The DOI matches its Crossref record; 37 of the 37 references it cites were found in a registry or an index.
Sudden Death and Defibrillators in Transposition of the Great Arteries With Intra-atrial Baffles: A Multicenter Study
Abstract
BACKGROUND: Transposition of the great arteries with intra-atrial baffle repair is among the congenital heart defects at highest risk of sudden death. Little is known about mechanisms of sudden death and the role of implantable cardioverter defibrillators. METHODS AND RESULTS: We conducted a multicenter cohort study in patients with transposition of the great arteries to determine actuarial rates of implantable cardioverter defibrillator shocks, identify risk factors, assess underlying arrhythmias, and characterize complications. Overall, 37 patients (age, 28.0+/-7.6 years; 89.2% male) were enrolled from 7 sites. Implantable cardioverter defibrillators were implanted for primary prevention in 23 (62.1%) patients and secondary prevention in 14 patients (37.8%). Annual rates of appropriate shocks were 0.5% and 6.0% in primary and secondary prevention, respectively (P=0.0366). Independent predictors were a secondary prevention indication (hazard ratio, 18.0; P=0.0341) and lack of beta-blockers (hazard ratio, 16.7; P=0.0301). In patients with appropriate shocks, intracardiac electrograms documented supraventricular tachycardia preceding or coexisting with ventricular tachycardia in 50%. No patient with inducible ventricular tachycardia received an appropriate shock in comparison with 37.5% of noninducible patients (P=0.0429). Inappropriate shocks occurred in 6.6% per year, more so in patients of lesser weight (hazard ratio, 0.91 per kg; P=0.0168). Additionally, 14 patients (37.8%) experienced complications: 5 (13.5%) acute, 1 (2.7%) late generator related, and 12 (32.4%) late lead related. CONCLUSIONS: In patients with transposition of the great arteries, high rates of appropriate shocks are noted in secondary but not primary prevention. Supraventricular arrhythmias may be implicated in the etiology of ventricular tachyarrhythmias; beta-blockers seem protective, and inducible ventricular tachycardia does not seem to predict future events. Inappropriate shocks and late lead-related complications are common.
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“Independent predictors for appropriate ICD shocks in multicentre cohort study of patients with transposition of the great arteries with intra-atrial baffle repair were, among others, lack of beta-blockers (hazard ratio, 16.7; P ¼ 0.0301).367”
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 [367]
In the citing paper
“Documented SVT preceded or coexisted with VT in 50% of patients with appropriate shocks.”
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 [367]
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“This is in complete agreement with prior reports, which have led to the current recommendations to omit such studies in D-TGA patients.(11,15) Given the risk associated with the occurrence of atrial arrhythmias; however, the importance of the EP studies lies in the detection and aggressive treatment of these arrhythmi…”
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“To date only one retrospective multicenter trial has focused on ICD therapy in adult Mustard patients [14], whereas the remaining literature is limited to case reports and small case series [15, 16].”
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“030).(56) All AADs have an increased risk for proarrhythmia and many also aggravate sinus node dysfunction as well as heart failure and require in-hospital observation.”
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Brouwer, C., Hazekamp, M., & Zeppenfeld, K. (2016) · Arrhythmia & Electrophysiology Review · doi:10.15420/aer.2016.19.2 · Cited by 15
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“IART typically has a slower atrial rate (150– 250 bpm) than typical CTI flutter, which may favor 1:1 conduction that can lead to hemodynamic instability and cardiac arrest in patients with ACHD with heart failure.(37) It is most often seen in older Fontan patients with atriopulmonary connections (up to 50%–60%) or lat…”
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“In a retrospective study on patients with TGA after atrial switch who had received an implantable cardioverter defibrillator (ICD), 50% of patients had SVT prior to VT (12).”
BackgroundAtrial Arrhythmia Ablation in Patients With D-Transposition of the Great Arteries and Atrial Switch
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“One possible benefit of beta-blocker therapy in the atrial-switch cohort specifically, is the reduction of atrial tachyarrhythmias which have been associated with precipitating ventricular tachycardia and sudden cardiac death [60].”
BackgroundPathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II
Zubrzycki, M., Schramm, R., Costard‐Jäckle, A., Morshuis, M., Gummert, J. F., & Zubrzycka, M. (2024) · Journal of Clinical Medicine · doi:10.3390/jcm13164823 · Cited by 12
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“The possible mechanisms for the development of AIC in this group of patients are similar to those in other patients (such as high heart rates, ventricular dyssynchrony) (64-66).”
ResultDiagnostic Tools for Arrhythmia Detection in Adults with Congenital Heart Disease and Heart Failure
Mondesert, B. A., Dubin, A. M., & Khairy, P. (2013) · Heart Failure Clinics · doi:10.1016/j.hfc.2013.09.009 · Cited by 10
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“Two notable findings in this study were the presence of SVT immediately prior to VT in 50% of patients and the protective effect on the survival of beta-blockade medication.(18)”
BackgroundManagement of tachyarrhythmias in pregnancy – A review
Kugamoorthy, P., & SPEARS, D. A. (2020) · Obstetric Medicine · doi:10.1177/1753495x20913448 · Cited by 9
“Beta blockade is typically the first choice for arrhythmia management, with the advantage of reducing ventricular arrhythmias in high risk women.(117,118) Nondihydropyridine calcium channel blockers may also be effective at controlling ventricular rate.”
BackgroundTachyarrhythmias in congenital heart disease
Pascual, J. F., Mallofré, N., Santos‐Ortega, A., & Rivas‐Gándara, N. (2024) · Frontiers in Cardiovascular Medicine · doi:10.3389/fcvm.2024.1395210 · Cited by 9
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BackgroundManaging Heart Failure in Adults with Congenital Heart Disease
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“reported data from tetralogy of Fallot and transposition of the great arteries patient populations, demonstrating detection rates in patients receiving an appropriate shock of 213 bpm and 222 bpm, respectively.(34,81) Additionally, in a population of ICD patients with Brugada syndrome, the mean detected tachycardia ra…”
ResultNartowicz, S., Jakielska, E., Ciepłucha, A., Ratajczak, P., Grajek, S., Lesiak, M., & Trojnarska, O. (2023) · Kardiologia Polska · doi:10.33963/kp.a2022.0209 · Cited by 6
“The remaining 2 studies were multi-center international studies with 2 to 3 involved countries and 7 clinical sites each [8, 20].”
BackgroundAn unmet need: arrhythmia detection by implantable loop recorder in the systemic right ventricle
Kakarla, J., Crossland, D. S., Murray, S., Adhvaryu, K., Jansen, K., Rybicka, J., Hermuzi, A., Martin, R. I. R., Shepherd, E., Seller, N., et al. (2023) · EP Europace · doi:10.1093/europace/euad304 · Cited by 6
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“On the basis of current evidence, the primary disadvantages to ICD placement in this group appear to be a high frequency of inappropriate shocks, difficulty in device implantation, complications that include (but are not limited to) lead fracture and failure, and uncertain efficacy in preventing SCD.”
BackgroundMethodResultInfluentialMicrovolt T-wave alternans in adults with complex congenital heart diseases
Trojnarska, O., Ciepłucha, A., Bartczak, A., Kramer, L., & Grajek, S. (2013) · Cardiology Journal · doi:10.5603/cj.a2013.0124 · Cited by 6
“Sub-pulmonary ventricle pressure overload along with cyanosis-related tissue hypoxia, as well as segmental dilation and tortuosity of the coronary arteries typical for increased blood viscosity lead to the myocardial fibrosis creating a substrate for ventricular arrhythmia [28, 29].”
