congenital heart disease for the adult cardiologist · y the time patients with congenital heart...

Arrhythmias in Adult Patients With CongenitalHeart Disease

Edward P. Walsh, MD; Frank Cecchin, MD

By the time patients with congenital heart disease (CHD)reach adolescence and early adulthood, rhythm status

has often become an active issue, if not the central issue, intheir cardiac management. For some, arrhythmias are intrin-sic to the structural malformation itself, as is the case withWolff-Parkinson-White syndrome in the setting of Ebstein’sanomaly, or atrioventricular (AV) block in the setting of“congenitally corrected” transposition of the great arteries(L-TGA). For most other CHD patients, arrhythmias repre-sent an acquired condition related to the unique myocardialsubstrate created by surgical scars in conjunction with cya-nosis and abnormal pressure/volume loads of long duration.This review will attempt to address the major rhythm diffi-culties faced by adults with CHD, concentrating on electro-physiological features that distinguish CHD from more con-ventional forms of adult heart disease. A listing of specificarrhythmias and commonly associated congenital defects isprovided in Table 1 to serve as an outline for the discussion.

PrevalenceAs of 2001, there were estimated to be 800 000 adults withCHD living in the United States,1 a number that has grownsteadily as more survivors of childhood surgery reach matu-rity. Of these, roughly 45% are considered to have mild formsof CHD (eg, atrial septal defect, valvar pulmonary stenosis),40% are classified as having moderate disease (eg, tetralogyof Fallot, Ebstein’s anomaly), and 15% are considered tohave complex disease (eg, single ventricle, transposition ofthe great arteries). Although arrhythmias can develop withinany of the 3 subgroups, the incidence is highest for patients inthe moderate and severe categories. Tetralogy of Fallot is aninstructive example of moderate CHD with a large arrhyth-mia burden. As many as one third of patients with repairedtetralogy develop symptomatic atrial tachycardias by adult-hood,2 �10% develop high-grade ventricular arrhythmias,3

and �5% require pacemaker implantation for surgicallyacquired AV block or sinus node dysfunction.4 In addition, asmall but increasing number are now receiving implantablecardioverter defibrillators (ICDs) for treatment of ventriculartachyarrhythmias.5 Clearly, serious arrhythmias develop in asizable segment of the population with repaired tetralogy, allof which contribute to a sudden death risk that is currently

measured at �2% per decade of follow-up.6 Even when usingconservative population assumptions, this translates on anational level to more than 50 000 adult patients with tetral-ogy who require close electrophysiological attention, andperhaps as many as 100 sudden arrhythmic deaths per year ifpatients were left untreated. Although the magnitude of thissudden death risk may seem small compared with the threatfaced by older patients with ischemic heart disease, theemotional impact of unexpected death in a young adult whohas struggled with CHD throughout his or her abbreviatedlifetime is nonetheless profound.

The situation is equally concerning for patients falling intothe category of severe CHD. The Fontan operation for singleventricle, as well as the atrial switch operations (Mustard orSenning) for transposition of the great arteries, results inextensive suture lines and abnormal hemodynamics thatpredispose patients to atrial tachycardias and sinus nodedysfunction. Up to 50% of patients with single ventricle whohave undergone older-style Fontan operations will developatrial tachycardia within a decade of surgery,7 and hardly anytransposition patients remain in reliable sinus rhythm 10years after Mustard or Senning repairs.8 These patients posechallenges in terms of both rhythm control9 and prevention ofthromboembolic complications.10 Any center with a sizableadult CHD program is now likely to be involved withongoing care for hundreds of such cases.

The rhythm prognosis for older CHD patients is sobering,but in some ways, it might be viewed as an indirect triumphfor pediatric cardiology and cardiac surgery. Patients who inpast years would have perished during infancy are nowsurviving thanks to accurate anatomic diagnosis and earlysurgical intervention. Late-onset arrhythmias in adulthoodappear to have become the price to pay for decades ofeffective hemodynamic palliation, and fortunately, treatmentoptions for the arrhythmias themselves are improving rapidlyto meet the emerging demand. Perhaps the most optimisticfacet of the arrhythmia experience in CHD involves modernrefinements in surgical strategy that have arisen in responseto dissatisfaction with earlier electrophysiological outcomes.Nowadays, transposition is managed with direct arterialswitch rather than large atrial baffles, Fontan connections areconstructed in such a way as to minimize right atrial dilation,

From the Electrophysiology Division, Department of Cardiology, Children’s Hospital Boston, and Department of Pediatrics, Harvard Medical School,Boston, Mass.

