Atrial Tachyarrhythmia in Congenital Heart Disease

HomeCirculation: Arrhythmia and ElectrophysiologyVol. 10, No. 9Atrial Tachyarrhythmia in Congenital Heart Disease Free AccessEditorialPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessEditorialPDF/EPUBAtrial Tachyarrhythmia in Congenital Heart DiseaseBeyond the Suture Lines Elisabeth M.J.P. Mouws, MD and Natasja M.S. de Groot, MD, PhD Elisabeth M.J.P. MouwsElisabeth M.J.P. Mouws From the Departments of Cardiothoracic Surgery (E.M.J.P.M.) and Cardiology (E.M.J.P.M., N.M.S.d.G.), Erasmus Medical Center, Rotterdam, The Netherlands. and Natasja M.S. de GrootNatasja M.S. de Groot From the Departments of Cardiothoracic Surgery (E.M.J.P.M.) and Cardiology (E.M.J.P.M., N.M.S.d.G.), Erasmus Medical Center, Rotterdam, The Netherlands. Originally published12 Sep 2017https://doi.org/10.1161/CIRCEP.117.005697Circulation: Arrhythmia and Electrophysiology. 2017;10:e005697See Article by Ávila et alAtrial tachyarrhythmias, including atrial fibrillation (AF) and regular atrial tachycardia (AT), are important complications after cardiac surgery for congenital heart disease (CHD) that lead to increased morbidity and mortality.1 As the adult population of CHD patients is steadily increasing as a result of improved healthcare and surgical techniques, the prevalence of AF and AT among these patients also continues to rise. To date, prevalence rates of atrial tachyarrhythmias in patients with CHD are 3 times higher than in the general population.2 In addition, ventricular tachycardia and ventricular fibrillation, though less prevalent, still contribute to sudden death and reduce long-term survival rates in patients with CHD.3In adult patients with CHD, the majority of atrial tachyarrhythmias are intra-atrial reentrant tachycardia or AF although the incidence differs between the various types of CHD. Patients with a univentricular heart (UVH) who underwent a Fontan procedure undoubtedly have the highest risk for atrial tachyarrhythmia; 50% of patients will develop AT or AF within 10 years increasing to 100% after 26 years.4,5 Other CHD patients at high risk for atrial arrhythmia include those with transposition of the great arteries (TGA) or tetralogy of Fallot. TGA patients who have had an atrial switch procedure have a risk of 30% to develop atrial tachyarrhythmias within 10 years after Mustard or Senning repair, which rises up to 40% within 35years after surgery.6,7 In tetralogy of Fallot patients, up to 20% will develop intra-atrial reentrant tachycardia or atrial flutter (AFL) over time.8,9Several studies have elucidated possible risk factors, which may inform modifications of surgical approaches. In tetralogy of Fallot patients, for instance, the transition from performing a primary ventriculotomy to a transatrial transpulmonary approach to access the right ventricle, with limited transannular patching, has led to a lower incidence of ventricular tachycardia and improved long-term preservation of ventricular function.10 In addition, the implementation of the lateral tunnel instead of the atriopulmonary Fontan for UVH patients has led to a significant decrease in the incidence of atrial tachyarrhythmias.11 One report of the use of an extracardiac tunnel in UVH patients even reported no AT/AF during 10 years of follow-up.11 Similarly, the advent of the arterial switch procedure for TGA patients has decreased incidences of AT/AF to 5%.12However, surgical interference is not always the main cause of arrhythmia development. Both atrial and ventricular tachyarrhythmias may also arise because of long-term hemodynamic consequences of the congenital defect itself.In patients with unoperated atrial septal defect (ASD), prevalence of atrial arrhythmias ranges between 10% and 20% depending on age, and ASD closure is reported to decrease AT/AF prevalence, as patients with preprocedural arrhythmias revert to sinus rhythm.13 Yet, age at ASD closure plays an important role; the risk of postprocedural de novo AT/AF is particularly high in patients aged >40 years with an incidence