PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease
2014; Elsevier BV; Volume: 30; Issue: 10 Linguagem: Inglês
10.1016/j.cjca.2014.09.002
ISSN1916-7075
AutoresPaul Khairy, George F. Van Hare, Seshadri Balaji, Charles I. Berul, Frank Cecchin, Mitchell I. Cohen, Curt J. Daniels, Barbara J. Deal, Joseph A. Dearani, Natasja de Groot, Anne M. Dubin, Louise Harris, Jan Janoušek, Ronald J. Kanter, Peter P. Karpawich, James C. Perry, Stephen P. Seslar, Maully J. Shah, Michael J. Silka, John K. Triedman, Edward P. Walsh, Carole A. Warnes,
Tópico(s)Cardiac Arrhythmias and Treatments
ResumoNearly one third of all major congenital anomalies are heart defects, with an estimated 9 per 1000 live births afflicted by congenital heart disease (CHD) worldwide.1van der Linde D. Konings E.E. Slager M.A. Witsenburg M. Helbing W.A. Takkenberg J.J. Roos-Hesselink J.W. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis.J Am Coll Cardiol. 2011; 58: 2241-2247Abstract Full Text Full Text PDF PubMed Scopus (125) Google Scholar Remarkable advances in care have resulted in impressive gains in survival such that over 90% of children with CHD in developed countries today are expected to survive into adulthood.2Khairy P. Ionescu-Ittu R. Mackie A.S. Abrahamowicz M. Pilote L. Marelli A.J. Changing mortality in congenital heart disease.J Am Coll Cardiol. 2010; 56: 1149-1157Abstract Full Text Full Text PDF PubMed Scopus (85) Google Scholar Consequently, the past decades have witnessed historical shifts in population demographics, as adults now outnumber children with CHD. Population-based estimates indicate that there are currently over 1 million adults with CHD in the United States alone, over 100,000 in Canada, and 1.8 million in Europe.3Marelli A.J. Mackie A.S. Ionescu-Ittu R. Rahme E. Pilote L. Congenital heart disease in the general population: changing prevalence and age distribution.Circulation. 2007; 115: 163-172Crossref PubMed Scopus (401) Google Scholar, 4Moons P. Engelfriet P. Kaemmerer H. Meijboom F.J. Oechslin E. Mulder B.J. Delivery of care for adult patients with congenital heart disease in Europe: results from the Euro Heart Survey.Eur Heart J. 2006; 27: 1324-1330Crossref PubMed Scopus (43) Google Scholar, 5Go A.S. Mozaffarian D. Roger V.L. Benjamin E.J. Berry J.D. Borden W.B. Bravata D.M. Dai S. Ford E.S. Fox C.S. Franco S. Fullerton H.J. Gillespie C. Hailpern S.M. Heit J.A. Howard V.J. Huffman M.D. Kissela B.M. Kittner S.J. Lackland D.T. Lichtman J.H. Lisabeth L.D. Magid D. Marcus G.M. Marelli A. Matchar D.B. McGuire D.K. Mohler E.R. Moy C.S. Mussolino M.E. Nichol G. Paynter N.P. Schreiner P.J. Sorlie P.D. Stein J. Turan T.N. Virani S.S. Wong N.D. Woo D. Turner M.B. Heart disease and stroke statistics—2013 update: a report from the American Heart Association.Circulation. 2013; 127: e6-e245Crossref PubMed Scopus (1072) Google Scholar Rhythm disorders, which span the entire spectrum of brady- and tachyarrhythmias, are among the most prominent complications encountered by adults with CHD.6Walsh E.P. Cecchin F. Arrhythmias in adult patients with congenital heart disease.Circulation. 2007; 115: 534-545Crossref PubMed Scopus (108) Google Scholar Arrhythmias range in symptomatology and significance, from inconsequential and benign to poorly tolerated and potentially fatal. Taken together, arrhythmias are a leading cause of morbidity, impaired quality of life, and mortality in adults with CHD. In light of the unique issues, challenges, and considerations involved in managing arrhythmias in this growing, aging, and heterogeneous patient population,7Khairy P. EP challenges in adult congenital heart disease.Heart Rhythm. 