A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects
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Objectives This study sought to define 1) the risk of sudden death after operation for common congenital heart defects; and 2) factors associated with an increased risk of sudden death.Background Although the prognosis for patients with congenital heart defects is improved by surgical treatment, they remain at a well recognized but poorly defined risk of late sudden death.Methods This population-based study evaluated all patients < 19 years old undergoing surgical treatment of common forms of congenital heart disease in the state of Oregon between 1958 and 1996. Patients were identified retrospectively through 1958, with prospective biannual follow-up beginning in 1982. The incidence and cause of late sudden death were evaluated for 3,589 patients surviving operation for the following defects: atrial, ventricular and atrioventricular septal defects; patent ductus arteriosus; pulmonary stenosis; aortic stenosis; coarctation of the aorta; tetralogy of Fallot; and D-transposition of the great arteries.Results There were 41 unexpected late sudden deaths during 45,857 patient-years of follow-up, an overall event rate of 1/1,118 patient-years. Thirty-seven of the 41 late sudden deaths occurred in patients with aortic stenosis, coarctation, transposition of the great arteries or tetralogy of Fallot, an event rate of 1/454 patients-years. In contrast, only four sudden deaths occurred among the other defects, an event rate of 1/7,154 patient-years (p < 0.01). The risk of late sudden death increased incrementally 20 years after operation for tetralogy of Fallot, aortic stenosis and coarctation. However, risk was not dependent on patient age at operation or surgical era. The causes of sudden death were arrhythmia in 30 patients, circulatory (embolic or aneurysm rupture) in 7 and acute heart failure in 4.Conclusions The risk of late sudden death for patients surviving operation for common congenital heart defects is 25 to 100 times greater than an age-matched control population. This increased risk is primarily represented by patients with cyanotic or left heart obstructive lesions. The risk of sudden death appears to be time dependent, increasing primarily after the second postoperative decade.
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Even though the incidence of SCD is low (0.09% per year) in the CHD population, it is higher than in age-matched controls, and related to arrhythmias in 14% of all deaths after initial repair.
“Even though the incidence of SCD is low (0.09% per year) in the CHD population, it is higher than in age-matched controls, 351 and related to arrhythmias in 14% of all deaths after initial repair.352,353”
From European Heart Rhythm Association (EHRA) consensus document on the management of supraventricular arrhythmias, endorsed by Heart Rhythm Society (HRS), Asia-Pacific Heart Rhythm Society (APHRS), and Sociedad Latinoamericana de Estimulacion Cardiaca y Electrofisiologia (SOLAECE) (2017) · cites this paper as [351]
Among young persons with known congenital heart disease, the highest risks of sudden death (0.2% per year) occur with specific conditions such as aortic stenosis.
“Among young persons with known congenital heart disease, the highest risks of sudden death (0.2% per year) occur with specific conditions such as aortic stenosis [128].”
From European guidelines on managing adverse effects of medication for ADHD (2011) · cites this paper as [128]
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Hot Topics in Tetralogy of Fallot
Villafañe, J., Feinstein, J. A., Jenkins, K. J., Vincent, R. N., Walsh, E. P., Dubin, A. M., Geva, T., Towbin, J. A., Cohen, M. S., Fraser, C. D., et al. (2013) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2013.07.100 · Cited by 232 · see citing works