BackgroundPreventing Sudden Death in the Adult with Congenital Heart Disease
Tanel, R. E. (2011) · Current Cardiology Reports · doi:10.1007/s11886-011-0189-y · Cited by 6
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Surgical Therapy of Cardiac Arrhythmias
Mavroudis, C. D., Deal, B. J., & Backer, C. L. (2012) · Wiley eBooks · doi:10.1002/9781118320754.ch41 · Cited by 6
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“Latemortality appears tobedue to two main problems: (i) sudden cardiac death; and (ii) heart failure-related mortality.(5,6) Both remain problematic but create challenges at different levels: while the first is in principle addressable by implantable cardioverter-defibrillator (ICD) therapy, patient selection and the…”
BackgroundCardiac Arrhythmias and Pregnancy
SPEARS, D. A., & Elkayam, U. (2019) · Wiley eBooks · doi:10.1002/9781119409861.ch15 · Cited by 6
Real-World Use and Outcomes of Apixaban for Early Post-Surgical Fontan Thromboprophylaxis
Adkins, K., Williams, R. J., Esteso, P., Kobayashi, R., Gauvreau, K., VanderPluym, C. J., & Hellinger, A. (2024) · Pediatric Cardiology · doi:10.1007/s00246-024-03641-3 · Cited by 5
Tachyarrhythmias in Congenital Heart Diseases: From Ion Channels to Catheter Ablation
Waldmann, V., Guichard, J., Marijon, É., & Khairy, P. (2022) · Journal of Cardiovascular Development and Disease · doi:10.3390/jcdd9020039 · Cited by 7
“Sudden death has been reported as a result of rapidly conducting atrial tachyarrhythmias in patients with systemic right ventricles and univentricular hearts [40].”
BackgroundNozica, N., Asatryan, B., Aur, S., Bouchardy, J., Schwerzmann, M., Guan, F., Pascale, P., Gass, M., Duru, F., Reichlin, T., et al. (2023) · Journal of the American Heart Association · doi:10.1161/jaha.122.028956 · Cited by 4
Horduna, I., & Ernst, S. (2012) · Progress in Pediatric Cardiology · doi:10.1016/j.ppedcard.2012.08.003 · Cited by 4
Cardiac resynchronization and implantable defibrillators in adults with congenital heart disease
Chubb, H., & Motonaga, K. S. (2019) · Heart Failure Reviews · doi:10.1007/s10741-019-09886-y · Cited by 4
Brouillard, A. M., Al-Hammadi, N. J., Hunt, C. J.-G., Barger, P. M., Ludbrook, P. A., & Gleva, M. J. (2020) · International Journal of Cardiology · doi:10.1016/j.ijcard.2020.03.007 · Cited by 4
Ventricular Tachycardia Ablation in Adult Congenital Heart Disease
Wallet, J., Kimura, Y., & Zeppenfeld, K. (2022) · Cardiac Electrophysiology Clinics · doi:10.1016/j.ccep.2022.06.008 · Cited by 4
Ventricular arrhythmia in congenital heart diseases with a systemic right ventricle
Ladouceur, M., Waldmann, V., Bartoletti, S., Chaix, M., & Khairy, P. (2023) · International Journal of Cardiology Congenital Heart Disease · doi:10.1016/j.ijcchd.2023.100463 · Cited by 4
Arrhythmias in Adult Congenital Heart Disease Heart Failure.
Dodeja, A. K., & Upadhyay, S. (2024) · Heart Failure Clinics · doi:10.1016/j.hfc.2023.12.004 · Cited by 3
Thomas, V. C., Peterson, M., McDaniel, M. B., Restrepo, H., Rothman, A., & Jain, A. (2017) · Pediatric Cardiology · doi:10.1007/s00246-017-1635-4 · Cited by 4
Goldstein, S. A., Ward, C., & Al‐Khatib, S. M. (2017) · Journal of Innovations in Cardiac Rhythm Management · doi:10.19102/icrm.2018.090103 · Cited by 4
Lluri, G., & Aboulhosn, J. (2021) · Current Opinion in Cardiology · doi:10.1097/hco.0000000000000933 · Cited by 4
“Both bradyarrhythmias and tachyarrhythmias are commonly seen with sudden cardiac death (SCD) being a devastating clinical scenario.”