2008; 5: 1464-1472Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar it appears both timely and essential to critically appraise and synthesize optimal treatment strategies. The purpose of this consensus statement is, therefore, to define optimal conditions for the delivery of care regarding arrhythmias in adults with CHD and provide expert and, where possible, evidence-based recommendations on best practice procedures for the evaluation, diagnosis, and management of arrhythmias, including medical treatment, catheter-based interventions, device therapy, and surgical options. The Pediatric and Congenital Electrophysiology Society (PACES), in conjunction with the Heart Rhythm Society (HRS), appointed a 22-member writing committee from the United States, Canada, and Europe with complementary multidisciplinary expertise in pediatric and adult electrophysiology, adult CHD, and CHD surgery. The writing committee included representation from the American College of Cardiology (ACC), American Heart Association (AHA), European Heart Rhythm Association (EHRA), Canadian Heart Rhythm Society (CHRS), and International Society for Adult Congenital Heart Disease (ISACHD). The committee was divided into subgroups to review key aspects in the recognition and management of arrhythmias in adults with CHD. Experts in the topics under consideration were tasked with performing formal literature reviews, weighing the strength of evidence for or against diagnostic and therapeutic interventions, estimating expected health outcomes where relevant, and proposing practical clinical recommendations. Wherever possible, recommendations are evidence-based. However, unlike some practice guidelines, there is not a sizeable body of literature with definitive evidence to support most recommendations in this emerging field of cardiology. In order to maximize the value and credibility of consensus-based recommendations, a high-threshold (i.e., 80% or greater agreement among writing members) was required to constitute a consensus. Supportive evidence is indicated where appropriate, and variations in opinion are nuanced in the text. As a general recommendation, the committee strongly supports expanding the evidence base related to arrhythmias in adults with CHD through participation in research and clinical registries. The consensus statement was organized by arrhythmia-related topics rather than by heart defect. Depending, in part, on the particular issue and available evidence, recommendations range from being broadly applicable to adults with CHD at large to a more focused lesion-specific scope. The detailed index should assist the reader in rapidly locating sections of interest for specific heart defects. In addition, the writing committee retained the nomenclature for complexity of CHD (i.e., simple, moderate, complex/severe) proposed by the ACC/AHA task force on practice guidelines for adults with CHD,8Warnes C.A. Williams R.G. Bashore T.M. Child J.S. Connolly H.M. Dearani J.A. Del Nido P. Fasules J.W. Graham Jr, T.P. Hijazi Z.M. Hunt S.A. King M.E. Landzberg M.J. Miner P.D. Radford M.J. Walsh E.P. Webb G.D. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: executive summary.J Am Coll Cardiol. 2008; 52: 1890-1947Abstract Full Text Full Text PDF Scopus (40) Google Scholar summarized in Table 1.1.Table 1.1Classification of CHD complexity in adultsAdapted from Warnes CA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease. J Am Coll Cardiol. 2008;52:1890-1947.8Warnes C.A. Williams R.G. Bashore T.M. Child J.S. Connolly H.M. Dearani J.A. Del Nido P. Fasules J.W. Graham Jr, T.P. Hijazi Z.M. Hunt S.A. King M.E. Landzberg M.J. Miner P.D. Radford M.J. Walsh E.P. Webb G.D. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: executive summary.J Am Coll Cardiol. 