From Selective Parasympathetic Modulation of AV Node to Rate Control Therapy
Billette, J., & Lavallée, M. (2005) · Journal of Cardiovascular Electrophysiology · doi:10.1111/j.1540-8167.2005.00271.x · Cited by 222 · see citing works
Silversides, C. K., Kiess, M. C., Beauchesne, L., Bradley, T. J., Connelly, M. S., Niwa, K., Mulder, B. J. M., Webb, G. D., Colman, J. M., & Therrien, J. (2010) · Canadian Journal of Cardiology · doi:10.1016/s0828-282x(10)70355-x · Cited by 210 · see citing works
Sudden death in children and adolescents
Wren, C., O’Sullivan, J., & Wright, C. (2000) · Heart · doi:10.1136/heart.83.4.410 · Cited by 186 · see citing works
Katritsis, D. G., Boriani, G., Cosio, F. G., Hindricks, G., Jaïs, P., Josephson, M. E., Keegan, R., Kim, Y.-H., Knight, B. P., Kuck, K.-H., et al. (2017) · Europace · doi:10.1093/europace/euw301 · Cited by 188 · see citing works
Zipes, D. P., Camm, A. J., Borggrefe, M., Buxton, A. E., CHAITMAN, B. R., FROMER, M. A., Gregoratos, G., Klein, G. J., Myerburg, R. J., Quiñones, M. A., et al. (2006) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2006.07.008 · Cited by 182 · see citing works
Self-citationKhairy, P., Landzberg, M. J., Lambert, J., & O’Donnell, C. P. (2004) · Cardiology in the Young · doi:10.1017/s1047951104003063 · Cited by 181 · see citing works
Interventional Electrophysiology in Patients With Congenital Heart Disease
Walsh, E. P. (2007) · Circulation · doi:10.1161/circulationaha.106.655753 · Cited by 173 · see citing works
Sudden Cardiac Death in the Young
Ackerman, M. J., Atkins, D. L., & Triedman, J. K. (2016) · Circulation · doi:10.1161/circulationaha.115.020254 · Cited by 169 · see citing works
Pillutla, P., Shetty, K. D., & Foster, E. (2009) · American Heart Journal · doi:10.1016/j.ahj.2009.08.014 · Cited by 165 · see citing works
Giardini, A., Specchia, S., Tacy, T. A., Coutsoumbas, G., Gargiulo, G., Donti, A., Formigari, R., Bonvicini, M., & Picchio, F. M. (2007) · The American Journal of Cardiology · doi:10.1016/j.amjcard.2006.12.076 · Cited by 160 · see citing works
2023 HRS expert consensus statement on the management of arrhythmias during pregnancy
Joglar, J. A., Kapa, S., Saarel, E. V., Dubin, A. M., Görenek, B., Hameed, A. B., de Melo, S. L., Leal, M. A., Mondesert, B. A., Pacheco, L. D., et al. (2023) · Heart Rhythm · doi:10.1016/j.hrthm.2023.05.017 · Cited by 165 · see citing works
Shah, M. J., Silka, M. J., Silva, J. N. A., Balaji, S., Beach, C. M., Benjamin, M. N., Berul, C. I., Cannon, B. C., Cecchin, F., Cohen, M. I., et al. (2021) · Heart Rhythm · doi:10.1016/j.hrthm.2021.07.038 · Cited by 155 · see citing works
Self-citationThe impact of pulmonary valve replacement after tetralogy of Fallot repair: a matched comparison
Gengsakul, A., Harris, L., Bradley, T. J., Webb, G. D., Williams, W. G., Siu, S. C., Merchant, N., & McCrindle, B. W. (2007) · European Journal of Cardio-Thoracic Surgery · doi:10.1016/j.ejcts.2007.06.009 · Cited by 145 · see citing works
Circumstances of death in adult congenital heart disease
Zomer, A. C., Vaartjes, I. C. H., Uiterwaal, C. S. P. M., van der Velde, E. T., van den Merkhof, L. F. M., Baur, L. H. B., Ansink, T. J. M., Cozijnsen, L., Pieper, P. G., Meijboom, F. J., et al. (2010) · International Journal of Cardiology · doi:10.1016/j.ijcard.2010.09.015 · Cited by 138 · see citing works
Risk of sudden death in the Williams–Beuren syndrome
Wessel, A., Gravenhorst, V. D., Buchhorn, R., Gosch, A., Partsch, C., & Pankau, R. (2004) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.30012 · Cited by 133 · see citing works
Unexpected death after reconstructive surgery for hypoplastic left heart syndrome
Mahle, W. T., Spray, T. L., Gaynor, J. W., & Clark, B. J. (2001) · The Annals of Thoracic Surgery · doi:10.1016/s0003-4975(00)02324-9 · Cited by 132 · see citing works