Correspondence to Edward P. Walsh, MD, Cardiac Electrophysiology, Children’s Hospital Boston, 300 Longwood Ave, Boston MA 02115. [email protected]

(Circulation. 2007;115:534-545.)© 2007 American Heart Association, Inc.

Circulation is available at http://www.circulationaha.org DOI: 10.1161/CIRCULATIONAHA.105.592410

534

Congenital Heart Disease for the Adult Cardiologist

by guest on October 4, 2017

http://circ.ahajournals.org/D

ownloaded from

http://circ.ahajournals.org/

and tetralogy of Fallot is being repaired at younger ages withsmaller right ventricular (RV) incisions and attempts topreserve competence of the pulmonary valve. Modificationsof this sort have resulted in a promising reduction in theincidence of late arrhythmias11–14 for the younger generationof CHD patients.

Tachycardias in Adults With CHDAccessory Pathways and Twin AV NodesThe embryological accidents responsible for congenital heartdefects can have a direct impact on development of theconduction system. Sometimes this takes the form of simpledisplacement of the AV node and His bundle away from theusual septal position in Koch’s triangle,15 but occasionally,the malformation results in accessory or duplicated AVconnections with the potential for reentrant tachyarrhythmias.The most familiar example involves Ebstein’s anomaly of thetricuspid valve, which is complicated by Wolff-Parkinson-White syndrome in �20% of cases.16 The accessory path-ways in Ebstein’s anomaly are typically located along theposterior and septal aspect of the tricuspid ring where the

valve leaflets are most abnormal,17 and nearly half of thesepatients will be found to have multiple accessory path-ways.18,19 The same observations hold true for patients withL-TGA, who not infrequently have Ebstein’s malformation inassociation with accessory pathways along their left-sidedtricuspid valve.

Tachycardia events for Ebstein’s patients become increas-ingly problematic in adolescent and adult years when atrialdilation increases the likelihood of recurrent atrial flutter oratrial fibrillation with potentially rapid anterograde conduc-tion over the accessory pathway(s). Definitive therapy withcatheter ablation is now viewed as the standard of care forEbstein’s patients with Wolff-Parkinson-White syndrome.However, compared with ablation for simple accessory path-ways in a structurally normal heart, it must be recognized thatthe short-term success rate appears lower, and the risk ofrecurrence higher, in Ebstein’s anomaly.20,21 These differ-ences result from the challenges of distorted anatomic land-marks, difficulty identifying the true AV groove,22 extremelyfractionated electrograms, and the high incidence of multiplepathways.

Figure 1. Representation of twin AV nodes with aMönckeberg sling. The cardiac anatomy in thissketch includes a large septal defect in the AVcanal region, shown in a right anterior oblique proj-ection. Both an anterior and posterior AV node aredepicted (each with its own His bundle) along witha connecting “sling” between the 2 systems. Thisconduction arrangement can produce 2 distinctnonpreexcited QRS morphologies (depending onwhich AV node is engaged earliest by the atrialactivation wave front), and a variety of reentranttachycardias.

TABLE 1. Specific Arrhythmias and Associated Defects in Adults With Congenital Heart Disease

Arrhythmias Associated Defects

Tachycardias

Accessory pathways Ebstein’s anomaly; L-TGA

Twin AV nodes Heterotaxy syndrome

Intra-atrial reentrant tachycardia (atrial flutter) Postoperative Mustard; postoperative Senning; postoperative Fontan; others

Atrial fibrillation Mitral valve disease; aortic stenosis; unrepaired single ventricle

Ventricular tachycardia Tetralogy of Fallot; congenital aortic stenosis; others

Bradycardias

Congenital sinus node dysfunction Heterotaxy syndrome

Acquired sinus node dysfunction Postoperative Mustard; postoperative Senning; postoperative Fontan;postoperative Glenn; others

Congenital AV block Endocardial cushion defects; L-TGA

Acquired AV block VSD closure; subaortic stenosis relief; AV valve replacement

VSD indicates ventricular septal defect.

