Published online before print March 29, 2004, doi: 10.1161/01.CIR.0000126495.11040.BD
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
(Circulation. 2004;109:1994-2000.)
© 2004 American Heart Association, Inc.
Clinical Investigation and Reports |
Value of Programmed Ventricular Stimulation After Tetralogy of Fallot Repair
A Multicenter Study
From the Boston Adult Congenital Heart and Electrophysiology Services, Children’s Hospital Boston, Boston, Mass (P.K., M.J.L., M.E.A., E.P.W.); Adult Congenital Heart Unit, Royal Brompton Hospital, London, UK (M.A.G.); Department of Cardiology, Centre Hospitalier et Universitaire de Nancy, Nancy, France (H.L., F.M.); and Department of Biostatistics, Montreal Heart Institute, Montreal, Canada (J.L.).
Correspondence to Dr Paul Khairy, Department of Cardiology, Children’s Hospital Boston, 300 Longwood Ave, Boston, MA 02115 (e-mail pkhairy{at}cardio.tch.harvard.edu). Reprint requests to Dr Edward P. Walsh, Director, Electrophysiology Service, Department of Cardiology, Children’s Hospital Boston, 300 Longwood Ave, Boston, MA 02115 (e-mail epwalsh@cardio.tch.harvard.edu).
Received July 2, 2003; de novo received September 28, 2003; revision received January 26, 2004; accepted February 3, 2004.
 |
Abstract |
|---|
 Top Abstract IntroductionMethodsResultsDiscussionConclusionsReferences |
|---|
Background— Studies have consistently shown that ventricular tachycardia (VT) and sudden cardiac death (SCD) complicate the long-term outcome after tetralogy of Fallot repair, yet the diagnostic and predictive value of electrophysiological testing in this population is uncertain.
Methods and Results— A multicenter cohort of 252 patients with repaired tetralogy of Fallot undergoing programmed ventricular stimulation was followed up for 18.5±9.6 and 6.5±4.5 years after corrective surgery and electrophysiological testing, respectively. Clinical VT and/or SCD occurred in 24.6%. Sustained monomorphic VT and polymorphic VT were induced in 30.2% and 4.4%. Including polymorphic VT in the definition of inducibility improved sensitivity (66.1±6.0% versus 77.4±5.3%, P=0.0082) with a marginal reduction in specificity (81.6±2.8% versus 79.5±2.9%, P=0.0455). Positive and negative predictive values were 55.2±5.3% and 91.5±2.2%. Independent risk factors for inducibility were age at study 
18 years (OR, 3.3), palpitations (OR, 2.8), prior palliative surgery (OR, 3.1), modified Lown criteria 2 (OR, 5.6), and cardiothoracic ratio 0.6 (OR, 3.3). Event-free survival rates in noninducible and inducible patients at 1, 5, 10, and 15 years were 97.9%, 92.8%, 89.3%, and 89.3% versus 79.4%, 62.6%, 58.7%, and 50.3%, respectively (P<0.0001). Both inducible monomorphic VT [relative risk (RR), 5.0; P=0.0002] and polymorphic VT (RR, 12.9; P<0.0001) predicted future clinical VT and SCD. In a multivariate analysis, inducible sustained VT was an independent risk factor for subsequent events (RR, 4.7; 95% CI, 1.2 to 18.5; P=0.0268).
Conclusions— Programmed ventricular stimulation is of diagnostic and prognostic value in risk stratifying patients with repaired tetralogy of Fallot. In this patient population, inducible sustained polymorphic VT should not be disregarded as nonspecific.
Key Words: arrhythmia • death, sudden • electrical stimulation • tetralogy of Fallot
|
Introduction |
|---|
|
TopAbstractIntroductionMethodsResultsDiscussionConclusionsReferences |
|---|
Tetralogy of Fallot is the most common cyanotic heart disease beyond infancy, accounting for 10% of all congenital heart malformations.1 "Corrective" intracardiac repair has been performed for >40 years with excellent results.2,3 Nevertheless, ventricular arrhythmias have consistently been reported, and sudden cardiac death (SCD) is the most common cause of mortality late after repair.4–6 Considerable efforts have been directed toward identifying predictors to stratify patients into high- and low-risk categories for these events. Programmed ventricular stimulation has been used in selected patients at many centers; however, attempts to clarify its predictive value have been limited by small patient numbers and relatively low event rates.7–9 We sought to assess the diagnostic value and prognostic significance of programmed ventricular stimulation in a multicenter cohort with repaired tetralogy of Fallot.
