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• W2019914926 abstract "Arrhythmias and sudden death continue to plague a subset of adult patients with congenital heart disease. Despite investigative efforts spanning many decades, accurate identification of the high-risk patient remains challenging owing to a limited population size, relatively low event rate, and constantly evolving surgical approaches to the various malformations. Furthermore, until recently, most studies of the subject involved single-center formats with limited statistical power. The number of adult survivors has now reached a critical size where larger collaborative projects are beginning to generate more objective criteria for assessing risk. This review will provide an update on risk stratification for several of the major congenital cardiac lesions and outline the current recommendations for surveillance and management. Arrhythmias and sudden death continue to plague a subset of adult patients with congenital heart disease. Despite investigative efforts spanning many decades, accurate identification of the high-risk patient remains challenging owing to a limited population size, relatively low event rate, and constantly evolving surgical approaches to the various malformations. Furthermore, until recently, most studies of the subject involved single-center formats with limited statistical power. The number of adult survivors has now reached a critical size where larger collaborative projects are beginning to generate more objective criteria for assessing risk. This review will provide an update on risk stratification for several of the major congenital cardiac lesions and outline the current recommendations for surveillance and management. The late 1930s marked the beginning of a remarkable series of surgical advances for congenital heart disease (CHD) that permitted long-term survival for a unique group of patients who would otherwise have died during early childhood.1Gross R.E. Surgical management of the patent ductus arteriosus: with summary of four surgically treated cases.Ann Surg. 1939; 110: 321-356Crossref PubMed Google Scholar Improved longevity would eventually expose a number of unanticipated late complications, central among which were atrial and ventricular arrhythmias contributing to sudden cardiac death (SCD). Wolff et al2Wolff G.S. Rowland T.W. Ellison R.C. Surgically induced right bundle-branch block with left anterior hemiblock: an ominous sign in postoperative tetralogy of Fallot.Circulation. 1972; 46: 587-594Crossref PubMed Scopus (123) Google Scholar were the first to sound the alarm in 1972 when they published observations on disrupted conduction patterns and ventricular tachycardia (VT) associated with SCD in patients who have undergone repair of tetralogy of Fallot. Since then, the topic of late arrhythmias has received the attention of all cardiologists involved with the longitudinal care of this growing population. Arrhythmias in CHD arise from the abnormal myocardial substrate caused by variable pressure/volume loads, cyanosis, and certain anatomic features specific to the individual structural lesion. The situation is further complicated by palliative or corrective surgery, creating myocardial scars that can function as conduction barriers and central obstacles for macroreentrant circuits. Of note, the most malignant arrhythmias in the CHD population typically do not become manifest until the third decade of life or beyond, suggesting that a period of degenerative remodeling also plays a role in their genesis. For this reason, SCD looms as a far greater concern once CHD patients reach adulthood than it did during childhood and adolescence. The number of adults with CHD living in North America is now estimated to exceed 1 million. Included in this group are some cases with relatively minor disease in whom arrhythmia risk is known to be low (eg, repaired atrial septal defect or ligated ductus arteriosus), but the majority can be classified as having moderate or severe malformations3Warnes C.A. Liberthson R. Danielson G.K. Dore A. Harris L. Hoffman J.I. Somerville J. Williams R.G. Webb G.D. Task force 1: the changing profile of congenital heart disease in adult life.J Am Coll Cardiol. 