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  • 1. A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects Michael J. Silka, Bruce G. Hardy, Victor D. Menashe, and Cynthia D. Morris J. Am. Coll. Cardiol. 1998;32;245-251 This information is current as of December 6, 2010 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://content.onlinejacc.org/cgi/content/full/32/1/245 Downloaded from content.onlinejacc.org by on December 6, 2010
  • 2. JACC Vol. 32, No. 1 245July 1998:245–51A Population-Based Prospective Evaluation of Risk of Sudden CardiacDeath After Operation for Common Congenital Heart DefectsMICHAEL J. SILKA, MD, FACC, BRUCE G. HARDY, MD, VICTOR D. MENASHE, MD,CYNTHIA D. MORRIS, PHD, MPHPortland, Oregon Objectives. This study sought to define 1) the risk of sudden in patients with aortic stenosis, coarctation, transposition of thedeath after operation for common congenital heart defects; and 2) great arteries or tetralogy of Fallot, an event rate of 1/454factors associated with an increased risk of sudden death. patients-years. In contrast, only four sudden deaths occurred Background. Although the prognosis for patients with congen- among the other defects, an event rate of 1/7,154 patient-yearsital heart defects is improved by surgical treatment, they remain (p < 0.01). The risk of late sudden death increased incrementallyat a well recognized but poorly defined risk of late sudden death. 20 years after operation for tetralogy of Fallot, aortic stenosis and Methods. This population-based study evaluated all patients coarctation. However, risk was not dependent on patient age at<19 years old undergoing surgical treatment of common forms of operation or surgical era. The causes of sudden death werecongenital heart disease in the state of Oregon between 1958 and arrhythmia in 30 patients, circulatory (embolic or aneurysm1996. Patients were identified retrospectively through 1958, with rupture) in 7 and acute heart failure in 4.prospective biannual follow-up beginning in 1982. The incidence Conclusions. The risk of late sudden death for patients surviv-and cause of late sudden death were evaluated for 3,589 patients ing operation for common congenital heart defects is 25 to 100surviving operation for the following defects: atrial, ventricular times greater than an age-matched control population. Thisand atrioventricular septal defects; patent ductus arteriosus; increased risk is primarily represented by patients with cyanoticpulmonary stenosis; aortic stenosis; coarctation of the aorta; or left heart obstructive lesions. The risk of sudden death appearstetralogy of Fallot; and D-transposition of the great arteries. to be time dependent, increasing primarily after the second Results. There were 41 unexpected late sudden deaths during postoperative decade.45,857 patient-years of follow-up, an overall event rate of 1/1,118 (J Am Coll Cardiol 1998;32:245–51)patient-years. Thirty-seven of the 41 late sudden deaths occurred ©1998 by the American College of CardiologySurgical treatment has significantly improved the short- and among these patients during short-term follow-up. Further-midterm prognosis of patients with congenital heart disease. more, it is uncertain whether risk factors proposed for patientsHowever, as the population of long-term survivors increases, with TOF are relevant to postoperative patients with otherunexpected late sudden death due to cardiovascular causes is forms of congenital heart disease.increasingly recognized as a potential hazard after ostensibly This study of sudden cardiac death (SCD) is part of acorrective cardiac operations (1– 6). prospective population-based study of patients who have un- At least 20 factors have been proposed as predictive of an dergone surgical treatment of congenital heart disease. Theincreased risk of late sudden death in these patients, with most overall 25-year survival of this cohort has been the subject ofstudies focusing on previous surgical repair of tetralogy of another report (8). The purpose of the present study was toFallot (TOF) (7). The heterogeneity of these risk factors specifically evaluate the risk of SCD during long-termreflects uncertainties regarding the causes and mechanisms of follow-up after surgical treatment of congenital heart disease.sudden death in patients after surgical treatment of congenital Secondary