BackgroundInfluentialHerzrhythmusstörungen bei Erwachsenen mit angeborenem Herzfehler
Hessling, G. (2016) · Herzschrittmachertherapie & Elektrophysiologie · doi:10.1007/s00399-016-0430-x · Cited by 3
Ramos, D. B. B., Ortega, M. C., Hevia, J. C., Sánchez, M. D., Fernández, A. A. A., Chirino, O. C., Cardentey, M. C., López, F. M., & Rodríguez, R. F. (2017) · Pediatric Cardiology · doi:10.1007/s00246-017-1711-9 · Cited by 4
Hohmann, S., Duncker, D., König, T., Horke, A., Westhoff‐Bleck, M., & Veltmann, C. (2018) · Journal of Thoracic Disease · doi:10.21037/jtd.2018.01.159 · Cited by 4
Albertini, M., Santens, B., Fusco, F., Sarubbi, B., Gallego, P., Puras, M. J. R., Prokšelj, K., Kauling, R. M., Roos‐Hesselink, J. W., Labombarda, F., et al. (2024) · Journal of the American Heart Association · doi:10.1161/jaha.123.032174 · Cited by 3
“Compared with the Woudstra et al cohort, the European cohort was younger at baseline evaluation (median age, 24 years [IQR, 18–30] versus 28 [IQR, 24–36]; P <0.01), patients were more frequently operated on before 1 year of age (72% versus 47%, P <0.01), and sRV function was more commonly impaired…”
MethodInfluentialImplantable Cardioverter-defibrillators in Adult Congenital Heart Disease
Stewart, M. H., Macicek, S. L., & Morin, D. P. (2018) · Journal of Innovations in Cardiac Rhythm Management · doi:10.19102/icrm.2018.090601 · Cited by 3
Marzullo, R., Ladouceur, M., Gaio, G., Giordano, M., Russo, M. G., & Sarubbi, B. (2022) · International Journal of Cardiology · doi:10.1016/j.ijcard.2022.07.021 · Cited by 3
Updates in the management of congenital heart disease in adult patients
Massarella, D., & Alonso-González, R. (2022) · Expert Review of Cardiovascular Therapy · doi:10.1080/14779072.2022.2125870 · Cited by 3
“patients with d-TGA and arterial switch [5].”
BackgroundYao, J. V., Serra, X. C., Albadosh, K., Browne, A., Egorova, A. D., Ladouceur, M., Broberg, C. S., Nizam, M. A., Khairy, P., Padfield, G. J., et al. (2025) · CJC Pediatric and Congenital Heart Disease · doi:10.1016/j.cjcpc.2025.04.006 · Cited by 3
Williams, J. L., Stoll, V., Arif, S., Bowater, S. E., Thorne, S., Clift, P., & Hudsmith, L. (2021) · International Journal of Cardiology Congenital Heart Disease · doi:10.1016/j.ijcchd.2021.100123 · Cited by 3
Counting Down Before Blasting Off
Khairy, P., & Mansour, F. (2011) · Heart Rhythm · doi:10.1016/j.hrthm.2011.04.020 · Cited by 2
Ventricular Arrhythmias in Congenital Heart Disease
Zeppenfeld, K., Jongbloed, M. R. M., & Schalij, M. J. (2017) · Elsevier eBooks · doi:10.1016/b978-0-323-44733-1.00102-4 · Cited by 2
Pacemaker and Defibrillation Implantation in Patients with Transposition of the Great Arteries
Grubb, A. F., Shah, G. V., Aziz, P. F., & Krasuski, R. A. (2017) · Journal of Innovations in Cardiac Rhythm Management · doi:10.19102/icrm.2017.080405 · Cited by 2
“The most common problems affecting these patients long term include arrhythmias, heart failure, and sudden cardiac death (SCD).(3,7,8) Because of these complications, a large number of TGA patients eventually require pacemakers or implantable cardioverter-defibrillators (ICDs).”