up to 59% during long-term follow-up.13These previous studies have led us to conclude that both the complexity of the CHD and extent of surgical procedures contribute to a patients' risk of subsequent arrhythmia, which often presents decades after cardiac surgery.14 Although several studies have examined the frequency of AT/AF among CHD subtypes, distinguishing individual patients at risk at an early stage remains a challenge.In this issue of the journal, Ávila et al15 report their observations of AT and AF in a large cohort of 3 311 patients with various forms of CHD. Patients had a median age of 23 years at baseline and a median follow-up period of 11 years.15 Almost one third of the cohort had never undergone cardiac surgery; the remaining patients, however, have undergone (multiple) cardiac surgeries on their defect, for which the nomenclature natural history might be unfortunate. Nonetheless, observed incidences were 4.7% for AT and 4.8% for AF.15 CHD-specific incidences of AT were generally lower than previous reports as may be expected for this relatively young population. Highest incidences of AT were observed in UVH and TGA patients, reaching 23%.15 As expected, AT incidences rise with increasing complexity of the CHD.15Not surprisingly, treatment of AT generally has lower success rates in these patients than would be expected in non-CHD populations. Complexity of the defect often brings anatomic challenges for catheter ablation procedures.16 In TGA patients with an atrial switch procedure, the coronary sinus, the left atrium, and some areas of the right atrium may be difficult to reach by standard methods.16 UVH patients face similar difficulties, as with a lateral tunnel access to the left atrium and the septal portion of the right atrium is hampered.16 Reported acute success rates for catheter ablation in populations with a mix of CHD ranges from 65% to 96%.1 Long-term outcomes of ablative therapy were described in a study of 53 patients with primarily moderate or severe CHD17 who underwent catheter ablation for AFL, intra-atrial reentrant tachycardia, or focal AT. In this population, the AT recurrence rate within the first year after the initial ablation procedure was 55% and 28% of the patients underwent (multiple) redoablation procedures.17Although surgical interference certainly plays an important role in arrhythmogenesis of patients with CHD, Ávila et al15 should be complimented for their attention to patients' natural history, the timing of first cardiac surgery and AT development; patients with ASD, for instance, underwent correction at a relatively old age, exposing them to the sequelae of their defect for ≈40 years, whereas TGA patients were operated in early childhood. Not surprisingly, patients with ASD generally developed AT relatively soon after cardiac surgery as opposed to TGA patients who developed AT >20 years later.15 Whether surgical incisions or chronic exposure to volume and pressure overload is the most important factor in AT development remains debatable. Interestingly though, identified independent clinical predictors were primarily factors associated with long-term abnormal hemodynamic conditions, resulting either from cardiac surgery or from the defect itself, including univentricular physiology, intracardiac repair, systemic right ventricle, severe pulmonary hypertension, pulmonary regurgitation, pulmonary atrioventricular valve regurgitation, pulmonary ventricular dysfunction, and systemic ventricular dysfunction. Additionally, a clinical risk score was developed based on these independent predictors, which showed good discriminative value and may be used for guidance and management considering the future risk of AT development.The predictors identified by Ávila et al are usually easily obtained in clinical practice during follow-up visits. In patients without these predictors, AT risk is minimal, whereas patients with ≥3 risk factors have a substantial risk for AT development reaching 50% at the age of 40 years. It may be interesting to correlate the