|
Methods |
|---|
|
TopAbstractIntroductionMethodsResultsDiscussionConclusionsReferences |
|---|
Study Population
The cohort consisted of patients with repaired tetralogy of Fallot and programmed ventricular stimulation performed between 1985 and 2002 from Children’s Hospital Boston, Centre Hospitalier et Universitaire de Nancy, and 6 centers that recently identified noninvasive risk factors for tetralogy of Fallot5 (Royal Brompton Hospital, Children’s Hospital of Pittsburgh, University of Minnesota Hospital and Clinic/Variety Club Children’s Hospital, South Carolina Children’s Heart Center, Tokyo Women’s Medical College, and Toronto Hospital). Patients with unrepaired tetralogy, pulmonary atresia, AV canal, and double-outlet right ventricle were excluded. Aspects of subsets of these populations have previously been reported.5,8,10
Data Collection and Follow-Up
Data were collected in accordance with individual hospital institutional review board policies. Details about baseline characteristics, surgical history, symptoms before electrophysiological testing, hemodynamic parameters, ECG measures, Holter recordings, cardiothoracic ratios, sustained atrial arrhythmias, antiarrhythmic agents, pacemakers, implantable cardioverter-defibrillators (ICDs), and deaths were obtained. Sources included electronic and paper cardiological, surgical, electrophysiological, echocardiographic, hemodynamic, radiographic, and ECG charts and databases supplemented by records from referring physicians. Characteristics at the time of programmed ventricular stimulation or the most recent studies within 1 year before testing were recorded and analyzed.
ECG parameters, including QRS axis and maximum duration, were measured manually from standard 12-lead ECGs (25 mm/s and 1 mV/cm) in patients in sinus rhythm and off antiarrhythmic agents.11 Cardiothoracic ratios were calculated from posteroanterior chest radiographs.12 Data from M-mode, 2D, and Doppler echocardiography, cardiac catheterization, and cardiac MRI were recorded, with particular attention given to right ventricular pressures and severity of tricuspid and pulmonary regurgitation (graded absent, mild, moderate, or severe). Ventricular ectopy from standard Holter monitors were classified according to modified Lown criteria.13
The primary end point was a composite of sustained ventricular tachycardia (VT) or SCD, whether or not resuscitation was successful. VT was considered sustained if it persisted 30 seconds or required electrical conversion. SCD was defined as death attributed to a cardiac cause occurring within 1 hour of acute symptoms.14 To assess the prognostic significance of programmed ventricular stimulation, time 0 was defined as time of electrophysiological testing, and patient-years were accrued until occurrence of the primary outcome, study termination, or death resulting from other causes.
Programmed Ventricular Stimulation
Electrophysiological studies were performed in patients under conscious sedation in a drug-free state. Programmed ventricular stimulation was achieved at twice the diastolic threshold at 2 right ventricular sites (typically apex and outflow tract) with 2 eight-beat drive trains (cycle lengths between 400 and 600 ms) and up to 3 extrastimuli with coupling intervals 180 ms. In the absence of inducible VT, the protocol was repeated in all but 5 patients with an isoproterenol infusion titrated to increase heart rate by 20% to 50%. Ventricular burst pacing and S5 protocols were not routinely used. Results were classified into 3 categories: negative, sustained monomorphic VT, and sustained polymorphic VT. Sustained VT was defined as persisting 30 seconds or requiring cardioversion. Induction of nonsustained VT was not coded consistently by all contributing centers and could not be analyzed in this study. If >1 study was performed, results from the earliest were retained for analysis.
Standard definitions were used for sensitivity, specificity, diagnostic accuracy, and predictive values of programmed ventricular stimulation in identifying patients with clinical sustained VT or SCD. Positive [LR(+)] and negative [LR(–)] likelihood ratios were defined as sensitivity/(1–specificity) and (1–sensitivity)/specificity, respectively.15
Statistical Analysis
Continuous variables are presented as mean±SD and dichotomous variables as percentages. Baseline characteristics and clinical outcomes according to results of programmed ventricular stimulation were compared by use of 
2, Fisher’s exact, 1-way ANOVA, or McNemar’s test when appropriate. Logistic regression models were used to identify predictors of inducible VT. Event-free survival curves were plotted and compared by use of the Kaplan-Meier method and log-rank statistic.16 To assess the predictive value of inducible VT, multivariate Cox proportional-hazard models17 accounted for duration of follow-up and adjusted for clinical, surgical, hemodynamic, ECG, and radiographic variables. The proportional-hazards assumption was verified by time-dependent interactions and goodness-of-fit statistics (weighted Schoenfeld residuals). Two-tailed values of P<0.05 were considered statistically significant. Testing was performed with SAS software version 8 (SAS Institute).
|
Results |
|---|
|
TopAbstractIntroductionMethodsResultsDiscussionConclusionsReferences |
|---|
Baseline Characteristics
A total of 252 patients, 59.4% male, with repaired tetralogy of Fallot underwent programmed ventricular stimulation at 16.0±12.3 years (range, 3.3 to 55.6 years) of age. Follow-up duration after corrective surgery and programmed ventricular stimulation was 18.5±9.6 and 6.5±4.5 years. ECGs were available in 100%; chest x-rays, 97%; echocardiograms, 97%; Holters, 95%; cardiac catheterization, 94%; and cardiac MRI, 6%. Valvular regurgitation severity by MRI and echocardiography was concordant in all but 1 patient in whom "probably mild" pulmonary regurgitation by echocardiography was graded moderate by MRI; the latter grade was retained.