2008; 52: 1890-1947Abstract Full Text Full Text PDF Scopus (40) Google ScholarComplexityType of congenital heart disease in the adult patientsSimpleNative diseaseIsolated congenital aortic valve diseaseIsolated congenital mitral valve disease (except parachute valve, cleft leaflet)Small atrial septal defectIsolated small ventricular septal defect (no associated lesions)Mild pulmonary stenosisSmall patent ductus arteriosusRepaired conditionsPreviously ligated or occluded ductus arteriosusRepaired secundum or sinus venosus atrial septal defect without residuaRepaired ventricular septal defect without residuaModerateAorto-left ventricular fistulasAnomalous pulmonary venous drainage, partial or totalAtrioventricular septal defects, partial or completeCoarctation of the aortaEbstein anomalyInfundibular right ventricular outflow obstruction of significanceOstium primum atrial septal defectPatent ductus arteriosus, not closedPulmonary valve regurgitation, moderate to severePulmonary valve stenosis, moderate to severeSinus of Valsalva fistula/aneurysmSinus venosus atrial septal defectSubvalvular or supravalvular aortic stenosisTetralogy of FallotVentricular septal defect with:Absent valve or valvesAortic regurgitationCoarctation of the aortaMitral diseaseRight ventricular outflow tract obstructionStraddling tricuspid or mitral valveSubaortic stenosisSevere/complexConduits, valved or nonvalvedCyanotic congenital heart disease, all formsDouble-outlet ventricleEisenmenger syndromeFontan procedureMitral atresiaSingle ventricle (also called double inlet or outlet, common, or primitive) Pulmonary atresia, all formsPulmonary vascular obstructive diseaseTransposition of the great arteriesTricuspid atresiaTruncus arteriosus/hemitruncusOther abnormalities of atrioventricular or ventriculoarterial connection not included above (e.g., crisscross heart, isomerism, heterotaxy syndromes, ventricular inversion) Open table in a new tab Recommendations were subject to a previously described standardized classification process (Methodology Manual and Policies from the ACCHF and AHA Task Force on Practice Guidelines June 2010)9Methodology manual and policies from the ACCF/AHA task force on practice guidelines.American College of Cardiology Foundation and American Heart Association. 2010; : 1-88Google Scholar that ranked each item (Classes I, IIa, IIb, III) and its accompanying level of evidence (Levels A, B, C), as summarized in Table 1.2.Table 1.2Classification of recommendations and levels of evidence9Methodology manual and policies from the ACCF/AHA task force on practice guidelines.American College of Cardiology Foundation and American Heart Association. 2010; : 1-88Google ScholarClassification of RecommendationsClass IConditions for which there is evidence and/or general agreement that a given procedure or treatment plan is beneficial, useful, and effectiveClass IIConditions for which there is conflicting evidence and/or divergence of opinion about the usefulness/efficacy of a procedure or treatment Class IIaWeight of evidence/opinion is in favor of usefulness/efficacy Class IIbUsefulness/efficacy is less well established by evidence/opinionClass IIIConditions for which there is conflicting evidence and/or general agreement that a procedure or treatment is not useful/effective and in some cases may be harmfulLevels of EvidenceLevel of evidence AData derived from multiple randomized clinical trials or meta-analysesLevel of evidence BData derived from a single randomized trial or nonrandomized studiesLevel of evidence COnly consensus opinion of experts, case studies, or standard of care Open table in a new tab The PACES/HRS Task Force made every effort to