objectives were to compare the relative risks andheart disease and the relatively low incidence of sudden death causes of sudden death among the various heart defects and determine whether objective factors could be identified that predicted an increased association with late sudden death. From the Congenital Heart Disease Research Center Divisions of PediatricCardiology and Medical Informatics and Clinical Outcomes, Oregon HealthSciences University, Portland, Oregon. This study was supported by Grants R 01 Methods39052 and R 29 4L36856 from the National Heart, Lung, and Blood Institute,National Institutes of Health, Bethesda, Maryland. Patients. The study evaluated all Oregon residents under- Manuscript received October 17, 1997; revised manuscript received February going surgical treatment of congenital heart disease between2, 1998, accepted March 16, 1998. 1958 and 1996. Patient inclusion criteria were age 19 years at Address for correspondence: Dr. Michael J. Silka, mail code UHN-60,Oregon Health Sciences University, 3181 SW Sam Jackson, Portland, Oregon initial operation and survival 30 days after operation. During97201. E-mail: silkami@ohsu.edu. the study interval, 99.5% of operations for congenital heart©1998 by the American College of Cardiology Downloaded from content.onlinejacc.org by on December 6, 2010 0735-1097/98/$19.00Published by Elsevier Science Inc. PII S0735-1097(98)00187-9
  • 3. 246 SILKA ET AL. JACC Vol. 32, No. 1 SUDDEN DEATH AND CONGENITAL HEART DISEASE July 1998:245–51 D-TGA with right ventricular hypoplasia were excluded from Abbreviations and Acronyms this analysis. AS aortic stenosis Definitions. SCD was defined as acute cardiovascular col- AV atrioventricular lapse from which biologic death occurred or from which the CoA coarctation of the aorta patient never regained consciousness. The mechanisms of SCD sudden cardiac death D-TGA D-transposition of the great arteries death were classified according to modification of criteria TOF tetralogy of Fallot proposed by the Cardiac Arrhythmia Pilot Study (9): 1) sudden arrhythmic (proven or presumed); 2) sudden nonarrhythmic (i.e., embolism or aneurysm rupture); 3) nonsudden cardiac death (i.e., reoperation or congestive heart failure); and 4) noncardiac (i.e., trauma, malignancy). Patients with non-disease were performed at five hospitals in Oregon, with 0.5% sudden cardiac death or noncardiac causes of death wereof operations performed in another state. included in the cohort until the time of death. A registry of all patients with congenital heart disease was Statistical analysis. The statistical methods for this analy-initially established in 1982, with retrospective identification of sis have been previously reported in detail (8). Survival wasall patients undergoing surgical treatment of congenital heart estimated by the Kaplan-Meier method, defined as the elapseddisease since 1958. Patient accrual has been prospective since time from the date of operation to date of death or last knownthat time. After establishment of the registry, biannual follow-up. The Mantel-Cox statistic was used for comparisonfollow-up has been determined by clinical evaluation, tele- of survival between groups of patients.phone interview or mailed questionnaire. If no response wasobtained, the National Death Index was reviewed for identifi-cation of missing patients. Patients in whom earlier follow-uphad been obtained, but whose status was not determined in themost recent data cycle, were included in analysis up to the time Resultsof most recent follow-up. The most recent data cycle was Patients. According to the aforementioned inclusion crite-completed in 1996. ria, a total of 3,589 patients were identified, from whom a total For the purposes of this study, patients with the following follow-up of 45,857 patient-years was derived. The distributioncongenital heart defects were evaluated: secundum atrial sep- of the individual forms of congenital heart disease andtal defect, ventricular septal defect, atrioventricular (AV) follow-up are shown in Table 1. In this registry, 91.5% ofseptal defect (including primum atrial septal defect), patent patients are non-Hispanic white, 2.4% Asian, 3.2% Hispanic,ductus arteriosus, pulmonary stenosis, aortic valve stenosis 2.1% African-American and 0.8% native-American. The gen-(AS), coarctation of the aorta (CoA), TOF and D-transposition der distribution differs markedly by defect on the basis ofof the great arteries (D-TGA). These defects were selected natural occurrence; the groups with TOF, D-TGA, AS andbecause they represent all defects that would allow at least 20 CoA are predominately male, whereas those with an atrial oryears of postsurgical follow-up and adequate numbers to AV septal defect or patent ductus arteriosus are predominatelypermit inferential statistical analysis. Patients with complex female. Follow-up for survival through 1994 (the beginning offorms of these defects, such as AV septal defect with TOF or the last completed data cycle) was completed in 90% of the Table 1. Specific Congenital Heart Defects and Incidence of Sudden and Nonsudden Cardiac Death Nonsudden Cardiac Sudden Cardiac Death Death No. (%) of Pts Total With Complete Follow-Up Incidence/ Incidence/ Follow-Up (pt-yr) No. 1,000 Pt-yr No. 1,000 Pt-yr ASD 622 (86%) 7,904 0 0 0 0 VSD 527 (87%) 6,354 1 0.2 8 1.2 AVSD 254 (87%) 2,217 2 0.9 15 6.7 PDA 623 (82%) 8,753 0 0 4 0.4 PS 241 (91%) 3,568 1 0.3 2 0.6 AS 169 (94%) 1,860 10 5.4 9 4.8 CoA 536 (92%) 6,706 9 1.3 17 2.5 TOF 445 (91%) 7,082 11 1.5 9 1.3 D-TGA 172 (95%) 1,413 7 4.9 10 6.9 Total 3,589 45,857 41 0.9 74 1.6 AS aortic stenosis; ASD atrial septal defect; AVSD atrioventricular septal defect; CoA coarctation of the aorta; PDA patent ductus arteriosus; PS pulmonary stenosis; pt-yr patient-years; Pts patients; D-TGA dextro-transposition of the great arteries; TOF tetralogy of Fallot; VSD ventricular septal defect. Downloaded from content.onlinejacc.org by on December 6, 2010
  • 4. JACC Vol. 32, No. 1 SILKA ET AL. 247July 1998:245–51 SUDDEN DEATH AND CONGENITAL HEART DISEASEFigure 1. Documented or probable etiologies of death among the 41patients who died suddenly (arrhythmia [hatched bars], circulatory Figure 2. Actuarial probability of SCD-free survival after surgical[open bars], congestive heart failure [solid bars]). The anatomic treatment of cyanotic congenital heart defects.diagnoses of the other patients were pulmonary stenosis in one patient,ventricular septal defect in one and AV septal defect in two. specific congenital heart defects. For patients with D-TGA, the risk of late SCD was relatively constant, with the incidence ofentire cohort, ranging from 82% in patients with a patent SCD estimated at 4 7% at 10 years and 9 4% at 20 yearsductus arteriosus to 95% in patients with D-TGA. of follow-up (mean SEE). In contrast, the incidence of late Events. There were a total of 41 sudden, unexpected late sudden death for patients with a previous repair of TOFdeaths during the 45,857 patient-years, for an event rate of increased primarily after 20 years of follow-up. At 20 years of0.9/1,000 patient-years, or 1 event/1,118 patient-years. Thirty- follow-up, the incidence of SCD was 2 1%, increasing to 4seven of the 41 late SCDs occurred in patients with four 1% at 25 years and 6 7% at 30 years (Fig. 2).defects: TOF (n 11), D-TGA (n 7), CoA (n 9) and AS An apparent time-dependent incremental risk of late SCD(n 10). The cumulative event rate for patients with these was also observed for patients with left-heart obstructivefour defects was 1/454 patient-years, or 2.2 events/1,000 patient lesions. The estimated incidence of late SCD for patients withyears. In comparison, the SCD event rate for patients with left AS was 3 2% at 10 years of follow-up, increasing to 13 5%to right shunt lesions or pulmonary stenosis was 1/7,154 patient at 20 years and 20 8% at 30 years. For patients with CoA, theyears, or 0.14/1,000 patient years (p 0.001). The observed incidence of SCD was 1 0.5% at 20 years of follow-up,incidence of sudden death ranged from 0 in patients with an increasing to 5 2% at 25 years and 8 3% at 30 years (Fig.atrial septal defect or patent ductus to 5/1,000 patient-years 3). Two of the nine patients with CoA who died suddenly hadfor patients with AS or D-TGA (Table 1). During this study undergone subsequent operation for AS at 14 and 15 yearsperiod, 74 other patients died of non–sudden cardiac causes after their initial operation, respectively. However, because the(reoperation [n 32], chronic congestive heart failure [n 22] index surgical procedure for entry into this study was for CoA,and pulmonary hypertension [n 20]), whereas 61 patients these patients were