk stratification. Purpose To identify high-risk patients with tetralogy of Fallot in the setting of primary prevention of sudden cardiac death. Methods The DAI-T4F study is a large ongoing national French registry including all patients with tetralogy of Fallot and ICD (NCT03837574). Information have been collected prospectively since 2010 with annual update. Baseline patient characteristics and clinical events during the follow-up were analyzed with central adjudication. Cox proportional hazard models were used to identify factors associated with appropriate ICD therapies. Results Among 134 patients enrolled, 47 (35.1%) underwent ICD implantation for primary prevention (median age 49.1 years, 76.6% males). At baseline, 20 (42.6%) patients had NSVT, 17 (36.2%) had severe altered LVEF ≤35%, 16 (34.0%) had positive programmed ventricular stimulation, and 16 (34.0%) had QRS duration ≥180ms. Overall, 20 (42.6%), 15 (31.9%), and 6 (12.8%) patients had respectively one, two, or ≥ three of these risk factors. Six (12.8%) patients were implanted for other indications. During a median (IQR) follow-up duration of 5.3 (2.1–8.0) years, 14 (29.8%) patients had at least one appropriate ICD therapy. The annual incidence of appropriate ICD therapies were 2.8%, 4.6%, 6.3%, and 8.6% in patients with none, one, two, or ≥ three of these factors (p for trend = 0.145). None of predictors, considered isolated, was significantly associated with ICD appropriate therapies. Patients with non-sustained ventricular tachycardia (NSVT) and positive programmed ventricular stimulation had a significant increased risk of ICD appropriate therapies (HR=3.8, 95% CI: 1.1–14.3, p=0.035), as well as patients with NSVT and QRSd ≥180 ms (HR=7.2, 95% CI: 1.6–32.7, p=0.003). No patient with severe altered LVEF without other risk factor had appropriate ICD therapy. Patients with congestive heart failure and/or altered LVEF had a higher risk of non-sudden death or cardiac transplantation (HR=14.4, 95% CI: 1.8–112.7, p<0.001). Seventeen (36.2%) patients experienced at least one ICD-related complication. Conclusions Our data illustrate that specific risk stratification and primary prevention for sudden cardiac death in patients with tetralogy of Fallot may be improved. The value of a severely altered LVEF appears low in the absence of other risk factors, and combination of different predictors is essential. The high rate of complications as well as consideration of competing risk situation have to be integrated in the benefit-risk equation.

scholarly journals 1228 New predictors of sudden cardiac death in hypertrophic cardiomyopathy

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D M Adamczak ◽  
A Rogala ◽  
M Antoniak ◽  
Z Oko-Sarnowska
Keyword(s):  
Risk Factors ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Volume Index ◽  
Lv Function ◽  
Icd Implantation ◽  
Significant Difference