Median age at corrective surgery was 4.5 years, and 57.2% had transannular right ventricular outflow tract patches. Surgical palliation with systemic to pulmonary shunting was performed before repair in 46.3%. Age at repair and prior palliative shunt did not differ significantly by enrollment site. At the time of programmed ventricular stimulation, QRS duration was 146±36 ms, with QRS 180 ms in 19.4% and left anterior hemiblock in 22.6%. Right ventricular systolic and end-diastolic pressures were 44±16 and 8±4 mm Hg, with 74.2% and 14.3% having at least moderate pulmonary and tricuspid regurgitation, respectively. The average cardiothoracic ratio was 56.3±6.1%. Before electrophysiological testing, 27.7% reported palpitations, 23.6% reported syncope, 16.7% had documented sustained VT, and 1.2% were resuscitated from cardiac arrest. Characteristics stratified according to results of programmed ventricular stimulation are summarized in Table 1.
|
Diagnostic Value of Programmed Ventricular Stimulation
Sustained monomorphic VT was induced in 30.2% (n=76), sustained polymorphic VT was induced in 4.4% (n=11), and 65.5% (n=165) were noninducible. An average of 2.7±0.6 extrastimuli were required for induction, with isuprel infusion in 23.5%. Of initially negative studies, 11% became positive with isuprel. Of 87 patients with inducible sustained VT, 26 received antiarrhythmic therapy alone, 15 had ICDs alone, and 8 had both antiarrhythmics and ICDs. VT ablation was performed in 3 patients at the initial study (and 4 others after recurrent events). The primary outcome occurred in 62 patients (24.6%): VT alone in 45, VT and SCD in 14, and SCD without documented VT in 3 patients. Three died of other causes: end-stage congestive heart failure, recreational drug overdose, and cerebral edema after right ventricular outflow tract revision. The last patient had experienced clinical sustained VT before his death.
Table 2 classifies patients according to 2 definitions of inducibility, depending on whether sustained polymorphic VT is considered positive. Diagnostic characteristics of programmed ventricular stimulation are summarized accordingly in Table 3. Including polymorphic VT resulted in greater sensitivity (77.4±5.3% versus 66.1±6.0%, P=0.0082) with a marginal decrease in specificity (P=0.0455). Overall diagnostic accuracy approached 80%. More than 3-fold increase in odds of clinical VT or SCD followed a positive study [LR(+), 3.77]. Compared with inducible monomorphic VT alone, a negative study was associated with a greater reduction in odds of the combined outcome [LR(–), 0.28 versus 0.42]. Predictors of inducible sustained monomorphic or polymorphic VT, ORs, and 95% CIs are summarized in Table 4. Of note, presence of a left anterior hemiblock did not predict inducibility (P=0.6757).
|
|
|
Prognostic Significance of Programmed Ventricular Stimulation
After programmed ventricular stimulation, 16.8% experienced sustained VT or SCD: VT alone, 8.1%; SCD alone, 3.7%; and VT and SCD, 5.0%. Actuarial event-free survival rates of the entire cohort at 1, 5, 10, and 15 years were 91.5%, 82.7%, 79.2%, and 74.3%, respectively (Figure 1A). Induction of sustained monomorphic [relative risk (RR), 5.0] and polymorphic (RR, 12.9) VT was a powerful predictor of subsequent events (Table 5 and Figure 1B). Event-free survival rates in noninducible and inducible patients at 1, 5, 10, and 15 years were 97.9%, 92.8%, 89.3%, and 89.3% versus 79.4%, 62.6%, 58.7%, and 50.3%, respectively (P<0.0001). Kaplan-Meier event-free survival curves are illustrated in Figure 2. Mode of VT induction, including number of required extrastimuli, was not predictive of subsequent events (P=0.9162).
|
|
|
Inducible sustained monomorphic or polymorphic VT remained an independent predictor of outcome (RR, 4.7; 95% CI, 1.2 to 18.5; P=0.0268) in multivariate regression analyses controlling for age at corrective surgery and electrophysiological testing, gender, presence of a transannular patch, prior palliative surgery, clinical symptoms, QRS duration, right ventricular pressures, at least moderate tricuspid or pulmonary regurgitation, atrial arrhythmias, cardiothoracic ratio, class I or III antiarrhythmic agents, and presence of an ICD. When the 29 patients with ICDs and 3 with primary VT ablation (2 also had ICDs) were excluded, positive programmed ventricular stimulation remained a powerful predictor of clinical VT and SCD (RR, 4.9; 95% CI, 1.9 to 12.6; P=0.0012).