avoid all potential conflicts of interest relevant to this consensus statement, whether actual or perceived, among members of the writing committee. Members of the writing committee (Appendix 1) and peer reviewers (Appendix 2) were required to disclose all actual or potential direct or indirect conflicts. Committee members were obliged to refrain from voting on issues related to the potential conflict. The document was reviewed by the PACES executive committee, additional members of HRS, and official reviewers nominated by ACC, AHA, EHRA, CHRS, and ISACHD. All writing members approved this final version. The advent of cardiopulmonary bypass and early surgical innovations for CHD of the 1960s and 1970s, coupled with advances in clinical care, have culminated in an increasing and aging cohort with CHD.10Tutarel O. Kempny A. Alonso-Gonzalez R. Jabbour R. Li W. Uebing A. Dimopoulos K. Swan L. Gatzoulis M.A. Diller G.P. Congenital heart disease beyond the age of 60: emergence of a new population with high resource utilization, high morbidity, and high mortality.Eur Heart J. 2014; 35: 725-732Crossref PubMed Scopus (4) Google Scholar Survival beyond the first year of life has risen from an estimated 25% 50 years ago to >90% expected survival into adulthood.11Wren C. O'Sullivan J.J. Survival with congenital heart disease and need for follow up in adult life.Heart. 2001; 85: 438-443Crossref PubMed Google Scholar, 12Warnes C.A. The adult with congenital heart disease: born to be bad?.J Am Coll Cardiol. 2005; 46: 1-8Abstract Full Text Full Text PDF PubMed Scopus (178) Google Scholar In a population-based cohort study of patients with CHD, an overall mortality reduction of 31% was observed from 1987 to 2005, largely driven by improved survival in infants.2Khairy P. Ionescu-Ittu R. Mackie A.S. Abrahamowicz M. Pilote L. Marelli A.J. Changing mortality in congenital heart disease.J Am Coll Cardiol. 2010; 56: 1149-1157Abstract Full Text Full Text PDF PubMed Scopus (85) Google Scholar Most notably, the median age of death in patients with severe forms of CHD increased from 2 to 23 years of age. The older adult with CHD can also anticipate a considerably longer life expectancy, with one study reporting a median age at death of 57 years in 2007 compared to 37 years in 2002.13van der Bom T. Zomer A.C. Zwinderman A.H. Meijboom F.J. Bouma B.J. Mulder B.J. The changing epidemiology of congenital heart disease.Nat Rev Cardiol. 2011; 8: 50-60Crossref PubMed Scopus (56) Google Scholar Although causes of death appear to have remained more or less consistent over the past two decades, recent years have seen a shift in the profile of the patient at risk. While lesion severity and surgical results are major determinants of outcome in infants and children, heart failure, arrhythmias, and pulmonary hypertension become increasingly important in adulthood. Additional prognostic factors in older patients include systemic ventricular dysfunction, chronic renal disease, coronary artery disease, malignancies, and conventional risk factors such as diabetes, hypertension, and obesity.10Tutarel O. Kempny A. Alonso-Gonzalez R. Jabbour R. Li W. Uebing A. Dimopoulos K. Swan L. Gatzoulis M.A. Diller G.P. Congenital heart disease beyond the age of 60: emergence of a new population with high resource utilization, high morbidity, and high mortality.Eur Heart J. 2014; 35: 725-732Crossref PubMed Scopus (4) Google Scholar, 14Moons P. Van Deyk K. Dedroog D. Troost E. Budts W. Prevalence of cardiovascular risk factors in adults with congenital heart disease.Eur J Cardiovasc Prev Rehabil. 2006; 13: 612-616Crossref PubMed Scopus (61) Google Scholar, 15Afilalo J. Therrien J. Pilote L. Martucci G. Ionescu-Ittu R. Marelli A.J. Geriatric congenital heart disease: trends in prevalence and predictors of mortality.Circulation. 