included in the analysis of the cohort withdied of noncardiac causes. this defect. Mechanisms of unexpected SCD. On the basis of informa- Causes of late SCD. Several observations were madetion obtained from witnesses, medical or rescue squad reports among the patients who died suddenly: 1) of the seven patientsand autopsy data, a determination of the actual or probable with D-TGA who died suddenly, five were participating incause of SCD was made. The causes of the 41 SCDs werearrhythmia in 30 patients, circulatory in 7 (embolic in 5,aneurysm rupture in 2) and acute ventricular failure in 4. The Figure 3. Actuarial probability of SCD-free survival after surgical treatment of left heart obstructive lesions.causes of SCD among specific forms of congenital heartdisease are illustrated in Figure 1. Of the 30 patients with an arrhythmic cause of SCD, 14 hadventricular tachycardia or fibrillation documented at time ofattempted resuscitation, whereas 2 had complete AV blockwith an agonal idioventricular rhythm. Of the 14 patients witha presumed arrhythmic cause of SCD, 6 collapsed suddenlyduring exertion, and 4 died in their sleep; in 4 patients, thecircumstances at time of cardiovascular collapse were notclearly recorded. Temporal analysis of the incidence of SCD. The incidenceof late SCD was evaluated as a function of the duration offollow-up, both for the entire cohort and for patients with Downloaded from content.onlinejacc.org by on December 6, 2010
  • 5. 248 SILKA ET AL. JACC Vol. 32, No. 1 SUDDEN DEATH AND CONGENITAL HEART DISEASE July 1998:245–51Table 2. Probability of Sudden Death-Free Survival by Surgical Era those operated on in 1980 and later. This time frame wasand Duration of Follow-Up chosen because these intervals would allow comparable Postoperative Follow-Up Duration follow-up of patients with up to 15 years of follow-up dataSurgical No. of (mean SEE) during both periods. This analysis was restricted to patients Era Pts 5 Years 10 Years 15 Years with TOF, CoA and AS because of the limited number of SCD events among patients with other forms of CHD and becauseTOF 1980 265 0.99 0.01 0.99 0.01 0.99 0.01 the surgical techniques for D-TGA have evolved from the 1980 180 0.99 0.01 0.99 0.01 0.99 0.01 Mustard and Senning atrial switch procedures to arterialAS switch methods during the past decades. There was no ob- 1980 63 0.98 0.02 0.97 0.02 0.95 0.03 served difference in the incidence of late SCD for the three 1980 106 0.97 0.02 0.97 0.02 0.88 0.09 aforementioned defects through 15 years of follow-up, whetherCoA operated on before 1980 or later (Table 2). However, these 1980 236 1.0 0.99 0.01 0.99 0.01 data do not reflect the significant reductions in the perioper- 1980 300 0.99 0.01 0.99 0.01 0.99 0.01 ative deaths that have occurred over the decades. Abbreviations as in Table 1. Age at primary operation was the second variable that was evaluated in relation to the risk of late SCD. This analysis compared patients undergoing surgical repair of their congen-active physical exertion at the time of death. Polymorphic ital heart disease at age 2 years compared with thoseventricular tachycardia or fibrillation was the terminal cardiac undergoing operation at 2 years of age. Analysis for the riskrhythm in the four patients with D-TGA for whom electrocar- of late SCD by age at operation was not performed for patientsdiographic documentation was available. Six of these patients with D-TGA because the operation was usually performedhad undergone a previous Mustard procedure, whereas an within the first 3 months of life. After 15 years of follow-up, noarterial switch procedure had been performed in the other association was observed between age at operation and inci-patient. 2) Profound ventricular hypertrophy was a notable dence of late SCD for any defect (Table 3). Additionally, nofinding among several of the patients with CoA who died statistically significant association between SCD and eithersuddenly. 3) Agonal bradycardia due to advanced AV block gender, race or presence of genetic or extracardiac abnormal-was the initial documented rhythm in two patients with TOF. ities was identified.Both patients had transient postoperative AV block andresidual bifascicular block. 