Value of Programmed Ventricular Stimulation in Patients with Congenital Heart Disease
Alexander, M. E., Walsh, E. P., Saul, J. P., EPSTEIN, M. R., & Triedman, J. K. (1999) · Journal of Cardiovascular Electrophysiology · doi:10.1111/j.1540-8167.1999.tb00274.x · Cited by 122 · see citing works
Vehmeijer, J. T., Brouwer, T. F., Limpens, J., Knops, R. E., Bouma, B. J., Mulder, B. J. M., & de Groot, J. R. (2016) · European Heart Journal · doi:10.1093/eurheartj/ehv735 · Cited by 119 · see citing works
2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease
Gurvitz, M. Z., Krieger, E. V., Fuller, S., Davis, L. L., Kittleson, M. M., Aboulhosn, J., Bradley, E. A., Buber, J., Daniels, C. J., Dimopoulos, K., et al. (2025) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2025.09.006 · Cited by 132 · see citing works
Zipes, D. P., Camm, A. J., Borggrefe, M., Buxton, A. E., CHAITMAN, B. R., FROMER, M. A., Gregoratos, G., Klein, G. J., Moss, A. J., Myerburg, R. J., et al. (2006) · Circulation · doi:10.1161/circulationaha.106.178104 · Cited by 118 · see citing works
Self-citationArrhythmias in adults with congenital heart disease
Triedman, J. K. (2002) · Heart · doi:10.1136/heart.87.4.383 · Cited by 115 · see citing works
Natural and unnatural history of pulmonary atresia
Leonard, H., Derrick, G. P., O'Sullivan, J., & Wren, C. (2000) · Heart · doi:10.1136/heart.84.5.499 · Cited by 115 · see citing works
Yap, S., Roos‐Hesselink, J. W., Hoendermis, E. S., Budts, W., Vliegen, H. W., Mulder, B. J. M., van Dijk, A. P. J., Schalij, M. J., & Drenthen, W. (2006) · European Heart Journal · doi:10.1093/eurheartj/ehl306 · Cited by 112 · see citing works
Gallego, P., Gonzalez, A. E., Sanchez-Recalde, A., Peinado, R., Polo, L., Gomez-Rubin, C., Lopez-Sendon, J. L., & Oliver, J. M. (2012) · The American Journal of Cardiology · doi:10.1016/j.amjcard.2012.02.057 · Cited by 106 · see citing works
Predicting Survival in Repaired Tetralogy of Fallot
Ghonim, S., Gatzoulis, M. A., Ernst, S., Li, W., Moon, J. C., Smith, G. C., Heng, E. L., Keegan, J., Ho, S. Y., McCarthy, K. P., et al. (2022) · JACC: Cardiovascular Imaging · doi:10.1016/j.jcmg.2021.07.026 · Cited by 104 · see citing works
Long-Term and Developmental Outcomes of Children with Complex Congenital Heart Disease
Brown, M. D., Wernovsky, G., Mussatto, K. A., & Berger, S. (2005) · Clinics in Perinatology · doi:10.1016/j.clp.2005.09.008 · Cited by 101 · see citing works
Pregnancy Outcomes After Atrial Repair for Transposition of the Great Arteries
Canobbio, M. M., Morris, C. D., Graham, T. P., & Landzberg, M. J. (2006) · The American Journal of Cardiology · doi:10.1016/j.amjcard.2006.03.050 · Cited by 100 · see citing works
Self-citationKrieger, E. V., Zeppenfeld, K., DeWitt, E. S., Duarte, V. E., Egbe, A. C., Haeffele, C. L., Lin, K. Y., Robinson, M., Sillman, C., & Upadhyay, S. (2022) · Circulation Arrhythmia and Electrophysiology · doi:10.1161/hae.0000000000000084 · Cited by 96 · see citing works
Rydman, R., Gatzoulis, M. Α., Ho, S. Y., Ernst, S., Swan, L., Li, W., Wong, T., Sheppard, M. N., McCarthy, K. P., Roughton, M., et al. (2015) · Circulation Cardiovascular Imaging · doi:10.1161/circimaging.114.002628 · Cited by 95 · see citing works
Risk stratification in surgically repaired tetralogy of Fallot
Khairy, P., Dore, A., Poirier, N. C., Marcotte, F., Ibrahim, R., Mongeon, F., & Mercier, L. (2009) · Expert Review of Cardiovascular Therapy · doi:10.1586/erc.09.38 · Cited by 95 · see citing works
Radbill, A. E., Triedman, J. K., Berul, C. I., Fynn‐Thompson, F., Atallah, J., Alexander, M. E., Walsh, E. P., & Cecchin, F. (2009) · Heart Rhythm · doi:10.1016/j.hrthm.2009.10.014 · Cited by 94 · see citing works
Thromboprophylaxis for atrial arrhythmias in congenital heart disease: A multicenter study.