2001; 37: 1170-1175Abstract Full Text Full Text PDF PubMed Scopus (1088) Google Scholar with the potential for life-threatening rhythm disturbances. In several large series examining long-term outcomes after CHD surgery, sudden unexpected events (usually arrhythmic, but occasionally vascular or thrombotic) ultimately accounted for 20% or more of the total mortality in patients with complex lesions.4Oechslin 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 (429) Google Scholar, 5Nieminen 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 (195) Google Scholar, 6Verheugt 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 (423) Google Scholar Three important articles have provided detailed data on the overall SCD risk in large populations with CHD. Silka et al7Silka 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 (453) Google Scholar examined outcomes for patients from the state of Oregon who underwent CHD surgery between 1958 and 1996 and identified sudden arrhythmic death in 30 individuals from a cohort of 3589. When event rate was adjusted according to specific lesion type and follow-up duration, the incidence appeared highest for adult patients with transposition of the great arteries (TGA) and a systemic right ventricle (RV) after atrial baffling operations, followed by those with left ventricular (LV) outflow obstruction (aortic stenosis or coarctation of the aorta), followed by those with tetralogy of Fallot. A different study format was used by Koyak et al8Koyak 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. Sudden cardiac death in adult congenital heart disease.Circulation. 2012; 126: 1944-1954Crossref PubMed Scopus (257) Google Scholar in their multinational case-control study of more than 25,000 adult CHD patients that included surgical follow-up as well as natural history of nonoperable cases. They identified 171 cases of SCD due to arrhythmias within this large group. Denominators were not reported for specific lesion type to allow calculation of incidence, but the conditions with the highest number of SCD events included Eisenmenger syndrome, TGA with a systemic RV, and tetralogy of Fallot. More recently, Gallego et al9Gallego P. Gonzalez A.E. Sanchez-Recalde A. Peinado R. Polo L. Gomez-Rubin C. Lopez-Sendon J.L. Oliver J.M. Incidence and predictors of sudden cardiac arrest in adults with congenital heart defects repaired before adult life.Am J Cardiol. 2012; 110: 109-117Abstract Full Text Full Text PDF PubMed Scopus (81) Google Scholar reported their single center experience of more than 3000 adult patients with CHD, in which patients with TGA and a systemic RV were again the highest risk group, followed by those with a single ventricle. These studies are among the few that have looked at SCD among CHD patients in a global sense, and all 3 arrived at nearly identical conclusions: (1) malignant arrhythmias will occur in 1% of all patients with some form of CHD over a mean follow-up period of 10 years, (2) these events will be largely concentrated in adult aged patients with complicated hemodynamic lesions for whom the SCD risk may reach 10% per decade of follow-up, and (3) abnormal “systemic” ventricular function (whether this involves an anatomic RV or LV) is among the strongest predictors of malignant arrhythmias. Most other studies of the SCD issue in CHD tend to focus on a specific lesion, with an understandable predilection to choose a common malformation with an effective surgical solution and a large number of patients surviving into middle age. Hence, tetralogy of Fallot has been studied more extensively than any other condition, and so the mechanisms for SCD and its risk factors have been worked out reasonably well. Knowledge is less developed for other forms of CHD, such as TGA or single ventricle, either because the malformation is less common or because survival into adulthood is