4) The etiologies of sudden deathwere diverse among patients with valvular AS. Significantresidual valvular stenosis or insufficiency was present in three Discussionof four patients with arrhythmic SCD. Four patients died of The unexpected SCD of a young person from cardiovascu-cerebral or coronary embolic complications, associated with lar disease is a tragic, but fortunately, extremely uncommonaortic valve endocarditis in two, whereas two patients died of event in the general population. Several population-basedlow cardiac output secondary to acute ventricular failure. studies (10 –12) have evaluated the risk of SCD in young Secondary risk factor analysis. The possible associations patients ( 20 years old), with the reported incidence betweenbetween surgical era and patient age at definitive operation 1/25,000 and 1/100,000 patient-years. The incidence of unex-and the occurrence of late SCD were evaluated. The risk of pected SCD has also been the subject of study among selectedlate SCD as a function of surgical era was considered a subpopulations of young patients, particularly young athletescomparison of patients undergoing operation before 1980 with or patients with known cardiovascular diagnoses, such as Table 3. Probability of Sudden Death-Free Survival by Age at Operation and Duration of Postoperative Follow-Up No. of Postop Follow-Up Duration (mean SEE) Age at Postop Operation (yr) Survivors 5 Years 10 Years 15 Years 20 Years 25 Years TOF 2 236 0.99 0.01 0.99 0.01 0.99 0.01 0.99 0.01 0.95 0.04 2 209 0.98 0.01 0.98 0.01 0.98 0.01 0.97 0.01 0.96 0.02 AS 2 59 0.98 0.02 0.98 0.02 0.98 0.02 0.98 0.02 2 110 0.98 0.02 0.96 0.07 0.93 0.03 0.85 0.05 0.85 0.02 CoA 2 262 0.98 0.01 0.98 0.01 0.98 0.01 0.98 0.01 0.98 0.01 2 274 1.0 0.99 0.01 0.99 0.01 0.99 0.01 0.94 0.03 Postop postoperative; other abbreviations as in Table 1. Downloaded from content.onlinejacc.org by on December 6, 2010
  • 6. JACC Vol. 32, No. 1 SILKA ET AL. 249July 1998:245–51 SUDDEN DEATH AND CONGENITAL HEART DISEASEWolff-Parkinson-White syndrome or the congenital long QT Late SCD after repair of TOF. The incidence and risksyndromes (13–15). Most of these studies have excluded factors for late SCD after operation for TOF have both beenpatients with previous surgical treatment for congenital heart the subject of multiple studies that have involved singledisease because of a presumed higher risk of late SCD. institutions as well as collaborative efforts (1,17,19,20). Al- As the general population of patients surviving operation though comparison of those studies is limited by differences infor congenital heart defects continues to achieve greater design, definition and length of follow-up, a meta-analysis of 39longevity, questions regarding the long-term prognosis for studies (21) reported a 1.8% incidence of late SCD amongthese patients become increasingly pertinent (16). These in- 4,583 patients with an average of 8.3 years of follow-up afterclude the relative risk of late SCD among patients with diverse surgical treatment of TOF. This relatively low incidence offorms of congenital heart disease, potential identification of SCD is consistent with the findings of the present study, whererisk factors for late SCD and the requirement for ongoing the incidence of late SCD at 10 years of follow-up was 1.2%. Amedical evaluation decades after ostensibly corrective cardiac relatively low incidence of late SCD after surgical treatment ofsurgery. The purpose of the present study was to address these TOF was also observed in our study during the secondissues in a population-based study of patients after operation postoperative decade, with a cumulative 20-year actuarialfor common forms of congenital heart disease, in whom incidence of late SCD of 2.2%. However, incremental in-long-term survival would be anticipated. creases in the incidence of SCD to 4% at 25 years and 6% at Late SCD, years or decades after operation for congenital 30 years of follow-up suggest a time-dependent risk of lateheart disease, has been the subject of several reports (1– 6). death.However, most reports have focused on one specific defect, Murphy et al. (22) also reported a 6% incidence of latesuch as TOF or D-TGA, in which there is a relatively high SCD among patients with 30 years of follow-up after surgicalincidence of late SCD (17,18). In contrast, one purpose of the treatment of TOF (10 deaths among 163 patients). However,present population-based study was to evaluate the compara- although