incidence of these predictors with the complexity of the defect, given the fact that the existing categorization into simple, moderate, and severe CHD also identified risk of AT development according to age.15 Patients with complex CHD reached an AT risk of 55% at the age of 40 years.15However, by identifying clinical predictors, this study contributes to the understanding of underlying mechanisms of AT in individual CHD patients. Often, the surgical repair has been the scapegoat blamed for arrhythmia development in this population,18–20 with little appreciation of the potential contribution of cardiac remodeling due to chronic volume or pressure overload. The results of this study, however, further emphasize the importance of well-balanced hemodynamic status for optimal outcomes in patients with CHD.DisclosuresNone.FootnotesThe opinions expressed in this article are not necessarily those of the editors or of the American Heart Association.Circ Arrhythm Electrophysiol is available at http://circep.ahajournals.org.Correspondence to: Natasja M.S. de Groot, MD, PhD, Unit Translational Electrophysiology, Department of Cardiology, Erasmus Medical Center PO Box 616, 's Gravendijkwal 230, 3015CE Rotterdam, The Netherlands. E-mail [email protected]References1. Khairy P, Van Hare GF, Balaji S, Berul CI, Cecchin F, Cohen MI, Daniels CJ, Deal BJ, Dearani JA, de Groot N, Dubin AM, Harris L, Janousek J, Kanter RJ, Karpawich PP, Perry JC, Seslar SP, Shah MJ, Silka MJ, Triedman JK, Walsh EP, Warnes CA. PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease.Heart Rhythm. 2014; 11:1–64.Google Scholar2. Bouchardy J, Therrien J, Pilote L, Ionescu-Ittu R, Martucci G, Bottega N, Marelli AJ. Atrial arrhythmias in adults with congenital heart disease.Circulation. 2009; 120:1679–1686. doi: 10.1161/CIRCULATIONAHA.109.866319.LinkGoogle Scholar3. Khairy P. Ventricular arrhythmias and sudden cardiac death in adults with congenital heart disease.Heart. 2016; 102:1703–1709. doi: 10.1136/heartjnl-2015-309069.CrossrefMedlineGoogle Scholar4. Quinton E, Nightingale P, Hudsmith L, Thorne S, Marshall H, Clift P, de Bono J. Prevalence of atrial tachyarrhythmia in adults after Fontan operation.Heart. 2015; 101:1672–1677. doi: 10.1136/heartjnl-2015-307514.CrossrefMedlineGoogle Scholar5. Durongpisitkul K, Porter CJ, Cetta F, Offord KP, Slezak JM, Puga FJ, Schaff HV, Danielson GK, Driscoll DJ. Predictors of early- and late-onset supraventricular tachyarrhythmias after Fontan operation.Circulation. 1998; 98:1099–1107.LinkGoogle Scholar6. Flinn CJ, Wolff GS, Dick M, Campbell RM, Borkat G, Casta A, Hordof A, Hougen TJ, Kavey RE, Kugler J. Cardiac rhythm after the Mustard operation for complete transposition of the great arteries.N Engl J Med. 1984; 310:1635–1638. doi: 10.1056/NEJM198406213102504.CrossrefMedlineGoogle Scholar7. Wheeler M, Grigg L, Zentner D. Can we predict sudden cardiac death in long-term survivors of atrial switch surgery for transposition of the great arteries?Congenit Heart Dis. 2014; 9:326–332. doi: 10.1111/chd.12145.CrossrefMedlineGoogle Scholar8. Wu MH, Lu CW, Chen HC, Chiu SN, Kao FY, Huang SK. Arrhythmic burdens in patients with tetralogy of Fallot: a national database study.Heart Rhythm. 2015; 12:604–609. doi: 10.1016/j.hrthm.2014.11.026.CrossrefMedlineGoogle Scholar9. Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J, Valente AM, Earing MG, Lui G, Gersony DR, Cook S, Ting JG, Nickolaus MJ, Webb G, Landzberg MJ, Broberg CS; Alliance for Adult Research in Congenital Cardiology (AARCC). Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.Circulation. 2010; 122:868–875. doi: 10.1161/CIRCULATIONAHA.109.928481.LinkGoogle Scholar10. Dietl CA, Cazzaniga ME, Dubner SJ, Pérez-Baliño NA, Torres AR, Favaloro RG. Life-threatening arrhythmias and RV dysfunction after surgical repair of tetralogy of Fallot. Comparison between transventricular and transatrial approaches.Circulation. 1994; 90(5 pt 2):II7–I12.MedlineGoogle Scholar11. d'Udekem Y, Iyengar AJ, Cochrane AD, Grigg LE, Ramsay JM, Wheaton GR, Penny DJ, Brizard CP. The Fontan procedure: contemporary techniques have improved long-term outcomes.Circulation. 