Abstract BACKGROUND Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium. HCM is the most common cause of sudden cardiac death (SCD) in young people and competitive athletes due to fatal ventricular arrhythmias. However, in most patients, HCM has a benign course. That is why it is of utmost importance to properly evaluate patients and identify those who would benefit from a cardioverter-defibrillator (ICD) implantation. The HCM SCD-Risk Calculator is a useful tool for estimating the risk of SCD. The parameters included in the model at evaluation are: age, maximum left ventricular (LV) wall thickness, left atrial (LA) dimension, maximum gradient in left ventricular outflow tract, family history of SCD, non-sustained ventricular tachycardia (nsVT) and unexplained syncope. Nevertheless, there is potential to improve and optimize the effectiveness of this tool in clinical practice. Therefore, the following new risk factors are proposed: LV global longitudinal strain (GLS), LV average strain (ASI) and LA volume index (LAVI). GLS and ASI are sensitive and noninvasive methods of assessing LV function. LAVI more accurately characterizes the size of the left atrium in comparison to the LA dimension. METHODS 252 HCM patients (aged 20-88 years, of which 49,6% were men) treated in our Department from 2005 to 2018, were examined. The follow-up period was 0-13 years (average: 3.8 years). SCD was defined as sudden cardiac arrest (SCA) or an appropriate ICD intervention. All patients underwent an echocardiographic examination. The medical and family histories were collected and ICD examinations were performed. RESULTS 76 patients underwent an ICD implantation during the follow-up period. 20 patients have reached an SCD end-point. 1 patient died due to SCA and 19 had an appropriate ICD intervention. There were statistically significant differences of GLS and ASI values between SCD and non-SCD groups; p = 0.026389 and p = 0.006208, respectively. The average GLS in the SCD group was -12.4% ± 3.4%, and -15.1% ± 3.5% in the non-SCD group. The average ASI values were -9.9% ± 3.8% and -12.4% ± 3.5%, respectively. There was a statistically significant difference between LAVI values in SCD and non-SCD groups; p = 0.005343. The median LAVI value in the SCD group was 45.7 ml/m2 and 37.6 ml/m2 in the non-SCD group. The ROC curves showed the following cut-off points for GLS, ASI and LAVI: -13.8%, -13.7% and 41 ml/m2, respectively. Cox’s proportional hazards model for the parameters used in the Calculator was at the borderline of significance; p = 0.04385. The model with new variables (GLS and LAVI instead of LA dimension) was significant; p = 0.00094. The important factors were LAVI; p = 0.000075 and nsVT; p = 0.012267. CONCLUSIONS The proposed new SCD risk factors were statistically significant in the study population and should be taken into account when considering ICD implantation.

P887Atrial fibrillation is associated with sudden cardiac death risk factors in patients with hypertrophic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
I A Marcuschamer ◽  
O Zusman ◽  
S Schwartzenberg ◽  
M Vaturi ◽  
Y Shapira ◽  
...  
Keyword(s):  
Risk Factors ◽  
Ventricular Tachycardia ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Nearest Neighbor ◽  
Nyha Class ◽  
Left Ventricular ◽  
Similar Distribution ◽  
Icd Implantation

Abstract Background Atrial Fibrillation (AF) is prevalent in a fourth of patients with Hypertrophic Cardiomyopathy (HCM), but its clinical impact in these patients remains ill-defined. Aim To compare clinical characteristics in HCM patients with vs without AF and assess indirectly potential sudden cardiac death (SCD) risk. Methods Retrospective study in a single tertiary referral HCM center. Patients with HCM and AF were compared with matched controlled HCM patients without AF. NYHA class was assessed by a single physician. Propensity score matching was performed with a ratio of 2:1 by nearest neighbor with adjustment for age, sex, and left ventricular tract obstruction (LVOTO). Ordinal regression was used with NYHA as outcome. Results Among 298 patients with HCM, 68 patients (22.8%) had AF. After propensity matching, 66 patients with AF and 112 without AF had similar distribution of age (67.1 vs. 65.1 years), gender (57.6% vs. 61% males) and Basal Surface Area (1.88 vs. 1.87 m2) respectively. The prevalence of LVOTO (57.6% vs. 58.5%) and apical hypertrophy (19.7% vs 19.5%) was similar in the two groups. Cardiac risk factors including Hypertension (60.6% vs. 60.2%) and Diabetes Mellitus (15.2 vs. 20.3%) were similar in both groups. AF patients were diagnosed with HCM at a younger age than patients without HCM (48.5 vs. 55 years; p=0.01). HCM patients with AF had significantly lower LVOT gradients compared with patients without AF (28.1 mmHg vs 47.4 mmHg, p=0.005), had a higher prevalence of non-sustained ventricular tachycardia (39.4% vs. 9.4%; p<0.01), and ventricular tachycardia (9.1% vs 1.7%; p<0.04) and were more likely to have undergone implantation of an internal cardioverter defibrillator (ICD) (23.1% vs. 8.5%; p=0.001), respectively. Dyspnea was the most prevalent symptom in both groups (51.1% and 46.6% in AF and non-AF respectively). NYHA Class was similar in both groups: 1.88±0.69 in patients with AF vs. 1.73±0.74 in patients without AF (p=0.17). NYHA class did not differ in 26 patients with chronic persistent AF vs. 42 patients with paroxysmal AF (being in sinus rhythm at evaluation). Conclusion AF does not seem to impact functional level class in patients with HCM, but carries a higher burden of sudden cardiac death prognostic factors, incurring a higher rate of ICD implantation. Acknowledgement/Funding None