identical rates of late SCD during equivalenttive risk of late SCD among patients with various common follow-up are reported in that (22) and our study, a late ordefects, given the long-term follow-up, high rates of patient time-dependent risk for late SCD was not observed by Murphyfollow-up and unbiased population study design. et al. In light of the small numbers of events in both series, the Factors associated with increased risk of late SCD. The need for cautious interpretation of data would seem prudent.two principal findings of this study were the marked variance in Conversely, it would seem reasonable to conclude that thethe relative risks for late SCD among patients with various earlier estimates of a 5% to 10% risk of late SCD duringdefects and the apparent time-dependent nature of this risk for adolescence after repair of TOF have not been substantiatedcertain defects. Overall, the incidence of late SCD in the entire by long-term follow-up.cohort was 0.9/1,000 patient-years, representing an increased Late SCD in patients with AS. The highest incidence of laterisk of between 25 and 100 times that for the general popula- SCD in the present series was observed among patients withtion. However, the incidence of late SCD of 1/7,154 patient- AS, with an incidence of 3% at 10 years of follow-up, increas-years for patients with left to right intracardiac shunt lesions or ing to 13% at 20 years and 20% at 30 years of follow-up. Thispulmonary stenosis approaches that of the general population finding is somewhat surprising in view of the paucity of(1/25,000 to 100,000 patient years). In contrast, the incidence published data on SCD in young patients with AS. The issue ofof late SCD was markedly increased in patients with left heart late SCD among postsurgical patients with AS was noted byobstructive lesions and cyanotic defects, with the estimated risk the Second Natural History Study of Congenital Heart Defectsbetween 50 and 200 times that of the general population. The (23). In that study, there were 19 late unexpected SCDsmarked increase in the incidence of late SCD was primarily (presumably arrhythmic) and 4 deaths from bacterial endocar-accounted for by patients with AS and D-TGA, in whom the ditis among 240 surgically managed patients. These eventsincidence of late SCD was 1/200 patient-years. occurred over 15 years of follow-up and represent a nearly Late SCD after repair of D-TGA. Further evaluation of the 10% incidence of late SCD during this interval. A significantincidence of SCD for specific defects demonstrated an early incidence of arrhythmic SCD was also observed among youngonset and relatively constant risk for patients undergoing the patients with AS in registry data reported for patients who hadMustard or Senning procedures for D-TGA, with estimated undergone implantable cardioverter-defibrillator placementSCD-free survival rates of 96% at 10 years and 91% at 20 years after resuscitation from sudden cardiac arrest (24). Also, theof postoperative follow-up. The risk of late SCD in patients risk of late SCD from embolic sequelae of endocarditis may bewith D-TGA in this series is similar to that reported in separate an underestimated factor in the evaluation of these patients.series by Gewillig et al. (6) and Gelatt et al. (18), although Late SCD after repair of CoA. The finding of late SCDthose two studies primarily evaluated total actuarial survival among patients with CoA was unanticipated because only 1 ofrather than late SCD. In those two studies, actuarial total 536 survivors died suddenly during the first 20 years ofcardiac death-free survival rates of 67% and 76% at 20 years of follow-up. However, recent studies (25) have reported unex-postsurgical follow-up were reported. By comparison, the total plained advanced left ventricular hypertrophy in patients withcardiac death-free survival rate for patients in the present previous repair of CoA, with the suggestion that the biologicstudy with D-TGA at 20 years was 71%. template for advanced ventricular hypertrophy may be estab- Downloaded from content.onlinejacc.org by on December 6, 2010