2009; 120: S562Google Scholar Arrhythmias increase in prevalence as adults with CHD age and are the most frequent reason for hospital admission.16Kaemmerer H. Fratz S. Bauer U. Oechslin E. Brodherr-Heberlein S. Zrenner B. Turina J. Jenni R. Lange P.E. Hess J. Emergency hospital admissions and three-year survival of adults with and without cardiovascular surgery for congenital cardiac disease.J Thorac Cardiovasc Surg. 2003; 126: 1048Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar, 17Kaemmerer H. Bauer U. Pensl U. Oechslin E. Gravenhorst V. Franke A. Hager A. Balling G. Hauser M. Eicken A. Hess J. Management of emergencies in adults with congenital cardiac disease.Am J Cardiol. 2008; 101: 521-525Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar Along with heart failure, arrhythmias are the leading cause of death.18Silka M.J. Hardy B.G. Menashe V.D. Morris C.D. A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects.J Am Coll Cardiol. 1998; 32: 245-251Abstract Full Text Full Text PDF PubMed Scopus (247) Google Scholar, 19Oechslin E.N. Harrison D.A. Connelly M.S. Webb G.D. Siu S.C. Mode of death in adults with congenital heart disease.Am J Cardiol. 2000; 86: 1111-1116Abstract Full Text Full Text PDF PubMed Scopus (208) Google Scholar, 20Nieminen H.P. Jokinen E.V. Sairanen H.I. Causes of late deaths after pediatric cardiac surgery: a population-based study.J Am Coll Cardiol. 2007; 50: 1263-1271Abstract Full Text Full Text PDF PubMed Scopus (66) Google Scholar, 21Verheugt C.L. Uiterwaal C.S. van der Velde E.T. Meijboom F.J. Pieper P.G. van Dijk A.P. Vliegen H.W. Grobbee D.E. Mulder B.J. Mortality in adult congenital heart disease.Eur Heart J. 2010; 31: 1220-1229Crossref PubMed Scopus (83) Google Scholar Factors associated with pre- and postoperative arrhythmias in CHD are schematically depicted in Figure 3.1.22Escudero C. Khairy P. Sanatani S. Electrophysiologic considerations in congenital heart disease and their relationship to heart failure.Can J Cardiol. 2013; 29: 821-829Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Arrhythmias may reflect congenitally displaced or malformed sinus nodes or atrioventricular (AV) conduction systems, abnormal hemodynamics, primary myocardial disease, hypoxic tissue injury, residual or postoperative sequelae, and genetic influences.23Kanter R.J. Garson Jr, A. Atrial arrhythmias during chronic follow-up of surgery for complex congenital heart disease.Pacing Clin Electrophysiol. 1997; 20: 502-511Crossref PubMed Scopus (56) Google Scholar, 24Khairy P. Dore A. Talajic M. Dubuc M. Poirier N. Roy D. Mercier L.A. Arrhythmias in adult congenital heart disease.Expert Rev Cardiovasc Ther. 2006; 4: 83-95Crossref PubMed Scopus (31) Google Scholar, 25Khairy P. Balaji S. Cardiac arrhythmias in congenital heart diseases.Indian Pacing Electrophysiol J. 2009; 9: 299-317PubMed Google Scholar The entire spectrum of arrhythmias may be encountered in adults with CHD, with several subtypes often coexisting. Bradyarrhythmias may involve disorders of the sinus node, AV node, His–Purkinje system, or intra-atrial propagation. It has been estimated that approximately 50% of 20-year-olds with CHD will develop an atrial tachyarrhythmia during their lifetime.26Bouchardy J. Therrien J. Pilote L. Ionescu-Ittu R. Martucci G. Bottega N. Marelli A.J. Atrial arrhythmias in adults with congenital heart disease.Circulation. 