Abstract 14575: Implantable Cardiac Defibrillator in the Setting of Tetralogy of Fallot: Long-Term Follow-Up of a French National Multicenter Retrospective Registry

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Abdeslam Bouzeman ◽  
Maxime De Guillebon ◽  
Guillaume Duthoit ◽  
Magalie Ladouceur ◽  
Raphael Martins ◽  
...  
Keyword(s):  
Risk Factors ◽  
Ventricular Tachycardia ◽  
Primary Prevention ◽  
Secondary Prevention ◽  
Tetralogy Of Fallot ◽  
Primary And Secondary Prevention ◽  
Long Term Follow Up ◽  
Appropriate Therapy

Background: Tetralogy of Fallot (TOF) is the most frequent form of congenital heart disease managed by EP physicians for potential ICD. However, few studies have reported long-term outcomes of TOF patients with ICD. Methods: Between 2005 and 2014, all TOF patients with ICD in 17 French centers were enrolled in a specific evaluation aiming to determine characteristics at implantation as well as outcomes (overall mortality, appropriate ICD therapies, and device-related complications). Results: Overall 78 patients (45±13 years, 64% males) were enrolled. A majority of patients were implanted in the setting of secondary prevention (73%), whereas the remaining (27%) in primary prevention. Among the latest group, known risk factors for sudden cardiac death were: severe pulmonary regurgitation (30%,) prior palliative shunt (50%), syncope with unknown origin (25%), inducible ventricular tachycardia (45%), QRS duration ≥180ms (18%), non-sustained ventricular tachycardia (25%), and documented sustained supra ventricular tachycardia (45%).Overall, patients implanted in the setting of primary prevention presented with a mean of 3.1±1.4 risk factors. After a mean follow-up of 4.9±3.8 years, 35 patients (45%) experienced at least one appropriate therapy (25% in the primary prevention group compared to 53% in the secondary prevention group), giving annual-incidences of 6.9% (95%CI 0.14-13.7) and 21.3% (12.4-30.3) respectively (P=0,01). The mean time between ICD implantation and the first appropriate therapy was 2.2±3.2 years, without significant differences between primary and secondary prevention. Overall, ≥one ICD-related complication occurred in 30 patients (38%), including inappropriate shock (n=9), major pocket hematoma (n=1), lead dysfunction (n=12), infection (n=4), shoulder algodystrophia (n=2), device failure or dislodgement needing reintervention (n=2). Eventually, four patients were transplanted (5%), and six patients (8%) died during the course of follow-up. Conclusions: Considering relatively long-term follow-up, patients with TOF and ICDs experience high rates of appropriate ICD therapies, in both primary and secondary prevention. Major ICD-related complications remain, however, high.

Abstract 11447: Low Incidence of Appropriate Therapy Following Defibrillator Implantation for Primary Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Amalie C Thavikulwat ◽  
Todd T Tomson ◽  
Bradley P Knight ◽  
Robert O Bonow ◽  
Lubna Choudhury
Keyword(s):  
Risk Factors ◽  
Risk Factor ◽  
Hypertrophic Cardiomyopathy ◽  
Primary Prevention ◽  
Sudden Cardiac Death ◽  
Secondary Prevention ◽  
Cardiac Death ◽  
Icd Implantation ◽  
Icd Therapy ◽  
Appropriate Therapy