  • 7. 250 SILKA ET AL. JACC Vol. 32, No. 1 SUDDEN DEATH AND CONGENITAL HEART DISEASE July 1998:245–51lished at a young age, with complete expression only during failure or coronary embolism, although these arrhythmias wereearly adulthood. This finding emphasizes the need for ongoing interpreted as sequelae rather than causes of SCD.follow-up of these patients despite apparent complete relief of Conclusions. 1) The risk of late SCD during long-termaortic obstruction at rest. Similar caution and concern are also follow-up is diverse among patients with heterogeneous formswarranted for patients after patch aortoplasty repair of CoA, of congenital heart disease. For some, the risk is only slightlywho require long-term follow-up for both the occurrence and higher than the general population; conversely, a much highersignificance of aortic aneurysm after this procedure (26). risk is present for patients with other defects, such as AS or Factors not associated with increased risk of late SCD. D-TGA. 2) The risk of late SCD may be time dependent forTwo additional issues that were examined in our study were high risk defects, increasingly primarily 20 years after oper-impact of the surgical era on late incidence of SCD and ation. Further follow-up will be required to determine whetherwhether early repair (age 2 years) was associated with a this risk is linear or exponential. 3) Most but not all SCD inlower risk of late SCD. With regard to surgical era (before these patients is presumably due to arrhythmic causes. Future1980 vs. 1980 and later), no difference in the incidence of late studies should consider whether some late SCDs may be due toSCD was observed for the same defects with equal duration of circulatory rather than arrhythmic causes.follow-up. However, there were distinct differences in theoperative mortalities for the two eras, a subject that has beenaddressed previously (8). Additional follow-up of the cohort Referencesthrough 30 years after operation will be required to determine 1. Gillette PC, Yeoman MA, Mullins CE, McNamara DG. Sudden death afterwhether the trend for increasing rates of mortality over a repair of tetralogy of Fallot: electrocardiographic and electrophysiologiclonger postoperative interval will be repeated in the group abnormalities. Circulation 1977;56:566 –71.operated on in the more recent era. However, it would appear 2. Garson A, McNamara DG. Sudden death in a pediatric cardiology popula- tion, 1958 to 1983: relation to prior arrhythmias. J Am Coll Cardiol 1985;5that the duration of postsurgical follow-up rather than the Suppl B:134B–7B.surgical era may be the critical factor in defining the risk of late 3. Bharati S, Lev M. Conduction system in cases of sudden death in congenitalSCD. heart disease many years after surgical correction. Chest 1986;90:861– 8. With recent advances in surgical techniques, it has been 4. Shen WK, Holmes DR Jr, Porter CJ, McGoon DC, Ilstrup DM. Sudden death after repair of double-outlet right ventricle. Circulation 1990;81:128 –proposed that early primary repair may reduce the risk of late 36.SCD (27). However, others have suggested that longer 5. Silka MJ, Kron J, Walance CG, Cutler JE, McAnulty JH. Assessment andfollow-up will be required to substantiate this observation. The follow-up of pediatric survivors of sudden cardiac death. Circulation 1990; 82:341–9.results of the present study are consistent with the latter 6. Gewillig M, Cullen S, Mertens B, Lesaffre E, Deanfield J. Risk factors forproposal: Decades rather than years of follow-up may be arrhythmia and death after Mustard operation for simple transposition ofrequired to validate a benefit of early primary repair. In our the great arteries. Circulation 1991;84 Suppl III:III-187–92.study, comparison of patients undergoing operation at 2 7. Bricker JT. Sudden death and tetralogy of Fallot: risks, markers and causes. Circulation 1995;92:162–3.years versus 2 years of age failed to demonstrate a significant 8. Morris CD, Menashe VD. Twenty-five-year mortality after surgical repair ofdifference in the incidence of late SCD with up to 25 years of congenital heart defect in childhood: a population-based cohort study.follow-up. Although it is possible that significant differences JAMA 1991;266:3447–52. 