2007; 116(11 Suppl):I157–I164. doi: 10.1161/CIRCULATIONAHA.106.676445.LinkGoogle Scholar12. Khairy P, Clair M, Fernandes SM, Blume ED, Powell AJ, Newburger JW, Landzberg MJ, Mayer JE. Cardiovascular outcomes after the arterial switch operation for D-transposition of the great arteriesclinical perspective.Circulation. 2013; 127.LinkGoogle Scholar13. Chubb H, Whitaker J, Williams SE, Head CE, Chung NA, Wright MJ, O'Neill M. Pathophysiology and management of arrhythmias associated with atrial septal defect and patent foramen ovale.Arrhythm Electrophysiol Rev. 2014; 3:168–172. doi: 10.15420/aer.2014.3.3.168.CrossrefMedlineGoogle Scholar14. Wasmer K, Eckardt L. Management of supraventricular arrhythmias in adults with congenital heart disease.Heart. 2016; 4:heartjnl-2015–309068.Google Scholar15. Ávila P, Oliver JM, Gallego P, González-García A, Rodríguez-Puras MJ, Cambronero E, Ruiz-Cantador J, Campos A, Peinado R, Prieto R, Sarnago F, Yotti R, Fernández-Avilés F. Natural history and clinical predictors of atrial tachycardia in adults with congenital heart disease.Circ Arrhythm Electrophysiol. 2017; 10:e005396. doi: 10.1161/CIRCEP.117.005396.LinkGoogle Scholar16. Chubb H, Williams SE, Wright M, Rosenthal E, O'Neill M. Tachyarrhythmias and catheter ablation in adult congenital heart disease.Expert Rev Cardiovasc Ther. 2014; 12:751–770. doi: 10.1586/14779072.2014.914434.CrossrefMedlineGoogle Scholar17. de Groot NM, Atary JZ, Blom NA, Schalij MJ. Long-term outcome after ablative therapy of postoperative atrial tachyarrhythmia in patients with congenital heart disease and characteristics of atrial tachyarrhythmia recurrences.Circ Arrhythm Electrophysiol. 2010; 3:148–154. doi: 10.1161/CIRCEP.109.909838.LinkGoogle Scholar18. Gandhi SK, Bromberg BI, Rodefeld MD, Schuessler RB, Boineau JP, Cox JL, Huddleston CB. Spontaneous atrial flutter in a chronic canine model of the modified Fontan operation.J Am Coll Cardiol. 1997; 30:1095–1103.CrossrefMedlineGoogle Scholar19. Yang G, Du X, Ni B, Chen H, Qi R, Cai C, Fang Y, Yang B, Ju W, Zhang F, Li M, Gu K, Shao Y, Chen M. Prevention of postsurgical atrial tachycardia with a modified right atrial free wall incision.Heart Rhythm. 2015; 12:1611–1618. doi: 10.1016/j.hrthm.2015.03.026.CrossrefMedlineGoogle Scholar20. Pap R, Kohári M, Makai A, Bencsik G, Traykov VB, Gallardo R, Klausz G, Zsuzsanna K, Forster T, Sághy L. Surgical technique and the mechanism of atrial tachycardia late after open heart surgery.J Interv Card Electrophysiol. 2012; 35:127–135. doi: 10.1007/s10840-012-9705-2.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Tseng W, Huang C, Chiu S, Lu C, Wang J, Lin M, Chen C and Wu M (2021) Long-term outcomes of arrhythmia and distinct electrophysiological features in congenitally corrected transposition of the great arteries in an Asian cohort, American Heart Journal, 10.1016/j.ahj.2020.10.057, 231, (73-81), Online publication date: 1-Jan-2021. Jiang J, Yang Y, Liu C, Ma Y, Wang L, He J, Tang A, Hess P, Kerlan J, Feng C and Lan D (2019) Overdrive pacing mapping: An alternative approach used in scar associated localized atrial tachycardia, Journal of Cardiovascular Electrophysiology, 10.1111/jce.14200, 30:12, (2668-2677), Online publication date: 1-Dec-2019. Ramdjan T, Mouws E, Kik C, Roos-Hesselink J, Bogers A and de Groot N (2018) Concomitant arrhythmia surgery in patients with congenital heart disease, Interactive CardioVascular and Thoracic Surgery, 10.1093/icvts/ivy181, 27:6, (902-909), Online publication date: 1-Dec-2018. September 2017Vol 10, Issue 9 Advertisement Article InformationMetrics © 2017 American Heart Association, Inc.https://doi.org/10.1161/CIRCEP.117.005697PMID: 28899956 Manuscript receivedAugust 15, 2017Manuscript acceptedAugust 24, 2017Originally publishedSeptember 12, 2017 Keywordsheart diseasesthoracic surgeryventricular fibrillationEditorialsatrial fibrillationtachycardiaPDF download Advertisement SubjectsArrhythmiasCongenital Heart DiseaseRisk Factors