Introduction: Hypertrophic cardiomyopathy (HCM) is a leading cause of sudden cardiac death (SCD) in young adults. Implantable cardioverter defibrillators (ICD) effectively terminate ventricular tachycardia (VT) and fibrillation (VF) that cause SCD, but the reported prevalence of and patient characteristics leading to appropriate ICD therapy in HCM have been variable. Hypothesis: We hypothesized that some risk factors may be more prevalent than others in patients with HCM who receive appropriate ICD therapy and that the overall incidence of appropriate therapy may be lower than that reported previously. Methods: We retrospectively studied all patients with HCM who were treated with ICDs at our referral center from 2000-2013 to determine the rates of appropriate and inappropriate ICD therapies. Results: Of 1136 patients with HCM, we identified 135 who underwent ICD implantation (125 for primary and 10 for secondary prevention), aged 18-81 years (mean 48±17) at the time of implantation. The mean follow-up time was 5.2±4.5 years. Appropriate ICD intervention occurred in 20 of 135 patients (2.8%/year) by providing a shock or antitachycardia pacing in response to VT or VF. The annual rate of appropriate ICD therapy was 2.4%/year for primary and 7.2%/year for secondary prevention devices. Commonly used risk factors were equally prevalent among patients who received appropriate therapy and those who did not; furthermore, the likelihood of receiving appropriate therapy in the presence of each risk factor was similar (Figure). Inappropriate ICD therapy occurred in 27 patients (3.8%/year). Conclusions: ICDs provide clear benefit to patients who experience life-threatening arrhythmias, particularly those being treated for secondary prevention. However, the appropriate therapy rate for primary prevention was lower than previously reported, and no single risk factor appeared to have stronger association with appropriate ICD therapy than others.

Abstract 15416: Longitudinal Assessment of Electrical-Mechanical Interactions in Children and Young Adults With Repaired Tetralogy of Fallot

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Suellen M Yin ◽  
Laura M Mercer-rosa ◽  
Jungwon Min ◽  
Elizabeth Goldmuntz ◽  
Victoria L Vetter
Keyword(s):  
Young Adults ◽  
Sudden Cardiac Death ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Cardiac Death ◽  
Volume Ratio ◽  
Rate Of Change ◽  
Ventricular Ectopy ◽  
Repaired Tetralogy Of Fallot ◽  
Over Time

Introduction: Electrical-mechanical interactions contribute to arrhythmias, sudden cardiac death, and right ventricular remodeling in repaired tetralogy of Fallot (TOF). Hypothesis: There are significant changes in electrocardiographic properties and electrical-mechanical interactions that occur over time after complete TOF repair and with pulmonary valve replacement (PVR). Methods: This retrospective cohort study of 177 patients, initially recruited for a cross-sectional research protocol, underwent complete TOF repair at 0.3±0.9 years with 21.5±4.2 years of clinical follow-up. We assessed ECG, Holter, cardiopulmonary exercise testing (CPET), and MRI data. We used linear mixed effects models to examine QRS duration (QRSd) and its rate of change over time, associations between comparable ECG and MRI, Holter and MRI, ECG and Holter, ECG and CPET, and pre-PVR and post-PVR results. Results: QRSd increased after TOF repair, but the rate of change decreased from 5.2 ms/year 1 year post-operatively to 1.7 ms/year 20 years post-operatively. Twenty years from TOF repair, post-operative arrhythmias included ventricular ectopy: ventricular tachycardia (4 of 20 patients) on Holter and premature ventricular contractions (14 of 19 patients) on CPET. QRSd was positively associated with right ventricular (RV) volumes, RV:left ventricular (LV) end-diastolic volume ratio, and complex ventricular ectopy on Holter; and negatively associated with RV ejection fraction (EF). The association between QRSd and RV volumes was weaker post-PVR. QRSd and its rate of change were associated with increased LV volume post-PVR. Complex ventricular ectopy was associated with lower LV EF, and significant atrial ectopy was associated with higher LV mass-to-volume ratio. Conclusions: Substantial ventricular ectopy occurs in adolescents and young adults after repair of TOF. Electrophysiologic changes included QRSd prolongation that progressively slowed. QRSd and its rate of change were associated with published risk factors for arrhythmia and sudden cardiac death, and with indications for PVR. Our ongoing research aims to identify an optimal threshold of pre-PVR QRSd and its rate of change that preserves bi-ventricular electrical-mechanical coupling post-PVR.