2009; 120: 1679-1686Crossref PubMed Scopus (54) Google Scholar Table 3.1 summarizes atrial tachyarrhythmias typically encountered in common forms of CHD.27Khairy P. Mapping and imaging of supraventricular arrhythmias in adult complex congenital heart disesae.in: Shenasa M. Hindricks G. Borggrefe M. Breithardt G. Josephson M.E. Cardiac Mapping. Fourth Edition. Wiley-Blackwell, Oxford, UK2013: 771-787Google Scholar Atrial tachyarrhythmias may be mediated by accessory pathways, AV node reentry, twin AV nodes,28Epstein M.R. Saul J.P. Weindling S.N. Triedman J.K. Walsh E.P. Atrioventricular reciprocating tachycardia involving twin atrioventricular nodes in patients with complex congenital heart disease.J Cardiovasc Electrophysiol. 2001; 12: 671-679Crossref PubMed Google Scholar, 29Khairy P. Fournier A. Dubuc M. Monckeberg's sling.Can J Cardiol. 2003; 19: 717-718PubMed Google Scholar macroreentrant circuits, automatic foci, or nonautomatic foci.30Seslar S.P. Alexander M.E. Berul C.I. Cecchin F. Walsh E.P. Triedman J.K. Ablation of nonautomatic focal atrial tachycardia in children and adults with congenital heart disease.J Cardiovasc Electrophysiol. 2006; 17: 359-365Crossref PubMed Scopus (14) Google Scholar Intra-atrial reentry is the most common tachyarrhythmia in adults with CHD,31Khairy P. Aboulhosn J. Gurvitz M.Z. Opotowsky A.R. Mongeon F.P. Kay J. Valente A.M. Earing M.G. Lui G. Gersony D.R. Cook S. Ting J.G. Nickolaus M.J. Webb G. Landzberg M.J. Broberg C.S. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.Circulation. 2010; 122: 868-875Crossref PubMed Scopus (72) Google Scholar, 32Khairy P. Landzberg M.J. Lambert J. O'Donnell C.P. Long-term outcomes after the atrial switch for surgical correction of transposition: a meta-analysis comparing the Mustard and Senning procedures.Cardiol Young. 2004; 14: 284-292Crossref PubMed Scopus (54) Google Scholar, 33Khairy P. Fernandes S.M. Mayer Jr, J.E. Triedman J.K. Walsh E.P. Lock J.E. Landzberg M.J. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery.Circulation. 2008; 117: 85-92Crossref PubMed Scopus (214) Google Scholar although the prevalence of atrial fibrillation is on the rise as the population ages.31Khairy P. Aboulhosn J. Gurvitz M.Z. Opotowsky A.R. Mongeon F.P. Kay J. Valente A.M. Earing M.G. Lui G. Gersony D.R. Cook S. Ting J.G. Nickolaus M.J. Webb G. Landzberg M.J. Broberg C.S. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.Circulation. 2010; 122: 868-875Crossref PubMed Scopus (72) Google Scholar, 34Philip F. Muhammad K.I. Agarwal S. Natale A. Krasuski R.A. Pulmonary vein isolation for the treatment of drug-refractory atrial fibrillation in adults with congenital heart disease.Congenital heart disease. 2012; 7: 392-399Crossref PubMed Scopus (5) Google Scholar Ventricular arrhythmias are thought to be the leading cause of sudden death in several subtypes of CHD, with an overall risk that is up to 100-fold higher than in age-matched controls.18Silka M.J. Hardy B.G. Menashe V.D. Morris C.D. A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects.J Am Coll Cardiol. 1998; 32: 245-251Abstract Full Text Full Text PDF PubMed Scopus (247) Google Scholar, 19Oechslin E.N. Harrison D.A. Connelly M.S. Webb G.D. Siu S.C. Mode of death in adults with congenital heart disease.Am J Cardiol. 2000; 86: 1111-1116Abstract Full Text Full Text PDF PubMed Scopus (208) Google Scholar Fortunately, the absolute incidence of these devastating events remains relatively low, at approximately 0.1% per year.Table 3.1Summary of atrial tachyarrhythmias encountered in common forms of CHDCongenital heart disease typeTachyarrhythmiaAtrial septal defectIART/AF with increasing age, particularly if late closureAtrioventricular septal defectIART/AF following surgical repairEbstein anomalyIART; AV or atriofascicular (Mahaim) AP; sudden death if high risk or multiple APs; ectopic atrial tachycardia; AFLeft-sided obstructive lesionsIART/AFTGA