Routine Screening Versus Clinical Indication for Programmed Ventricular Stimulation
Overall, 93 patients (36.9%) had programmed ventricular stimulation as "routine" screening, whereas 159 (63.1%) had clinical symptoms and/or recorded ventricular arrhythmias that prompted further testing. Test characteristics in these patient subgroups are summarized in Table 6. Inducible sustained monomorphic or polymorphic VT independently predicted subsequent clinical VT or SCD in patients routinely screened (RR, 10.4; 95% CI, 1.1 to 100.2; P=0.0425) and with clinical indications (RR, 4.2; 95% CI, 1.6 to 11.2; P=0.0036) for programmed ventricular stimulation.
|
|
Discussion |
|---|
|
TopAbstractIntroductionMethodsResultsDiscussionConclusionsReferences |
|---|
Despite encouraging results with surgical repair of tetralogy of Fallot,2,3 late cardiac mortality resulting from ventricular arrhythmias and sudden death is observed in a substantial proportion of patients.4–6,18,19 Beyond the first 5 to 10 years after repair, a small but steady decline in freedom from VT and SCD has been observed, with 11.9% experiencing VT and 8.3% dying suddenly by 35 years.5 Given that >60% of patients in the present study had clinical indications prompting electrophysiological testing, this cohort represents a higher-risk population, consistent with the 23.4% incidence of VT and 6.7% incidence of SCD over 18.5±9.6 years.
Considerable progress has been made toward identifying noninvasive risk factors for VT and SCD in corrected tetralogy of Fallot. In the largest cohort study to date,5 independent predictors of clinical VT were QRS 180 ms and annual increase in QRS duration. Additional risk factors for SCD have included older age at repair, presence of a transannular right ventricular outflow tract patch, frequent ectopic beats,20,21 increased right ventricular systolic pressures,19,22,23 complete heart block,19,24 and increased JT dispersion.25,26 Despite advances in noninvasive risk stratification, identification of high-risk subgroups has not been sufficiently accurate to guide management decisions reliably. Attempts to clarify the utility of electrophysiological testing had previously been limited by small numbers of adequately studied patients and relatively infrequent occurrence of subsequent events, precluding meaningful estimates of diagnostic and predictive values.7–9
In this patient population, the 34.5% rate of inducible sustained VT was similar to the rate of 34.8% reported in postinfarction patients with ejection fractions 
40% and nonsustained VT.27 Moreover, the diagnostic value [sensitivity, 77.4%; specificity, 79.5%; diagnostic accuracy, 79.0%; LR(+), 3.77; and LR(–), 0.28] and prognostic significance (RR, 4.7 for subsequent clinical VT or SCD) compared favorably to programmed ventricular stimulation after myocardial infarction.28–30 Although several risk factors for inducible VT were identified, electrophysiological testing independently predicted future events after controlling for these and other clinical, ECG, and hemodynamic variables. Although the study was not designed to appraise therapeutic strategies, inducible sustained VT was a significant predictor of events in patients with and without antiarrhythmic agents, VT ablation, and ICDs.
The arrhythmic substrate in tetralogy has been likened to postinfarction scar-related VT. Induced VT is most commonly monomorphic and macroreentrant, rotating clockwise or counterclockwise around myotomy scars or surgical patches.20,31,32 In patients with coronary artery disease, inducible sustained polymorphic VT is considered either nonspecific or evidence of ventricular instability.33,34 Antiarrhythmic therapy may convert inducible polymorphic VT into a more stable substrate.35 Recent clinical trials have included sustained polymorphic VT in the definition of inducibility.27,36 Interestingly, in this tetralogy of Fallot population, induction of sustained polymorphic VT was a powerful predictor of future events (RR, 12.9), and its inclusion enhanced the diagnostic yield of programmed ventricular stimulation. Direct comparisons between inducible monomorphic and polymorphic VT should be interpreted with caution, given the small number of patients with the latter arrhythmia. Nevertheless, inducible polymorphic VT after tetralogy repair should not be disregarded as nonspecific. Although not assessed in the present study, inducible nonsustained VT has also been associated with decreased survival in a mixed cohort of congenital heart patients.10
Although the value of inducible VT as a risk factor after tetralogy of Fallot repair has been demonstrated, questions about patient selection for screening and the timing and frequency of testing remain to be elucidated. Test sensitivity, specificity, and likelihood ratios, increasingly considered the best available indexes to evaluate diagnostic tests, are independent of prevalence assumptions.15 Subgroup analyses have shown these parameters to be no different in patients routinely screened compared with those with clinical indications for testing. However, positive and negative predictive values are influenced by pretest probability. Studies are currently assessing whether risk assessment based on noninvasive parameters may be helpful in preselecting patients for further testing with programmed ventricular stimulation. Finally, because risk increases with age and changes in underlying substrate may occur over time, careful follow-up is warranted.