Ventricular tachycardia and sudden cardiac death might complicate the long-term outcome by patients after tetralogy of Fallot repair

2008 ◽  
Vol 149 (23) ◽  
pp. 1067-1069
Author(s):  
Attila Mihálcz ◽  
Csaba Földesi ◽  
Tamás Szili-Török
Keyword(s):  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Tetralogy Of Fallot ◽  
Cardiac Death ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Cardioverter Defibrillator ◽  
Tetralogy Of Fallot Repair

A Fallot-tetralógia miatti műtétet követően a hosszú távú túlélést befolyásoló tényezők közé tartozik a kamrai tachycardia és a hirtelen szívhalál. E betegek gondozásában érdemi segítséget jelent az implantálható cardioverter defibrillátor rendszer. A végleges pacemaker és/vagy implantálható cardioverter defibrillátor implantációját követően ritka, ám potenciálisan letális kimenetelű fertőzéses szövődmény az endocarditis. Ez esetben a leghatékonyabb kezelési mód a kombinált terápia, amely a beültetett készülék + elektródák teljes körű eltávolításából és agresszív antibiotikus kezelésből áll. Célkitűzés: Ilyen esetekben a tervezett reimplantáció különös óvatosságot igényel a nagyobb recidívaarány miatt, amelynek rizikója fokozottabb pacemakerdependencia esetén. Célunk olyan módszer alkalmazása volt, amelynek segítségével a recidíva kockázata minimálisra csökkenthető. Módszer: Esetünkben a korábban Fallot-tetralógia miatt többször műtött, pacemaker-, majd implantálható cardioverter defibrillátor beültetéseken átesett betegnél recidív endocarditis miatt készülék- és elektródaeltávolítást végeztünk, standard antibiotikus terápia alkalmazásával. A reimplantációt minithoracotomián keresztül végeztük. Az így elhelyezett sokkelektróda elégtelen működése miatt egy másik sokkelektródát szubkután vezettünk a hátsó mellkasfali régióba; rendszerünk az indukált kamrafibrillációt sikerrel szüntette meg. Megbeszélés: Esetismertetésünk demonstrálja a szubkután defibrillátorrendszer alkalmazhatóságát és előnyeit speciális körülmények fennállásakor. Felhívjuk a figyelmet arra a tényre, hogy ezt a technikát gyakrabban is lehetne alkalmazni olyan esetekben, amelyekben a transzvénás implantáció nem optimális.

Abstracts for the British Congenital Cardiac Association Annual Meeting: The Barbican, London, 24–25 November 2005: Oral Presentations: Early and midterm outcomes of Implantable Cardioverter Defibrillator (ICD) implantation in patients with repaired Tetralogy of Fallot and pulmonary stenosis – a retrospective review

2006 ◽  
Vol 16 (3) ◽  
pp. 314-315
Author(s):  
S. Viswanathan ◽  
K. English ◽  
M. E. C. Blackburn
Keyword(s):  
Ventricular Tachycardia ◽  
Success Rate ◽  
Tetralogy Of Fallot ◽  
Retrospective Review ◽  
Pulmonary Stenosis ◽  
Icd Implantation ◽  
Icd Therapy ◽  
Successful Termination ◽  
The Impact