Khairy, P., Aboulhosn, J., Broberg, C., Cohen, S., Cook, S. C., Dore, A., Fernandes, S. M., Fournier, A., Kay, J. D., Lévesque, S., et al. (2016) · International Journal of Cardiology · doi:10.1016/j.ijcard.2016.08.223 · Cited by 86 · see citing works
Sudden cardiac death in congenital heart disease
Khairy, P., Silka, M. J., Moore, J. P., DiNardo, J. A., Vehmeijer, J. T., Sheppard, M. N., Bruaene, A. V. D., Chaix, M., Brida, M., Moore, B. M., et al. (2022) · European Heart Journal · doi:10.1093/eurheartj/ehac104 · Cited by 92 · see citing works
Self-citationPremature death in adults with 22q11.2 deletion syndrome
Bassett, A. S., Chow, E. W. C., Husted, J., Hodgkinson, K. A., Oechslin, E. N., Harris, L., & Silversides, C. (2009) · Journal of Medical Genetics · doi:10.1136/jmg.2008.063800 · Cited by 90 · see citing works
Implantable cardioverter defibrillator (ICD) in children
Eicken, A., Kolb, C., Lange, S., Brodherr-Heberlein, S., Zrenner, B., Schreiber, C., & Hess, J. (2005) · International Journal of Cardiology · doi:10.1016/j.ijcard.2005.02.048 · Cited by 83 · see citing works
Update on Interventional Electrophysiology in Congenital Heart Disease
Sherwin, E. D., Triedman, J. K., & Walsh, E. P. (2013) · Circulation Arrhythmia and Electrophysiology · doi:10.1161/circep.113.000313 · Cited by 83 · see citing works
Congenital heart disease: A primer for the pediatric neuropsychologist
Cassidy, A. R., Ilardi, D. L., Bowen, S. R., Hampton, L., Heinrich, K. P., Loman, M. M., Sanz, J. H., & Wolfe, K. R. (2017) · Child Neuropsychology · doi:10.1080/09297049.2017.1373758 · Cited by 82 · see citing works
McCracken, C., Spector, L. G., Menk, J. S., Knight, J., Vinocur, J. M., Thomas, A. S., Oster, M. E., Louis, J. D. S., Moller, J. H., & Kochilas, L. K. (2018) · Journal of the American Heart Association · doi:10.1161/jaha.118.010624 · Cited by 75 · see citing works
Sudden death in children and adolescents
Wren, C. (2002) · Heart · doi:10.1136/heart.88.4.426 · Cited by 73 · see citing works
Lin, Y., Liu, P., Wu, L., Chen, Y., Chang, C., & Chu, P. (2014) · BMC Cardiovascular Disorders · doi:10.1186/1471-2261-14-38 · Cited by 73 · see citing works
Risk factors for excess mortality in adults with congenital heart diseases
Oliver, J. M., Gallego, P., Gonzalez, A. E., Garcia-Hamilton, D., Ávila, P., Yotti, R., González, I. F., & Fernández‐Avilés, F. (2016) · European Heart Journal · doi:10.1093/eurheartj/ehw590 · Cited by 73 · see citing works
Ventricular arrhythmias and sudden cardiac death in adults with congenital heart disease
Khairy, P. (2016) · Heart · doi:10.1136/heartjnl-2015-309069 · Cited by 72 · see citing works
Shah, M. J., Silka, M. J., Silva, J. N. A., Balaji, S., Beach, C. M., Benjamin, M. N., Berul, C. I., Cannon, B. C., Cecchin, F., Cohen, M. I., et al. (2021) · Cardiology in the Young · doi:10.1017/s1047951121003413 · Cited by 74 · see citing works
Self-citationClinical Use of Electrocardiography in Adults With Congenital Heart Disease
Khairy, P., & Marelli, A. J. (2007) · Circulation · doi:10.1161/circulationaha.107.691568 · Cited by 70 · see citing works
Biomaterials Advances in Patches for Congenital Heart Defect Repair
Pok, S., & Jacot, J. G. (2011) · Journal of Cardiovascular Translational Research · doi:10.1007/s12265-011-9289-8 · Cited by 68 · see citing works
