Sudden cardiac death in adult congenital heart disease
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BACKGROUND: Sudden cardiac death (SCD) is a major cause of mortality in adults with congenital heart disease (CHD). The aim of this study was to determine the adult CHD population at risk of SCD and the clinical parameters associated with SCD. METHODS AND RESULTS: We performed a multicenter case-control study. Patients who died suddenly as a result of proven or presumed arrhythmia were included (cases). For each case, 2 controls matched on diagnosis, type of surgical intervention, age, and gender were included. From 3 databases including 25 790 adults with CHD, 1189 deaths (5%) were identified, of whom 213 patients (19%) died suddenly. Arrhythmic death occurred in 171 of 1189 patients. The underlying cardiac lesions were mild, moderate, and severe CHD in 12%, 33%, and 55% of the SCD cases, respectively. Clinical variables associated with SCD were supraventricular tachycardia (odds ratio [OR], 3.5; 95% confidence interval [CI], 1.5-7.9; P=0.004), moderate to severe systemic ventricular dysfunction (OR, 3.4; 95% CI, 1.1-10.4; P=0.034), moderate to severe subpulmonary ventricular dysfunction (OR, 3.4; 95% CI, 1.1-10.2; P=0.030), increased QRS duration (OR, 1.34 [per 10-ms increase]; 95% CI, 1.10-1.34; P=0.008), and QT dispersion (OR, 1.22 [per 10-ms increase]; 95% CI, 1.22-1.48; P=0.008). CONCLUSIONS: The clinical parameters found to be associated with SCD in adults with a broad spectrum of CHD, including systemic right ventricles, are similar to those in ischemic heart disease. Moreover, even those patients with mild cardiac lesions are potentially at risk for SCD. This highlights the need for further prospective studies as well as vigilant ongoing follow-up of the adult with CHD.
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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 [352]
Atrial tachycardias and impaired ventricular function are important and consistent risk factors for SCD in CHD; corrected Tetralogy of Fallot, post-atrial switch Mustard or Senning, left heart obstructed lesions and univentricular hearts have the highest (2-9% per decade) risk of SCD.
“Atrial tachycardias and impaired ventricular function are important and consistent risk factors for SCD in patients with CHD, of which corrected Tetralogy of Fallot, post-atrial switch operation Mustard or Senning, left heart obstructed lesions and univentricular hearts have the highest (2 -9% per decade) risk of SCD.352 -354”
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 [352]
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- Authors
- Koyak, Z., Harris, L., de Groot, J. R., Silversides, C. K., Oechslin, E. N., Bouma, B. J., Budts, W., Zwinderman, A. H., Van Gelder, I. C., & Mulder, B. J. M.
- Affiliations
- University Medical Center Groningen (NL)
- From the Departments of Cardiology (Z.K., J.R.d.G., B.J.B., B.J.M.M.) and Clinical Epidemiology and Biostatistics (A.H.Z.), Academic Medical Center Amsterdam, Amsterdam, Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, Netherlands (Z.K., B.J.M.M.); Division of Cardiology, Peter Munk Cardiac Centre, Toronto Congenital Cardiac Centre for Adults, and University of Toronto, Toronto, Ontario, Canada (L.H., C.K.S., E.N.O.); Department of Cardiology, UZ Leuven, Leuven, Belgium...
- ORCID
- Venue
- Circulation
- Published
- 2012-10-16
- Volume
- 126
- Issue
- 16
- Pages
- 1944-1954
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2061341959
- Cited by
- 378
- References
- 32
- Citations by year
- 2026: 10 · 2025: 17 · 2024: 20 · 2023: 34 · 2022: 22 · 2021: 26 · 2020: 37 · 2019: 26 · 2018: 37 · 2017: 39 · 2016: 36 · 2015: 27 · 2014: 27 · 2013: 18 · 2012: 1
- FWCI
- 17.33
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- Pure Amsterdam UMC (publishedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Lirias (submittedVersion)
- University of Groningen research database (University of Groningen / Centre for Information Technology) (submittedVersion)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
- Keywords
- sudden cardiac death, adult congenital heart disease, arrhythmic death, supraventricular tachycardia, ventricular dysfunction, QRS duration, QT dispersion, risk factors, systemic right ventricle
- MeSH terms
- Adult, Aortic Diseases, Arrhythmias, Cardiac, Eisenmenger Complex, Electrocardiography, Female, Heart Defects, Congenital, Humans, Male, Middle Aged, Registries, Retrospective Studies, Risk Factors, Tetralogy of Fallot, Transposition of Great Vessels, Case-Control Studies, Death, Sudden, Cardiac
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