Study Limitations
Given that selection criteria for electrophysiological testing were not standardized, marginal distributions are not random, and prevalence values reflect the heterogeneous patient population. Although this may increase generalizability, prevalence estimates and positive and negative predictive values should be interpreted in this context. Unlike prognostic values, diagnostic test characteristics reflect a particular time point (ie, 18.5±9.6 years after corrective surgery) and may vary with duration of follow-up.
Medical and catheter-based therapeutic decisions were influenced by results of electrophysiological testing and were not randomly allocated. However, direction of this potential bias is expected to result in underestimation of the predictive value of programmed ventricular stimulation because inducible patients are more likely to receive therapy directed toward reducing risk of VT and SCD. Analyses were performed with and without adjustment for antiarrhythmic therapy but not ß-blockers (insufficient data) and were repeated after exclusion of the few patients with primary VT ablation. Results were robust and essentially unaltered.
In contrast to other therapies, ICD implantation should theoretically not reduce incidence of the combined primary outcome because ICD therapy for sustained ventricular arrhythmias qualifies as an end point. However, ICD implantation may result in a detection bias that overestimates the predictive ability of programmed ventricular stimulation because patients with inducible VT are more likely to receive these devices that detect and record ventricular arrhythmias. Nonetheless, analyses performed in patients with and without ICDs yielded similar results (RR, 4.7 versus 4.9).
|
Conclusions |
|---|
|
TopAbstractIntroductionMethodsResultsDiscussionConclusionsReferences |
|---|
In this multicenter cohort study, programmed ventricular stimulation was of diagnostic value and prognostic significance in risk stratifying patients with repaired tetralogy of Fallot. Inducible sustained polymorphic VT enhanced the diagnostic yield and predictive ability and therefore should not be disregarded as a nonspecific finding. Electrophysiological testing predicted future clinical VT and SCD above and beyond known noninvasive risk factors. These results suggest that electrophysiological testing may contribute importantly to risk-stratification algorithms designed to guide therapy to prevent clinical VT and SCD.
|
Acknowledgments |
|---|
This study was supported in part by a fellowship award (P.K.) from Canadian Institutes of Health Research. We are indebted to the following cardiologists for their invaluable contributions: Seshadri Balaji, MBBS, South Carolina Children’s Heart Center, Charleston; Steven A. Webber, MD, Department of Cardiology, Children’s Hospital of Pittsburgh, Pittsburgh, Penn; James H. Moller, MD, Department of Cardiology, University of Minnesota Hospital and Clinic/Variety Club Children’s Hospital, Minneapolis, Minn; Tokuko Shinohara, MD, and Makoto Nakazawa, MD, Heart Institute of Japan and the Tokyo Women’s Medical College, Tokyo, Japan; and Andrew N. Redington, MD, University of Toronto Congenital Cardiac Center for Adults, Toronto Hospital, Toronto, Canada.
|
References |
|---|
|
TopAbstractIntroductionMethodsResultsDiscussionConclusionsReferences |
|---|
1. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults: second of two parts. N Engl J Med. 2000; 342: 334–342.
2. Lillehei CW, Cohen M, Warden HE. Direct vision intracardiac surgical correction of the tetralogy of Fallot, pentalogy of Fallot, and pulmonary atresia defects: report of the first ten cases. Ann Surg. 1955; 142: 418–445.[Medline] [Order article via Infotrieve]
3. Kirklin JW, DuShane JW, Patrick RT. Intracardiac surgery with the aid of a mechanical pump-oxygenator system (Gibbon type): report of eight cases. Mayo Clin Proc. 1955; 30: 201–206.[Medline] [Order article via Infotrieve]
4. Gillette PC, Garson A Jr. Sudden cardiac death in the pediatric population. Circulation. 1992; 85: I-64–I-69.[Medline] [Order article via Infotrieve]
5. Gatzoulis MA, Balaji S, Webber SA, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet. 2000; 356: 975–981.[CrossRef][Medline] [Order article via Infotrieve]
6. Nollert G, Fischlein T, Bouterwek S, et al. Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair. J Am Coll Cardiol. 1997; 30: 1374–1383.[Abstract]
7. Garson A Jr, Porter CB, Gillette PC, et al. Induction of ventricular tachycardia during electrophysiologic study after repair of tetralogy of Fallot. J Am Coll Cardiol. 1983; 1: 1493–1502.[Abstract]
8. Lucron H, Marcon F, Bosser G, et al. Induction of sustained ventricular tachycardia after surgical repair of tetralogy of Fallot. Am J Cardiol. 1999; 83: 1369–1373.[CrossRef][Medline] [Order article via Infotrieve]
9. Gatzoulis MA, Balaji S, Webber SA, et al. Risk stratification for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot. In: Gatzoulis MA, Murphy DJ, eds. The Adult With Tetralogy of Fallot. Armonk, NY: Futura Publishing; 2001; 1–23.