BackgroundInfluentialArrhythmias in Adult Congenital Heart Disease
Triedman, J. K., & Walsh, E. P. (2017) · Elsevier eBooks · doi:10.1016/b978-0-7020-6929-1.00017-4 · Cited by 2
Miyazaki, A. (2018) · Japanese Society of Pediatric Cardiology and Cardiac Surgery · doi:10.9794/jspccs.34.172 · Cited by 2
Remote navigation for ablation of arrhythmias in patients with congenital heart disease
Horduna, I., & Ernst, S. (2012) · Progress in Pediatric Cardiology · doi:10.1016/j.ppedcard.2012.08.004 · Cited by 2
Heart Failure in Adults With Congenital Heart Disease
Burchill, L. J., Ross, H. J., & Opotowsky, A. R. (2017) · Elsevier eBooks · doi:10.1016/b978-0-12-802393-8.00026-0 · Cited by 2
Arrhythmias in Children and Patients with Congenital Heart Disease
Lopez, M. M., & Kanter, R. J. (2020) · Contemporary cardiology · doi:10.1007/978-3-030-41967-7_29 · Cited by 2
Perreux, Y., Chaix, M., Kamp, A. N., Mongeon, F., Pham, M., Boussel, L., Hénaine, R., Dore, A., Mond閟ert, B., Di‐Filippo, S., et al. (2020) · Congenital Heart Disease · doi:10.32604/chd.2020.013032 · Cited by 2
Taguchi, Y., Matsumoto, K., Ishikawa, T., Ogino, Y., Matsushita, H., & Hosoda, J. (2017) · Journal of Cardiology Cases · doi:10.1016/j.jccase.2016.11.008 · Cited by 2
“This difficulty in risk stratification may be due to unpredictable triggers for ventricular arrhythmias, such as supraventricular tachyarrhythmias [6] and exercise [7], that might be associated with myocardial ischemia, because c-TGA patients have insufficient coronary circulation to the systemic RV [8].”
BackgroundDellborg, M., Eriksson, P., & Mandalenakis, Z. (2021) · International Journal of Cardiology Congenital Heart Disease · doi:10.1016/j.ijcchd.2021.100129 · Cited by 2
Metadata
- Authors
- Khairy, P., Harris, L., Landzberg, M. J., Fernandes, S. M., Barlow, A., Mercier, L.-A., Viswanathan, S., Chetaille, P., Gordon, E., Dore, A., & Cecchin, F.
- Affiliations
- Boston Children's Hospital (US)
- From the Canadian Adult Congenital Heart Network, Montreal, Quebec, Canada (P.K., L.H., A.B., L.A.M., P.C., E.G., A.D.); Leeds General Infirmary, Leeds, United Kingdom (S.V., P.C.); and Children’s Hospital, Boston, Mass (P.K., M.J.L., S.M.F., F.C.).
- Venue
- Circulation Arrhythmia and Electrophysiology
- Published
- 2008-10
- Volume
- 1
- Issue
- 4
- Pages
- 250-257
- ISSN
- 1941-3084 (ISSN-L)
- 1941-3149
- OpenAlex
- W2150135622
- Cited by
- 269
- References
- 37
- Citations by year
- 2026: 9 · 2025: 11 · 2024: 10 · 2023: 14 · 2022: 16 · 2021: 11 · 2020: 15 · 2019: 10 · 2018: 31 · 2017: 35 · 2016: 23 · 2015: 19 · 2014: 14 · 2013: 15 · 2012: 12
- FWCI
- 5.94
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Circulation Arrhythmia and Electrophysiology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Centre International de Recherche sur le Cancer
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Congenital Heart Disease Studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac pacing and defibrillation studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Keywords
- transposition of the great arteries, sudden cardiac death, implantable cardioverter-defibrillator, appropriate shocks, inappropriate shocks, supraventricular tachycardia, ventricular tachycardia, beta-blockers, lead complications, primary and secondary prevention, congenital heart disease, arrhythmia mechanisms
- MeSH terms
- Adult, Canada, Female, Follow-Up Studies, Humans, Male, Prognosis, Retrospective Studies, Tachycardia, Transposition of Great Vessels, United States, Incidence, Death, Sudden, Cardiac, Defibrillators, Implantable
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