Walsh and Cecchin Arrhythmias in Adult CHD 535



ownloaded from


A more exotic type of redundant AV connection canoccasionally be identified in patients with a single ventricle ofthe heterotaxy variety, when there is a specific combinationof anatomic defects involving discordant AV alignment and alarge septal defect in the AV canal region. In this situation,patients can actually have 2 separate AV nodes (so-calledtwin AV nodes) with 2 discrete His bundles,23 often withevidence of a connecting fiber24 between these 2 systems(so-called Mönckeberg sling). A wide variety of reentranttachycardias can arise within this complicated network (Fig-ure 1), most of which can be eliminated by strategic ablationof 1 limb of the duplicated system.25,26 Although twin AVnodes are very rare, the condition epitomizes the peculiarconduction arrangements that are possible in CHD.

Intra-Atrial Reentrant Tachycardia (Atrial Flutter)The most common mechanism for symptomatic tachycardiain the adult CHD population is macroreentry within atrialmuscle.27 The terms “intra-atrial reentrant tachycardia”(IART) and “incisional tachycardia” have become customarylabels for this arrhythmia in order to distinguish it from thetypical variety of atrial flutter that occurs in structurallynormal hearts.28–30 Generally, IART tends to be slower thantypical flutter, with atrial rates in the range of 150 to 250 perminute. In the setting of a healthy AV node, such rates willfrequently conduct in a rapid 1:1 A:V pattern that can resultin hypotension, syncope, or possibly cardiac arrest.9,31 Even ifthe ventricular response rate is safely titrated, sustained IARTcan cause debilitating symptoms in some patients from loss ofAV synchrony and may contribute to thromboembolic com-plications10 when the duration is protracted.

Usually, IART appears many years after operations thatinvolved an atriotomy or other surgical manipulation of rightatrial tissue. It can follow simple procedures such as closureof an atrial septal defect on occasion, but the incidence ishighest among patients with advanced dilation, thickening,and scarring of their right atrium.32,33 Other risk factors forIART include concomitant sinus node dysfunction (tachy-brady syndrome) and older age at time of heart surgery.7

Hence, IART is especially problematic for older patients whohave undergone the Mustard, Senning, or older-style Fontanoperations, in which extensive suture lines and long-termhemodynamic stress result in markedly abnormal atrialmyocardium.

The route of propagation for an IART circuit variesaccording to the anatomic defect and type of surgical repair.34

It is usually restricted to right atrial tissue (regardless of thesurgical destination of this tissue) and is modulated byregions of fibrosis from suture lines or patches, whichfunction in combination with natural conduction barriers(crista terminalis, valve orifices, and the superior and inferiorcaval orifices) to channel the wave front along a macroreen-trant loop.35,36 If a tricuspid valve is present, the isthmusbetween the valve ring and the inferior vena cava is acommon component of such circuits, but when the tricuspidvalve is absent or otherwise deformed, the circuits follow lesspredictable paths that can only be deciphered by formalelectrophysiological mapping (Figure 2). Quite often, multi-ple IART circuits can be present in the same patient.37

The clinical diagnosis of IART can usually be made fromthe standard ECG. Uninitiated clinicians may occasionallymisinterpret IART with 2:1 or 3:1 conduction as sinus rhythmif atrial activity happens to be obscured by the QRS andT-wave (Figure 3). The index of suspicion for IART shouldalways be high in older CHD patients; if there is everuncertainty about the underlying rhythm, vagal maneuversought to be performed in an effort to uncover hidden P waves.Once recognized, IART can be reliably terminated withelectrical cardioversion, overdrive pacing maneuvers,38 oradministration of certain class I or class III antiarrhythmicdrugs. The far more difficult task is prevention of recurrence.Multiple strategies have been developed for IART preven-tion, all of which can have value in selected patients, but noneof which represents the universal solution. If the episodes areinfrequent, well tolerated, and recognized promptly, it may besufficient to rely on periodic cardioversion before embarkingon more involved therapy. However, if IART episodesbecome frequent, cause significant symptoms, or are associ-ated with atrial thrombus formation, aggressive treatment isindicated.