10. Alexander ME, Walsh EP, Saul JP, et al. Value of programmed ventricular stimulation in patients with congenital heart disease. J Cardiovasc Electrophysiol. 1999; 10: 1033–1044.[Medline] [Order article via Infotrieve]
11. Gatzoulis MA, Till JA, Somerville J, et al. Mechanoelectrical interaction in tetralogy of Fallot: QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death. Circulation. 1995; 92: 231–237.
12. Hammermeister KE, Chikos PM, Fisher L, et al. Relationship of cardiothoracic ratio and plain film heart volume to late survival. Circulation. 1979; 59: 89–95.
13. Lown B, Wolf M. Approaches to sudden death from coronary heart disease. Circulation. 1971; 44: 130–142.
14. Huikuri HV, Castellanos A, Myerburg RJ. Sudden death due to cardiac arrhythmias. N Engl J Med. 2001; 345: 1473–1482.
15. Weiss NS. Clinical epidemiology. In: Rothman KJ, Greenland S, eds. Modern Epidemiology. 2nd ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 1998; 519–528.
16. Greenland S. Applications of stratified analysis methods. In: Rothman KJ, Greenland S, eds. Modern Epidemiology. 2nd ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 1998; 281–300.
17. Cox DR. Regression models and life tables. J R Stat Soc B. 1972; 34: 187–220.
18. Bricker JT. Sudden death and tetralogy of Fallot: risks, markers, and causes. Circulation. 1995; 92: 158–159.
19. Jonsson H, Ivert T, Brodin LA, et al. Late sudden deaths after repair of tetralogy of Fallot. Scand J Thorac Cardiovasc Surg. 1995; 29: 131–139.[Medline] [Order article via Infotrieve]
20. Harrison DA, Harris L, Siu SC, et al. Sustained ventricular tachycardia in adult patients late after repair of tetralogy of Fallot. J Am Coll Cardiol. 1997; 30: 1368–1373.[Abstract]
21. Chandar JS, Wolff GS, Garson A Jr, et al. Ventricular arrhythmias in postoperative tetralogy of Fallot. Am J Cardiol. 1990; 65: 655–661.[CrossRef][Medline] [Order article via Infotrieve]
22. Garson A Jr, Nihill MR, McNamara DG, et al. Status of the adult and adolescent after repair of tetralogy of Fallot. Circulation. 1979; 59: 1232–1240.
23. Katz NM, Blackstone EH, Kirklin JW, et al. Late survival and symptoms after repair of tetralogy of Fallot. Circulation. 1982; 65: 403–410.
24. Hokanson JS, Moller JH. Significance of early transient complete heart block as a predictor of sudden death late after operative correction of tetralogy of Fallot. Am J Cardiol. 2001; 87: 1271–1277.[CrossRef][Medline] [Order article via Infotrieve]
25. Berul CI, Hill SL, Geggel RL, et al. Electrocardiographic markers of late sudden death risk in postoperative tetralogy of Fallot children. J Cardiovasc Electrophysiol. 1997; 8: 1349–1356.[Medline] [Order article via Infotrieve]
26. Vogel M, Sponring J, Cullen S, et al. Regional wall motion and abnormalities of electrical depolarization and repolarization in patients after surgical repair of tetralogy of Fallot. Circulation. 2001; 103: 1669–1673.
27. Buxton AE, Lee KL, Fisher JD, et al. A randomized study of the prevention of sudden death in patients with coronary artery disease. N Engl J Med. 1999; 341: 1882–1890.