with intraatrial baffleIART, NAFAT, AVNRT; VT/VF may be secondary to atrial arrhythmiasCongenitally corrected TGAAccessory pathway if Ebstein-like systemic AV valveTetralogy of FallotIART; NAFAT along the anterolateral right atriumHeterotaxy syndromeTwin AV node-mediated reentrant tachycardiaSingle ventricle with FontanIART; NAFAT; AF; may be poorly toleratedEisenmenger physiologyMAT; IART; AFAF = atrial fibrillation; AP = accessory pathway; AV = atrioventricular; AVNRT = AV nodal reentrant tachycardia; IART = intraatrial reentrant tachycardia; MAT = multifocal atrial tachycardia; NAFAT = nonautomatic focal atrial tachycardia; TGA = transposition of the great arteries; VF = ventricular fibrillation; VT = ventricular tachycardia. Adapted with permission from Khairy P. In: Shenasa M et al (eds). Cardiac Mapping. Fourth Edition. Oxford, UK: Wiley-Blackwell; 2013:771-777.27Khairy P. Mapping and imaging of supraventricular arrhythmias in adult complex congenital heart disesae.in: Shenasa M. Hindricks G. Borggrefe M. Breithardt G. Josephson M.E. Cardiac Mapping. Fourth Edition. Wiley-Blackwell, Oxford, UK2013: 771-787Google Scholar Open table in a new tab AF = atrial fibrillation; AP = accessory pathway; AV = atrioventricular; AVNRT = AV nodal reentrant tachycardia; IART = intraatrial reentrant tachycardia; MAT = multifocal atrial tachycardia; NAFAT = nonautomatic focal atrial tachycardia; TGA = transposition of the great arteries; VF = ventricular fibrillation; VT = ventricular tachycardia. Adapted with permission from Khairy P. In: Shenasa M et al (eds). Cardiac Mapping. Fourth Edition. Oxford, UK: Wiley-Blackwell; 2013:771-777.27Khairy P. Mapping and imaging of supraventricular arrhythmias in adult complex congenital heart disesae.in: Shenasa M. Hindricks G. Borggrefe M. Breithardt G. Josephson M.E. Cardiac Mapping. Fourth Edition. Wiley-Blackwell, Oxford, UK2013: 771-787Google Scholar A tabular representation of approximate expected risks for atrial arrhythmia, ventricular arrhythmia, AV block, and ventricular dyssynchrony are summarized in Figure 3.2. The prevalence and mechanism of arrhythmias vary according to factors such as age, underlying anatomic defect, and method of surgical repair.31Khairy P. Aboulhosn J. Gurvitz M.Z. Opotowsky A.R. Mongeon F.P. Kay J. Valente A.M. Earing M.G. Lui G. Gersony D.R. Cook S. Ting J.G. Nickolaus M.J. Webb G. Landzberg M.J. Broberg C.S. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.Circulation. 2010; 122: 868-875Crossref PubMed Scopus (72) Google Scholar For example, while 3%-5% of patients with congenitally corrected transposition will be born with complete AV block, it is estimated that an additional 20% will develop complete heart block by adulthood.35Beauchesne L.M. Warnes C.A. Connolly H.M. Ammash N.M. Tajik A.J. Danielson G.K. Outcome of the unoperated adult who presents with congenitally corrected transposition of the great arteries.J Am Coll Cardiol. 2002; 40: 285-290Abstract Full Text Full Text PDF PubMed Scopus (67) Google Scholar, 36Graham Jr, T.P. Bernard Y.D. Mellen B.G. Celermajer D. Baumgartner H. Cetta F. Connolly H.M. Davidson W.R. Dellborg M. Foster E. Gersony W.M. Gessner I.H. Hurwitz R.A. Kaemmerer H. Kugler J.D. Murphy D.J. Noonan J.A. Morris C. Perloff J.K. Sanders S.P. Sutherland J.L. Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study.J Am Coll Cardiol. 2000; 36: 255-261Abstract Full Text Full Text PDF PubMed Scopus (223) Google Scholar For others, prior surgery in the region of the sinus node or its arterial supply (e.g., Mustard, Senning, Glenn, or Fontan) will leave them predisposed to later sinus node dysfunction.32Khairy P. Landzberg M.J. Lambert J. O'Donnell C.P. Long-term outcomes after the atrial switch for surgical correction of transposition: a meta-analysis comparing the Mustard and Senning procedures.Cardiol Young. 