Introduction: Repair of Tetralogy of Fallot up until recent decades involved aggressive resection and annular enlargement through a right ventriculotomy. This resulted in ventricular scarring and pulmonary incompetence, with an increased risk of ventricular tachyarrhythmia and sudden death in young adulthood. Following the NICE guidelines, implantation of ICDs as primary prevention in patients with repaired Tetralogy is ever increasing. This study aims to determine the rate of appropriate and inappropriate discharges, the success rate of ICD therapy and the impact of ICD implantation on the use of anti-arrhythmic medication in this population of patients. Materials and Methods: This is a retrospective review of patients with repaired Tetralogy of Fallot (n = 18) and pulmonary stenosis (n = 2) with implantable cardioverter defibrillators managed at our tertiary centre. Patients were identified from our outpatient database, their notes and charts were examined and details regarding indication for ICD implantation, device specifications and complications following implantation were collected. Data was also collected on the incidence of appropriate and inappropriate therapies and the success rate of ICD therapy along with the impact of implantation on the usage of anti-arrhythmic medication in these patients. Results: Of the 20 patients, 18 had previous repair of Tetralogy of Fallot and 2 had pulmonary valvotomy and infundibular resection for pulmonary stenosis between 1969 and 1989. 70% (n = 14) of these patients required reoperation with 10 patients having pulmonary valve replacements (PVR), 3 having redo infundibular resections and 1 requiring aortic valve replacement. At the time of consideration for ICD implantation 80% had moderate to severe pulmonary incompetence and 60% had more than mild right ventricular dilatation on echocardiography. Indications for ICD implantation were symptomatic ventricular tachycardia requiring cardioversion (n = 8), ventricular tachycardia on 24 hr tape/Reveal or electrophysiological study (n = 8), ventricular fibrillation (VF)/pulseless ventricular tachycardia (VT) (n = 2) and syncope with an abnormal EPS other than VT (n = 2, high grade ventricular ectopics, sinus node dysfunction).The median age at implantation was 22 years (16.4–43 years). All our patients had dual chamber devices implanted with either dual (n = 13) or single coil (n = 6) ventricular leads. GEM3 AT (n = 5), Marquis DR (n = 8) and Maximo DR (n = 7) generators (Medtronic Inc.) were implanted in sub pectoral position and both anti-tachycardia pacing and cardioversion modes were programmed as part of individualised VT and VF protocols. Early post procedural complications included atrial lead displacement (n = 1) and pneumothorax requiring drainage (n = 1).During a median follow up of 1.6 years (0.03– 4.5 years) several episodes of inappropriate therapies were noted in 6 patients (30%) especially early after implantation. This was found to be mainly due to atrial tachyarrhythmia, double counting of T waves or inaccurate interpretation of varying PR intervals as AV dyssynchrony which were effectively dealt with by changes in device programming. There were 33 episodes of inappropriate anti-tachycardia pacing (ATP) in 4 patients and 19 episodes of inappropriate cardioversion in 5 patients. Appropriate ATP was instituted in 4 patients (25%) with successful termination of all 20 episodes (100% success rate) of ventricular tachycardia. One patient required cardioversion with successful termination of VF. One patient (5%) with troublesome tachyarrhythmia died suddenly of unknown cause, 10 months after AICD implantation having had no detections or therapies on his device.Prior to ICD implantation 8 patients were on amiodarone therapy. At the time of last follow up after AICD implantation all patients were established on anti-arrhythmic agents and of these 6 patients were on amiodarone with the others being effectively managed on beta-blockers and/or flecainide.Late complications of ICD implantation included lead failure in 1 patient requiring replacement 3.3 years after implantation and generator replacement in a patient who was pacemaker dependent a year after implantation due to an advisory issued by the manufacturer regarding the risk of sudden battery depletion. Conclusions: In our study we found a rate of 0.6 appropriate and 1.4 inappropriate therapies (0.9 episodes of inappropriate ATP and 0.5 episodes of inappropriate cardioversion) per patient-year of follow up following ICD implantation which is in keeping with published literature. The mortality in our study group was 5% which is acceptable given the high risk population. Implantation of an ICD allowed switching over from amiodarone to less toxic anti arrhythmic therapy in a proportion of patients. Anti-tachycardia pacing was very successful in terminating tachyarrhythmia in our population with 100% success in terminating ventricular tachycardia.