limited. The best available data suggest that it is hazardous to assume that lessons learned from tetralogy of Fallot are applicable to other forms of CHD. Only recently has multicenter attention been directed to SCD in alternate lesions, and risk stratification for such patients appears to differ in some important ways. It is useful, therefore, to evaluate arrhythmia risk in adult CHD on a lesion-by-lesion basis. The mechanism of SCD in tetralogy of Fallot has been under investigation for more than 4 decades. Early on there was concern that atrioventricular block accounted for these events,2Wolff G.S. Rowland T.W. Ellison R.C. Surgically induced right bundle-branch block with left anterior hemiblock: an ominous sign in postoperative tetralogy of Fallot.Circulation. 1972; 46: 587-594Crossref PubMed Scopus (123) Google Scholar but it quickly became evident that VT was the culprit in most cases.10Gillette P.C. Yeoman M.A. Mullins C.E. McNamara D.G. Sudden death after repair of tetralogy of Fallot: electrocardiographic and electrophysiologic abnormalities.Circulation. 1977; 56: 566-571Crossref PubMed Scopus (149) Google Scholar It is now understood that the intrinsic anatomy of the RV in tetralogy involves structural features that can potentially support macroreentry circuits near the outflow tract11Zeppenfeld K. Schalij M.J. Bartelings M.M. Tedrow U.B. Koplan B.A. Soejima K. Stevenson W.G. Catheter ablation of ventricular tachycardia after repair of congenital heart disease: electroanatomic identification of the critical right ventricular isthmus.Circulation. 2007; 116: 2241-2252Crossref PubMed Scopus (273) Google Scholar, 12Kriebel T. Saul J.P. Schneider H. Sigler M. Paul T. Noncontact mapping and radiofrequency catheter ablation of fast and hemodynamically unstable ventricular tachycardia after surgical repair of tetralogy of Fallot.J Am Coll Cardiol. 2007; 50: 2162-2168Abstract Full Text Full Text PDF PubMed Scopus (81) Google Scholar, 13Sherwin E.D. Triedman J.K. Walsh E.P. Update on interventional electrophysiology in patients with congenital heart disease: evolving solutions for complex hearts.Circ Arrhythm Electrophysiol. 2013; 6: 1032-1040Crossref PubMed Scopus (70) Google Scholar and that traditional surgical repair might reinforce this potential (Figure 1). Patients with tetralogy can also develop RV and LV dysfunction from hemodynamic stress, putting them at additional risk of more disorganized polymorphic VT and ventricular fibrillation, similar to arrhythmias seen in any other form of dilated cardiomyopathy. Beyond the VT risk, these patients also carry a heavy burden of atrial macroreentrant tachycardias14Khairy P. Aboulhosn J. Gurvitz M.Z. et al.Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.Circulation. 2010; 122: 868-875Crossref PubMed Scopus (381) Google Scholar, 15Mah D.Y. Alexander M.E. Cecchin F. Walsh E.P. Triedman J.K. The electroanatomic mechanisms of atrial tachycardia in patients with tetralogy of Fallot and double outlet right ventricle.J Cardiovasc Electrophysiol. 2011; 22: 1013-1017Crossref PubMed Scopus (51) Google Scholar involving the cavotricuspid isthmus and/or atriotomy scars (Figure 2). Atrial tachycardia, when conducted rapidly in a patient with tetralogy and depressed ventricular function, can contribute to the SCD risk in some individuals.Figure 2Electroanatomic maps of the right atrium demonstrating atrial macroreentry that developed in an adult with tetralogy of Fallot many years after surgical repair. Surgery included a right lateral atriotomy incision (hatched area). A: Typical counterclockwise macroreentry around the tricuspid valve (TV) through the cavotricuspid isthmus. B: Second macroreentry circuit on the lateral atrial wall that used the atriotomy scar as the central obstacle and traveled through a narrow gap between the lower edge of the scar and the inferior vena cava (IVC).View Large Image Figure ViewerDownload Hi-res image Download (PPT) More than 100 studies have been published examining SCD in repaired tetralogy since the mid-1970s. The largest of these permit estimation of the SCD risk in a given cohort with tetralogy of approximately 2% per decade of follow-up.7Silka 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 (453) Google Scholar, 16Murphy J.G. Gersh B.J. Mair D.D. Fuster V. McGoon M.D. Ilstrup D.M. McGoon D.C. Kirklin J.W. Danielson G.K. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot.N Engl J Med. 1993; 329: 593-599Crossref PubMed Scopus (862) Google Scholar, 17Nollert G. Fischlein T. Bouterwek S. Bohmer C. Klinner W. Reichart B. Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair.J Am Coll Cardiol. 1997; 30: 1374-1383Abstract Full Text Full Text PDF PubMed Scopus (601) Google Scholar, 18Norgaard M.A. Lauridsen P. Helvind M. Pettersson G. Twenty-to-thirty-seven-year follow-up after repair for tetralogy of Fallot.Eur J Cardiothorac Surg. 1999; 16: 125-130Crossref PubMed Scopus (102) Google Scholar, 19Gatzoulis M.A. Balaji S. Webber S.A. Siu S.C. Hokanson J.S. Poile C. Rosenthal M. Nakazawa M. Moller J.H. Gillette P.C. Webb G.D. Redington A.N. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study.Lancet. 2000; 356: 975-981Abstract Full Text Full Text PDF PubMed Scopus (1389) Google Scholar This figure, however, is based on study groups that include both pediatric and adult subjects with variable follow-up duration. If attention is directed exclusively to adult patients 25 years or more after surgical repair, the SCD risk rises to the range of 6%–10% per decade of follow-up.17Nollert G. Fischlein T. Bouterwek S. Bohmer C. Klinner W. Reichart B. Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair.J Am Coll Cardiol. 1997; 30: 1374-1383Abstract Full Text Full Text PDF PubMed Scopus (601) Google Scholar, 19Gatzoulis M.A. Balaji S. Webber S.A. Siu S.C. Hokanson J.S. Poile C. Rosenthal M. Nakazawa M. Moller J.H. Gillette P.C. Webb G.D. Redington A.N. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study.Lancet. 2000; 356: 975-981Abstract Full Text Full Text PDF PubMed Scopus (1389) Google Scholar The search for risk factors predicting VT and SCD in patients with tetralogy has generated an extensive catalog that includes patient age, surgical timing/technique, measures of hemodynamic status, electrocardiographic findings, noninvasive rhythm monitoring, and invasive electrophysiologic evaluation. The sheer length of the list reflects the challenge of working with a limited population size and low event rate, but perhaps the biggest obstacle to a strong predictive model is that the surgical approach to tetralogy has evolved substantially through the years. Patients managed from the 1940s through the 1970s were likely to receive initial palliation with a systemic pulmonary shunt procedure that imposed a volume load on the LV and delayed definitive repair. This was largely supplanted in the 1980s by complete repair during infancy,20Castaneda A.R. Freed M.D. Williams R.G. Norwood W.I. Repair of tetralogy of Fallot in infancy: early and late results.J Thorac Cardiovasc Surg. 1977; 74: 372-381PubMed Google Scholar, 21Walsh E.P. Rockenmacher S. Keane J.F. Hougen T.J. Lock J.E. Castaneda A.R. Late results in patients with tetralogy of Fallot repaired during infancy.Circulation. 1988; 77: 1062-1067Crossref PubMed Scopus (172) Google Scholar but early techniques for infant repair often involved a large transannular patch, resulting in chronic pulmonary regurgitation and eventual RV enlargement. Surgery for the current generation of tetralogy patients still favors early complete repair, but now stronger efforts are made to preserve pulmonary valve competence and minimize the size of right ventriculotomy incisions.22Hamada H. Terai M. Jibiki T. Nakamura T. Gatzoulis M.A. Niwa K. Influence of early repair of tetralogy of Fallot without an outflow patch on late arrhythmias and sudden death: a 27-year follow-up study following a uniform surgical approach.Cardiol Young. 