Arrhythmias in Patients with Congenital Heart Disease
Walsh, E. P. (2002) · Cardiac Electrophysiology Review · doi:10.1023/a:1021192526642 · Cited by 68 · see citing works
Cardiac MR Imaging Assessment Following Tetralogy of Fallot Repair
Norton, K. I., Tong, C., Glass, R. B. J., & Nielsen, J. C. (2006) · RadioGraphics · doi:10.1148/rg.261055064 · Cited by 67 · see citing works
Risks and Benefits of Exercise Training in Adults With Congenital Heart Disease
Chaix, M., Marcotte, F., Dore, A., Mongeon, F., Mondesert, B. A., Mercier, L., & Khairy, P. (2015) · Canadian Journal of Cardiology · doi:10.1016/j.cjca.2015.12.007 · Cited by 66 · see citing works
Khairy, P. (2007) · Nature Clinical Practice Cardiovascular Medicine · doi:10.1038/ncpcardio0882 · Cited by 66 · see citing works
Sudden cardiac death in children and adolescents (excluding Sudden Infant Death Syndrome)
Saul, P. J., & Gajewski, K. (2010) · Annals of Pediatric Cardiology · doi:10.4103/0974-2069.74035 · Cited by 65 · see citing works
Catheter ablation in transposition of the great arteries with Mustard or Senning baffles
Khairy, P., & Hare, G. F. V. (2008) · Heart Rhythm · doi:10.1016/j.hrthm.2008.11.022 · Cited by 65 · see citing works
Motonaga, K. S., & Dubin, A. M. (2014) · Circulation · doi:10.1161/circulationaha.113.001383 · Cited by 64 · see citing works
Arrhythmias in adult congenital heart disease
Khairy, P., Dore, A., Talajic, M. R., Dubuc, M., Poirier, N. C., Roy, D., & Mercier, L. (2006) · Expert Review of Cardiovascular Therapy · doi:10.1586/14779072.4.1.83 · Cited by 64 · see citing works
Kreipke, R. E., & Birren, S. J. (2015) · The Journal of Physiology · doi:10.1113/jp270917 · Cited by 65 · see citing works
- Authors
- Silka, M. J., Hardy, B. G., Menashe, V. D., & Morris, C. D.
- Affiliations
- Oregon Health & Science University (US)
- Corresponding author
- Silka, Michael J
- Venue
- Journal of the American College of Cardiology
- Publisher
- Elsevier BV
- Published
- 1998-07
- Volume
- 32
- Issue
- 1
- Pages
- 245-251
- ISSN
- 0735-1097 (ISSN-L)
- OpenAlex
- W2029421014
- Cited by
- 560
- References
- 28
- Citations by year
- 2026: 5 · 2025: 13 · 2024: 11 · 2023: 18 · 2022: 15 · 2021: 13 · 2020: 19 · 2019: 18 · 2018: 29 · 2017: 40 · 2016: 38 · 2015: 23 · 2014: 19 · 2013: 36 · 2012: 30
- FWCI
- 6.96
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of the American College of Cardiology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health
- U.S. Public Health Service (R 0139052, R 29 4L36856)
- National Heart, Lung, and Blood Institute
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cardiovascular and Diving-Related Complications
- Health Sciences › Medicine › Surgery › Cardiac Structural Anomalies and Repair
- Keywords
- sudden cardiac death, congenital heart disease, postoperative mortality, tetralogy of Fallot, transposition of the great arteries, coarctation of the aorta, aortic stenosis, cyanotic congenital heart disease, cardiac arrhythmias, risk factors, long-term follow-up
- MeSH terms
- Adolescent, Adult, Cause of Death, Child, Child, Preschool, Female, Follow-Up Studies, Heart Defects, Congenital, Humans, Infant, Infant, Newborn, Male, Oregon, Postoperative Complications, Prospective Studies, Risk, Survival Analysis, Death, Sudden, Cardiac
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