Risk Factors for Mortality and Ventricular Tachycardia in Patients With Repaired Tetralogy of Fallot: A Systematic Review and Meta-analysis

2020 ◽  
Vol 36 (11) ◽  
pp. 1815-1825 ◽  
Author(s):  
Mathias Possner ◽  
Stephanie Y. Tseng ◽  
Fares Alahdab ◽  
Jouke P. Bokma ◽  
Adam M. Lubert ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systematic Review ◽  
Ventricular Tachycardia ◽  
Tetralogy Of Fallot ◽  
Meta Analysis ◽  
Repaired Tetralogy Of Fallot

scholarly journals Dynamic predictive accuracy of electrocardiographic biomarkers of sudden cardiac death within a survival framework: the Atherosclerosis Risk in Communities (ARIC) study

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Erick A. Perez-Alday ◽  
Aron Bender ◽  
David German ◽  
Srini V. Mukundan ◽  
Christopher Hamilton ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Predictive Accuracy ◽  
Clinical Risk Factors ◽  
Short Term ◽  
Clinical Risk ◽  
Atherosclerosis Risk

Abstract Background The risk of sudden cardiac death (SCD) is known to be dynamic. However, the accuracy of a dynamic SCD prediction is unknown. We aimed to measure the dynamic predictive accuracy of ECG biomarkers of SCD and competing non-sudden cardiac death (non-SCD). Methods Atherosclerosis Risk In Community study participants with analyzable ECGs in sinus rhythm were included (n = 15,716; 55% female, 73% white, age 54.2 ± 5.8 y). ECGs of 5 follow-up visits were analyzed. Global electrical heterogeneity and traditional ECG metrics (heart rate, QRS, QTc) were measured. Adjudicated SCD was the primary outcome; non-SCD was the competing outcome. Time-dependent area under the receiver operating characteristic curve (ROC(t) AUC) analysis was performed to assess the prediction accuracy of a continuous biomarker in a period of 3,6,9 months, and 1,2,3,5,10, and 15 years using a survival analysis framework. Reclassification improvement as compared to clinical risk factors (age, sex, race, diabetes, hypertension, coronary heart disease, stroke) was measured. Results Over a median 24.4 y follow-up, there were 577 SCDs (incidence 1.76 (95%CI 1.63–1.91)/1000 person-years), and 829 non-SCDs [2.55 (95%CI 2.37–2.71)]. No ECG biomarkers predicted SCD within 3 months after ECG recording. Within 6 months, spatial ventricular gradient (SVG) elevation predicted SCD (AUC 0.706; 95%CI 0.526–0.886), but not a non-SCD (AUC 0.527; 95%CI 0.303–0.75). SVG elevation more accurately predicted SCD if the ECG was recorded 6 months before SCD (AUC 0.706; 95%CI 0.526–0.886) than 2 years before SCD (AUC 0.608; 95%CI 0.515–0.701). Within the first 3 months after ECG recording, only SVG azimuth improved reclassification of the risk beyond clinical risk factors: 18% of SCD events were reclassified from low or intermediate risk to a high-risk category. QRS-T angle was the strongest long-term predictor of SCD (AUC 0.710; 95%CI 0.668–0.753 for ECG recorded within 10 years before SCD). Conclusion Short-term and long-term predictive accuracy of ECG biomarkers of SCD differed, reflecting differences in transient vs. persistent SCD substrates. The dynamic predictive accuracy of ECG biomarkers should be considered for competing SCD risk scores. The distinction between markers predicting short-term and long-term events may represent the difference between markers heralding SCD (triggers or transient substrates) versus markers identifying persistent substrate.

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