2002; 12: 345-351Crossref PubMed Google Scholar To complicate matters further, late reintervention for transcatheter or surgical pulmonary valve replacement has become commonplace for older patients with tetralogy, and the electrophysiologic consequences of late valve replacement are still uncertain.23Therrien J. Siu S.C. Harris L. Dore A. Niwa K. Janousek J. Williams W.G. Webb G. Gatzoulis M.A. Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot.Circulation. 2001; 103: 2489-2494Crossref PubMed Scopus (387) Google Scholar, 24Harrild D.M. Berul C.I. Cecchin F. Geva T. Gauvreau K. Pigula F. Walsh E.P. Pulmonary valve replacement in tetralogy of Fallot: impact on survival and ventricular tachycardia.Circulation. 2009; 119: 445-451Crossref PubMed Scopus (258) Google Scholar It is reasonable to assume that the incidence and mechanisms of SCD may differ among all these subgroups. Nonetheless, certain risk factors tend to show up with high regularity in recent studies regardless of surgical history, and these have now become widely accepted as the variables of greatest interest for risk stratification in tetralogy (Table 1).Table 1Risk factors after repair of tetralogy of Fallot•Older age at the time of complete repair•Longer duration of follow-up since complete repair•History of syncope or rapid palpitations•Severe pulmonary regurgitation•Severe RV enlargement•Moderate to severe RV systolic dysfunction•Extensive RV fibrosis•Moderate-severe LV dysfunction•Elevated LV end-diastolic pressure•QRS duration ≥180 ms•Nonsustained VT on the Holter monitor•History of atrial tachycardia•Inducible VT at EPSEPS = electrophysiology study; LV = left ventricular; RV = right ventricular; VT = ventricular tachycardia. Open table in a new tab EPS = electrophysiology study; LV = left ventricular; RV = right ventricular; VT = ventricular tachycardia. RV size and function have always featured prominently in risk analysis for this group.19Gatzoulis M.A. Balaji S. Webber S.A. Siu S.C. Hokanson J.S. Poile C. Rosenthal M. Nakazawa M. Moller J.H. Gillette P.C. Webb G.D. Redington A.N. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study.Lancet. 2000; 356: 975-981Abstract Full Text Full Text PDF PubMed Scopus (1389) Google Scholar, 25Kavey R.E. Thomas F.D. Byrum C.J. Blackman M.S. Sondheimer H.M. Bove E.L. Ventricular arrhythmias and biventricular dysfunction after repair of tetralogy of Fallot.J Am Coll Cardiol. 1984; 4: 126-131Abstract Full Text PDF PubMed Scopus (58) Google Scholar, 26Harrison D.A. Harris L. Siu S.C. MacLoghlin C.J. Connelly M.S. Webb G.D. Downer E. McLaughlin P.R. Williams W.G. Sustained ventricular tachycardia in adult patients late after repair of tetralogy of Fallot.J Am Coll Cardiol. 1997; 30: 1368-1373Abstract Full Text Full Text PDF PubMed Scopus (233) Google Scholar Although residual pulmonary stenosis or ventricular septal defect are now unusual after tetralogy corrections, pulmonary regurgitation remains a common sequela that will contribute to volume overload and RV dysfunction. Multiple studies confirm that RV enlargement27Daliento L. Rizzoli G. Menti L. Baratella M.C. Turrini P. Nava A. Dalla Volta S. Accuracy of electrocardiographic and echocardiographic indices in predicting life threatening ventricular arrhythmias in patients operated for tetralogy of Fallot.Heart. 1999; 81: 650-655PubMed Google Scholar will elevate the VT and SCD risks. Gatzoulis et al28Gatzoulis M.A. Till J.A. Somerville J. Redington A.N. Mechanoelectrical interaction in tetralogy of Fallot. QRS prolongation relates to right ventricular size and predicts malignant ventricular arrhythmias and sudden death.Circulation. 1995; 92: 231-237Crossref PubMed Scopus (644) Google Scholar were the first to demonstrate the close correlation between QRS duration and RV size. From their data and several subsequent studies, the risk of SCD events increases whenever the RV is severely enlarged and QRS duration reaches or exceeds 180 ms. Depressed RV systolic function also appears to confer higher risk,29Valente A.M. Gauvreau K. Assenza G.E. et al.Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort.Heart. 