28. Bourke JP, Richards DA, Ross DL, et al. Routine programmed electrical stimulation in survivors of acute myocardial infarction for prediction of spontaneous ventricular tachyarrhythmias during follow-up. J Am Coll Cardiol. 1991; 18: 780–788.[Abstract]
29. Iesaka Y, Nogami A, Aonuma K, et al. Prognostic significance of sustained monomorphic ventricular tachycardia induced by programmed ventricular stimulation using up to three extrastimuli in survivors of acute myocardial infarction. Am J Cardiol. 1990; 65: 1057–1063.[CrossRef][Medline] [Order article via Infotrieve]
30. Uther JB, Richards DA, Denniss AR, et al. The prognostic significance of programmed ventricular stimulation after myocardial infarction: a review. Circulation. 1987; 75: III-161–III-168.[Medline] [Order article via Infotrieve]
31. Downar E, Harris L, Kimber S, et al. Ventricular tachycardia after surgical repair of tetralogy of Fallot: results of intraoperative mapping studies. J Am Coll Cardiol. 1992; 20: 648–655.[Abstract]
32. Gonska BD, Cao K, Raab J, et al. Radiofrequency catheter ablation of right ventricular tachycardia late after repair of congenital heart defects. Circulation. 1996; 94: 1902–1908.
33. Reddy CP, Sartini JC. Nonclinical polymorphic ventricular tachycardia induced by programmed ventricular stimulation: incidence, mechanisms, and clinical significance. Circulation. 1980; 62: 988–995.
34. Stevenson WG, Brugada P, Waldecker B, et al. Can potentially significant polymorphic ventricular arrhythmias initiated by programmed ventricular stimulation be distinguished from those that are nonspecific? Am Heart J. 1986; 111: 1073–1080.[CrossRef][Medline] [Order article via Infotrieve]
35. Buxton AE, Josephson ME, Marchlinski FE, et al. Polymorphic ventricular tachycardia induced by programmed stimulation: response to procainamide. J Am Coll Cardiol. 1993; 21: 90–98.[Abstract]
36. Moss AJ, Hall WJ, Cannom DS, et al. Improved survival with an implanted defibrillator in patients with coronary disease at high risk for ventricular arrhythmia. N Engl J Med. 1996; 335: 1933–1940.
This article has been cited by other articles:
 |
|
 |
|
  C. A. Warnes, R. G. Williams, T. M. Bashore, J. S. Child, H. M. Connolly, J. A. Dearani, P. del Nido, J. W. Fasules, T. P. Graham Jr, Z. M. Hijazi, et al. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons J. Am. Coll. Cardiol., December 2, 2008; 52(23): e143 - e263. [Full Text] [PDF]
|
|
|
|
 |
|
 C. A. Warnes, R. G. Williams, T. M. Bashore, J. S. Child, H. M. Connolly, J. A. Dearani, P. del Nido, J. W. Fasules, T. P. Graham Jr, Z. M. Hijazi, et al. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons Circulation, December 2, 2008; 118(23): e714 - e833. [Full Text] [PDF]
|
|
|
|
 |
|
 J. K. Triedman Should patients with congenital heart disease and a systemic ventricular ejection fraction less than 30% undergo prophylactic implantation of an ICD?: Implantable Cardioverter Defibrillator Implantation Guidelines Based Solely on Left Ventricular Ejection Fraction Do Not Apply to Adults With Congenital Heart Disease Circ Arrhythm Electrophysiol, October 1, 2008; 1(4): 307 - 316. [Full Text] [PDF]
|
|
|
|
|
|
P. Khairy, L. Harris, M. J. Landzberg, S. M. Fernandes, A. Barlow, L.-A. Mercier, S. Viswanathan, P. Chetaille, E. Gordon, A. Dore, et al. Sudden Death and Defibrillators in Transposition of the Great Arteries With Intra-atrial Baffles: A Multicenter Study Circ Arrhythm Electrophysiol, October 1, 2008; 1(4): 250 - 257. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. K. Witte, C. B. Pepper, J. C. Cowan, J. D. Thomson, K. M. English, and M. E. Blackburn Implantable cardioverter-defibrillator therapy in adult patients with tetralogy of Fallot Europace, August 1, 2008; 10(8): 926 - 930. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, D. L. Hayes, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons J. Am. Coll. Cardiol., May 27, 2008; 51(21): e1 - e62. [Full Text] [PDF]
|
|
|
|
|
|
A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. Mark Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, D. L. Hayes, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons J. Am. Coll. Cardiol., May 27, 2008; 51(21): 2085 - 2105. [Full Text] [PDF]
|
|
|
|
|
|
Writing Committee Members, A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons Circulation, May 27, 2008; 117(21): 2820 - 2840. [Full Text] [PDF]
|
|
|
|
|
|
Writing Committee Members, A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons Circulation, May 27, 2008; 117(21): e350 - e408. [Full Text] [PDF]