2004; 14: 284-292Crossref PubMed Scopus (54) Google Scholar, 37Puley G. Siu S. Connelly M. Harrison D. Webb G. Williams W.G. Harris L. Arrhythmia and survival in patients >18 years of age after the Mustard procedure for complete transposition of the great arteries.Am J Cardiol. 1999; 83: 1080-1084Abstract Full Text Full Text PDF PubMed Scopus (134) Google Scholar, 38Cohen M.I. Wernovsky G. Vetter V.L. Wieand T.S. Gaynor J.W. Jacobs M.L. Spray T.L. Rhodes L.A. Sinus node function after a systematically staged Fontan procedure.Circulation. 1998; 98 (discussion II358-9): II352-II358Crossref PubMed Google Scholar The relationship of heart failure to arrhythmogenesis and sudden cardiac death risk is increasingly appreciated.22Escudero C. Khairy P. Sanatani S. Electrophysiologic considerations in congenital heart disease and their relationship to heart failure.Can J Cardiol. 2013; 29: 821-829Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Hemodynamic and electrophysiologic conditions that lead to heart failure, clinical arrhythmias, and adverse outcomes in adults with CHD often extend over several decades. These include long-standing effects of prior atrial or ventricular volume loading, scarring, patches, baffles and surgical barriers, electromechanical dyssynchrony, ongoing deleterious effects on cell–cell electrical coupling, and underlying genetic aspects. Inevitably, the incidence of arrhythmias in the adult CHD population far exceeds that seen in younger patients. Unique forms of heart failure can also be encountered, including dysfunction of a systemic right ventricle or univentricular heart. Systemic left ventricular failure is often associated with congenital left-sided cardiac lesions. Left ventricular dysfunction in tetralogy of Fallot and Ebstein malformation of the tricuspid valve is more widely appreciated as a sequela associated with heightened risk for sudden cardiac death.31Khairy P. Aboulhosn J. Gurvitz M.Z. Opotowsky A.R. Mongeon F.P. Kay J. Valente A.M. Earing M.G. Lui G. Gersony D.R. Cook S. Ting J.G. Nickolaus M.J. Webb G. Landzberg M.J. Broberg C.S. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.Circulation. 2010; 122: 868-875Crossref PubMed Scopus (72) Google Scholar, 39Tzemos N. Harris L. Carasso S. Subira L.D. Greutmann M. Provost Y. Redington A.N. Rakowski H. Siu S.C. Silversides C.K. Adverse left ventricular mechanics in adults with repaired tetralogy of Fallot.Am J Cardiol. 2009; 103: 420-425Abstract Full Text Full Text PDF PubMed Scopus (44) Google Scholar, 40Khairy P. Harris L. Landzberg M.J. Viswanathan S. Barlow A. Gatzoulis M.A. Fernandes S.M. Beauchesne L. Therrien J. Chetaille P. Gordon E. Vonder Muhll I. Cecchin F. Implantable cardioverter-defibrillators in tetralogy of Fallot.Circulation. 2008; 117: 363-370Crossref PubMed Scopus (129) Google Scholar, 41Vermeer A.M. van Engelen K. Postma A.V. Baars M.J. Christiaans I. De Haij S. Klaassen S. Mulder B.J. Keavney B. Ebstein anomaly associated with left ventricular noncompaction: an autosomal dominant condition that can be caused by mutations in MYH7.Am J Med Genet C Semin Med Genet. 2013; 163C: 178-184Crossref PubMed Scopus (3) Google Scholar Right–left ventricular interactions are increasingly acknowledged, and subpulmonary right ventricular failure itself contributes to the complex interplay of factors associated with sudden death.42Davlouros P.A. Kilner P.J. Hornung T.S. Li W. Francis J.M. Moon J.C. Smith G.C. Tat T. Pennell D.J. Gatzoulis M.A. Right ventricular function in adults with repaired tetr
Referência(s)