2014; 100: 247-253Crossref PubMed Scopus (307) Google Scholar as does the finding of extensive RV fibrosis detected by magnetic resonance imaging.30Park SJ1 On YK Kim J.S. Park S.W. Yang J.H. Jun T.G. Kang I.S. Lee H.J. Choe Y.H. Huh J. Relation of fragmented QRS complex to right ventricular fibrosis detected by late gadolinium enhancement cardiac magnetic resonance in adults with repaired tetralogy of Fallot.Am J Cardiol. 2012; 109: 110-115Abstract Full Text Full Text PDF PubMed Scopus (79) Google Scholar Contemporary analysis of tetralogy risk has begun to shift away from exclusive RV evaluation to include more emphasis on LV function. Evidence is now accumulating that LV dysfunction may in fact be a better discriminator for high-risk status than traditional RV variables. No doubt there is a strong component of RV-LV interaction at work in patients with tetralogy,31Kempny A. Diller G.P. Orwat S. Kaleschke G. Kerckhoff G. Bunck ACh Maintz D. Baumgartner H. Right ventricular-left ventricular interaction in adults with tetralogy of Fallot: a combined cardiac magnetic resonance and echocardiographic speckle tracking study.Int J Cardiol. 2012; 154: 259-264Abstract Full Text Full Text PDF PubMed Scopus (106) Google Scholar but several measures of LV performance have been convincingly linked to risk. Khairy et al32Khairy P. Harris L. Landzberg M.J. et al.Implantable cardioverter-defibrillators in tetralogy of Fallot.Circulation. 2008; 117: 363-370Crossref PubMed Scopus (394) Google Scholar looked at discharge rate of implantable cardioverter-defibrillators (ICDs) in a large group of patients with tetralogy and found LV end-diastolic pressure to be the strongest predictor of appropriate shock. Similarly, Diller et al33Diller G.P. Kempny A. Liodakis E. Alonso-Gonzalez R. Inuzuka R. Uebing A. Orwat S. Dimopoulos K. Swan L. Li W. Gatzoulis M.A. Baumgartner H. Left ventricular longitudinal function predicts life-threatening ventricular arrhythmia and death in adults with repaired tetralogy of Fallot.Circulation. 2012; 125: 2440-2446Crossref PubMed Scopus (200) Google Scholar found that echocardiographic measures of LV function could enhance predictive accuracy when combined with RV functional measures. A recently completed multicenter study from the Pediatric and Congenital Electrophysiology Society (PACES) found that moderate to severe LV systolic dysfunction on the echocardiogram was a stronger predictor of serious events than any RV variable across all surgical eras.34Walsh E.P. Gonzales C. Atallah J. Pediatric and Congenital Electrophysiology Society. Multicenter case-control study of ventricular arrhythmia in tetralogy of Fallot [abstract].Heart Rhythm. 2013; 10: S46Abstract Full Text Full Text PDF PubMed Scopus (13) Google Scholar The results of these and other studies35Ghai A. Silversides C. Harris L. Webb G.D. Siu S.C. Therrien J. Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of Fallot.J Am Coll Cardiol. 2002; 40: 1675-1680Abstract Full Text Full Text PDF PubMed Scopus (358) Google Scholar serve to emphasize the importance of systemic ventricular performance in CHD, even when the hemodynamic and surgical stress is ostensibly weighted toward the subpulmonary ventricle. Noninvasive rhythm monitoring for ventricular ectopy burden has long been used as part of risk stratification, but because ectopy is rather ubiquitous in the adult population with tetralogy,36Jonsson H. Ivert T. Brodin L.A. Jonasson R. Late sudden deaths after repair of tetralogy of Fallot: electrocardiographic findings associated with survival.Scand J Thorac Cardiovasc Surg. 1995; 29: 131-139Crossref PubMed Scopus (33) Google Scholar there has always been concern regarding its specificity. Czosek et al37Czosek R.J. Anderson J. Khoury P.R. Knilans T.K. Spar D.S. Marino B.S. Utility of ambulatory monitoring in patients with congenital heart disease.Am J Cardiol. 2013; 111: 723-730Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar recently reported that nonsustained VT (4 or more beats) on the Holter monitor was indeed associated with sudden cardiac events in adults after tetralogy repair, while lower grades of ectopy (frequent isolated beats, couplets, and triplets) were not. A history of atrial tachycardia has also been shown to be associated with a higher risk of VT and death in older patients with tetralogy.29Valente A.M. Gauvreau K. Assenza G.E. et al.Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort.Heart. 2014; 100: 247-253Crossref PubMed Scopus (307) Google Scholar Specificity is again called into question since 20% or more of adult patients with tetralogy may have atrial flutter and/or fibrillation,38Roos-Hesselink J. Perlroth M.G. McGhie J. Spitaels S. Atrial arrhythmias in adults after repair of tetralogy of Fallot: correlations with clinical, exercise, and echocardiographic findings.Circulation. 1995; 91: 2214-2219Crossref PubMed Scopus (232) Google Scholar, 39Yap S.C. Harris L. Chauhan V.S. Oechslin E.N. Silversides C.K. Identifying high risk in adults with congenital heart disease and atrial arrhythmias.Am J Cardiol. 2011; 108: 723-728Abstract Full Text Full Text PDF PubMed Scopus (54) Google Scholar and there are undeniably strong links to hemodynamic variables such RV function, tricuspid regurgitation, and atrial size. Whether atrial tachycardia warrants attention as an independent variable remains uncertain, but it does appear useful when viewed in the context of RV hemodynamics. Often overlooked or unreported in many studies of SCD in tetralogy is the simple matter of historical symptoms in advance of a malignant event. A report from Koyak et al40Koyak Z. de Groot J.R. Van Gelder I.C. Bouma B.J. van Dessel P.F. Budts W. van Erven L. van Dijk A.P. Wilde A.A. Pieper P.G. Sieswerda G.T. Mulder B.J. Implantable cardioverter defibrillator therapy in adults with congenital heart disease: who is at risk of shocks?.Circ Arrhythm Electrophysiol. 2012; 5: 101-110Crossref PubMed Scopus (73) Google Scholar recently highlighted the importance of patient symptoms for risk stratification by demonstrating that a history of symptomatic (but not asymptomatic) nonsustained VT was the single best predictor of subsequent appropriate ICD discharge in their patients. Similarly, the multicenter PACES trial found that a history of syncope and/or rapid palpitations was the single most powerful item in differentiating high-risk patients from low-risk patients.34Walsh E.P. Gonzales C. Atallah J. Pediatric and Congenital Electrophysiology Society. Multicenter case-control study of ventricular arrhythmia in tetralogy of Fallot [abstract].Heart Rhythm. 2013; 10: S46Abstract Full Text Full Text PDF PubMed Scopus (13) Google Scholar No matter which risk factor is chosen from the studies published to date, the positive predictive accuracy for any single item can only be described as “modest.” Even QRS duration, which for a time was considered by many to indicate high-risk status whenever it exceeded 180 ms, has subsequently been shown to have limitations when analyzed in some other study groups.41Russo G. Folino A.F. Mazzotti E. Rebellato L. Daliento L. Comparison between QRS duration at standard ECG and signal-averaging ECG for arrhythmic risk stratification after surgical repair of tetralogy of Fallot.J Cardiovasc Electrophysiol. 2005; 16: 288-292Crossref PubMed Scopus (20) Google Scholar The uncertainty surrounding noninvasive risk assessment led some investigators to explore the merits of an invasive electrophysiology study (EPS) incorporating programmed ventricular stimulation. The single center study by Alexander et al42Alexander M.E. Walsh E.P. Saul J.P. Epstein M.R. Triedman J.K. Value of programmed ventricular stimulation in patients with congenital heart disease.J Cardiovasc Electrophysiol. 1999; 10: 1033-1044Crossref PubMed Scopus (104) Google Scholar and the subsequent multicenter study by Khai" @default.
• W2019914926 created "2016-06-24" @default.
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• W2019914926 date "2014-10-01" @default.
• W2019914926 modified "2023-09-24" @default.
• W2019914926 title "Sudden death in adult congenital heart disease: Risk stratification in 2014" @default.
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