9. Greene HL, Richardson DW, Barker AH, et al. Classification of deaths aftermay emerge with longer follow-up, these differences have yet myocardial infarction as arrhythmic or non-arrhythmic: the Cardiac Arrhyth-to be established. mia Pilot Study. Am J Cardiol 1989;63:1– 6. Study limitations. The present population-based study of 10. National Center for Health Statistics. Vital Statistics of the United States,patients undergoing operation for common congenital heart 1986, II, Section 6, Life tables. Washington (DC): Public Health Service, 1988. Department of Health and Human Services Publ PHS 88-1147.defects since 1958 included only residents of Oregon. A small 11. Neuspiel DR, Kuller LH. Sudden and unexpected death in childhood andnumber of patients have incomplete follow-up data because of adolescence. JAMA 1985;254:1321–5.patient relocation or failure to respond to inquiries on status. 12. Driscoll DJ, Edwards WD. Sudden unexpected death in childhood and adolescence. J Am Coll Cardiol 1985;5 Suppl B:118B–21B.However, it is unlikely that catastrophic events would not have 13. Maron BJ, Shirani J, Poliac LC, Mathenge R, Roberts WC, Mueller FO.been detected in light of the multiple methods of investigation Sudden death in young competitive athletes: clinical, demographic andand 90% follow-up through 1996. Also, because of the obser- pathologic profiles. JAMA 1996;276:199 –204.vational study design, there are distinct limitations in defining 14. Munger TM, Packer DL, Hammill SC, et al. A population study of the natural history of Wolff-Parkinson-White syndrome in Olmstead County,secondary risk factors for late SCD, such as the lack of Minnesota, 1953–1989. Circulation 1993;87:866 – 873.consistent objective data regarding measures of ventricular 15. Garson A Jr, Dick M II, Fournier A, et al. The long QT syndrome infunction. children: an international study of 287 patients. Circulation 1993;87:1866 – 72. 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  • 8. JACC Vol. 32, No. 1 SILKA ET AL. 251July 1998:245–51 SUDDEN DEATH AND CONGENITAL HEART DISEASE Long-term prognosis (15–26 years) after repair of tetralogy of Fallot: I. 24. Silka MJ, Kron J, Dunnigan A, Dick M II, for the Pediatric Electrophysiol- Survival and symptomatic status. Ann Thorac Surg 1984;38:151– 6. ogy Society. Sudden cardiac death and the use of implantable cardioverter-20. Chandar JS, Wolff GS, Garson A Jr, et al. Ventricular arrhythmias in defibrillators in pediatric patients. Circulation 1993;87:800 –7. postoperative tetralogy of Fallot. Am J Cardiol 1990;65:655– 61. 25. Kimball BP, Shurvell BL, Houle S, Fulop JC, Rakowski H, McLaughlin PR.21. Garson A Jr. Ventricular arrhythmias after repair of congenital heart Persistent ventricular adaptations in postoperative coarctation of the aorta. disease: who needs treatment? Cardiol Young 1991;1:177– 81. J Am Coll Cardiol 1986;8:172– 8.22. Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in patients 26. Bromberg BI, Beekman RH, Rocchini AP, et al. Aortic aneurysm after patch undergoing surgical repair of tetralogy of Fallot. N Engl J Med 1993;329: aortoplasty repair of coarctation: a prospective analysis of prevalence, 593–9. screening tests and risks. J Am Coll Cardiol 1989;14:734 – 41.23. Keane JF, Driscoll DJ, Gersony WM, et al. Second natural history study of 27. Walsh EP, Rockenmacher S, Keane JF, Hougen TJ, Lock JE, Castaneda congenital heart defects: results of treatment of patients with valvar aortic AR. Late results in patients with tetralogy of Fallot repaired during infancy. stenosis. Circulation 1993;87 Suppl I:I-16 –27. Circulation 1988;77:1062–7. Downloaded from content.onlinejacc.org by on December 6, 2010
  • 9. A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects Michael J. Silka, Bruce G. Hardy, Victor D. Menashe, and Cynthia D. Morris J. Am. Coll. Cardiol. 1998;32;245-251 This information is current as of December 6, 2010Updated Information including high-resolution figures, can be found at:& Services http://content.onlinejacc.org/cgi/content/full/32/1/245References This article cites 24 articles, 15 of which you can access for free at: http://content.onlinejacc.org/cgi/content/full/32/1/245#BIBLCitations This article has been cited by 40 HighWire-hosted articles: http://content.onlinejacc.org/cgi/content/full/32/1/245#otherartic lesRights & Permissions Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://content.onlinejacc.org/misc/permissions.dtlReprints Information about ordering reprints can be found online: http://content.onlinejacc.org/misc/reprints.dtl Downloaded from content.onlinejacc.org by on December 6, 2010

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