|
|
|
|
|
|
R. J. Sommer, Z. M. Hijazi, and J. F. Rhodes Pathophysiology of Congenital Heart Disease in the Adult: Part III: Complex Congenital Heart Disease Circulation, March 11, 2008; 117(10): 1340 - 1350. [Full Text] [PDF]
|
|
|
|
 |
|
 A L Knauth, K Gauvreau, A J Powell, M J Landzberg, E P Walsh, J E Lock, P J d. Nido, and T Geva Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair Heart, February 1, 2008; 94(2): 211 - 216. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Khairy, L. Harris, M. J. Landzberg, S. Viswanathan, A. Barlow, M. A. Gatzoulis, S. M. Fernandes, L. Beauchesne, J. Therrien, P. Chetaille, et al. Implantable Cardioverter-Defibrillators in Tetralogy of Fallot Circulation, January 22, 2008; 117(3): 363 - 370. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Khairy and A. J. Marelli Clinical Use of Electrocardiography in Adults With Congenital Heart Disease Circulation, December 4, 2007; 116(23): 2734 - 2746. [Full Text] [PDF]
|
|
|
|
|
|
A. Uebing, D. G. Gibson, S. V. Babu-Narayan, G. P. Diller, K. Dimopoulos, O. Goktekin, M. S. Spence, K. Andersen, M. Y. Henein, M. A. Gatzoulis, et al. Right Ventricular Mechanics and QRS Duration in Patients With Repaired Tetralogy of Fallot: Implications of Infundibular Disease Circulation, October 2, 2007; 116(14): 1532 - 1539. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S.-C. Yap, J. W. Roos-Hesselink, E. S. Hoendermis, W. Budts, H. W. Vliegen, B. J.M. Mulder, A. P.J. van Dijk, M. J. Schalij, and W. Drenthen Outcome of implantable cardioverter defibrillators in adults with congenital heart disease: a multi-centre study Eur. Heart J., August 1, 2007; 28(15): 1854 - 1861. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. P. Walsh Interventional Electrophysiology in Patients With Congenital Heart Disease Circulation, June 26, 2007; 115(25): 3224 - 3234. [Full Text] [PDF]
|
|
|
|
|
|
T. Oosterhof, H. W Vliegen, F. J Meijboom, A. H Zwinderman, B. Bouma, and B. J M Mulder Long-term effect of pulmonary valve replacement on QRS duration in patients with corrected tetralogy of Fallot Heart, April 1, 2007; 93(4): 506 - 509. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. P. Walsh and F. Cecchin Arrhythmias in Adult Patients With Congenital Heart Disease Circulation, January 30, 2007; 115(4): 534 - 545. [Full Text] [PDF]
|
|
|
|
|
|
E. A Shinebourne, S. V Babu-Narayan, and J. S Carvalho Tetralogy of Fallot: from fetus to adult. Heart, September 1, 2006; 92(9): 1353 - 1359. [Full Text] [PDF]
|
|
|
|
|
|
P. Khairy, M. J. Landzberg, M. A. Gatzoulis, L.-A. Mercier, S. M. Fernandes, J.-M. Cote, J.-P. Lavoie, A. Fournier, P. G. Guerra, A. Frogoudaki, et al. Transvenous Pacing Leads and Systemic Thromboemboli in Patients With Intracardiac Shunts: A Multicenter Study Circulation, May 23, 2006; 113(20): 2391 - 2397. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Frigiola, A. Giamberti, M. Chessa, M. Di Donato, R. Abella, S. Foresti, C. Carlucci, D. Negura, M. Carminati, G. Buckberg, et al. Right ventricular restoration during pulmonary valve implantation in adults with congenital heart disease Eur. J. Cardiothorac. Surg., April 1, 2006; 29(Suppl_1): S279 - S285. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. G. Williams, G. D. Pearson, R. J. Barst, J. S. Child, P. del Nido, W. M. Gersony, K. S. Kuehl, M. J. Landzberg, M. Myerson, S. R. Neish, et al. Report of the National Heart, Lung, and Blood Institute Working Group on Research in Adult Congenital Heart Disease J. Am. Coll. Cardiol., February 21, 2006; 47(4): 701 - 707. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Khairy, D. W. Ouyang, S. M. Fernandes, A. Lee-Parritz, K. E. Economy, and M. J. Landzberg Pregnancy Outcomes in Women With Congenital Heart Disease Circulation, January 31, 2006; 113(4): 517 - 524. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 I. Zeltser, J. W. Gaynor, M. Petko, R. J. Myung, M. Birbach, R. Waibel, R. F. Ittenbach, R. E. Tanel, V. L. Vetter, and L. A. Rhodes The roles of chronic pressure and volume overload states in induction of arrhythmias: An animal model of physiologic sequelae after repair of tetralogy of Fallot J. Thorac. Cardiovasc. Surg., December 1, 2005; 130(6): 1542 - 1548. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. A. Warnes The Adult With Congenital Heart Disease: Born To Be Bad? J. Am. Coll. Cardiol., July 5, 2005; 46(1): 1 - 8. [Abstract] [Full Text] [PDF]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||