The therapeutic options for IART include (1) antiarrhyth-mic drugs, (2) pacemaker implantation to correct bradycardiaand/or provide automatic atrial antitachycardia pacing, (3)catheter ablation, and (4) surgical intervention with a modi-

Figure 2. Electroanatomic map of an IART circuit involving theanterolateral surface of the right atrium in a patient with a previ-ous Fontan operation (cavopulmonary connection). A detailedanatomic shell was generated for the ablation procedure bymerging high-resolution computed tomography data with real-time data gathered from the 3D mapping catheter. The propa-gation pattern for the IART circuit is shown by the black arrowsand is also reflected by the color scheme (red � yellow � green� blue � purple). The critical component of the circuit appearedto be a narrow conduction channel through a region of scar(central gray area). A cluster of radiofrequency applications(maroon dots) was placed at the entrance zone to this narrowchannel and permanently eliminated this IART circuit. IVC indi-cates inferior vena cava; LPA, left pulmonary artery; RPA, rightpulmonary artery; SVC, superior vena cava; RA, right atrium;JXN, junction; and LAT, lateral.

536 Circulation January 30, 2007



ownloaded from


fied atrial maze operation. The choice must be tailored to thehemodynamic and electrophysiological status of the individ-ual patient. Chronic antiarrhythmic drugs are still prescribedin some cases, but the broad experience with pharmacologicaltherapy for this condition has been discouraging,9,39 evenwhen potent agents such as amiodarone are used. Pacemakerimplantation may be reasonable for patients with a picture oftachy-brady syndrome, because correcting slow atrial rates tothe physiological range often results in reduction of IARTfrequency.31 Pacemakers with advanced programming fea-tures that incorporate atrial tachycardia detection and auto-matic burst pacing to interrupt reentry may also be beneficialin select cases.31,40

Catheter ablation is now used at many centers as an earlyintervention for IART.41 The technique has evolved rapidlysince the introduction of 3D mapping for improved circuitlocalization28,35,37,42 and irrigated-tip or large-tip ablationcatheters for more effective lesion creation.42–44 When thesetechnologies are combined with good anatomic definition andtraditional electrophysiological mapping maneuvers, short-term success rates of nearly 90% can be achieved. Unfortu-nately, later tachycardia recurrence is still disappointinglycommon. The recurrence risk is particularly high (nearly40%) among Fontan patients, who tend to have the largest

number of IART circuits and the thickest/largest atrial dimen-sions. Although far from perfect, ablation outcomes for IARTare likely to improve with continued experience and evennow are far superior to the degree of control obtained withmedications alone. Furthermore, even if IART episodes arenot eliminated entirely by ablation, the procedure can oftenprovide substantial improvement by reducing the frequencyof episodes and eliminating the need for ongoing drugtherapy.42

If the above measures fail to prevent IART, or if the patientis returning to the operating room for hemodynamic reasons,consideration should be given to surgical ablation during aright atrial maze operation. This procedure is used most oftenfor the Fontan population with the most refractory variety ofIART and is usually combined with revision of the Fontanconnection from an older atriopulmonary anastomosis to amodern cavopulmonary connection in the same setting.45

Results are encouraging, with relatively low rates of IARTrecurrence (�12%), but surgical risks must be weighedagainst the electrophysiological benefit. A prospective trialaimed at comparing various IART management optionswould be welcome, particularly one that could address themuch debated issue of catheter ablation versus surgical mazein Fontan patients.42,45

B

RV

RVOT patch

TV

VSD patch

RV

LV

RVOT patch

MPA

A

Figure 4. Macroreentrant VT in tetralogy of Fallot. A, An autopsy specimen of repaired tetralogy with the anterior RV surface opened toreveal the ventricular septal defect (VSD) patch and the patch-augmented RV outflow tract (RVOT; the outflow patch in this case istransannular). A hypothetical reentry circuit is traced onto this image (black arrows), with the superior portion of the loop travelingthrough the conal septum (upper rim of the VSD). B, Actual electroanatomic map of sustained VT from an adult tetralogy patient, show-ing a nearly identical circuit. The propagation pattern is shown by the black arrows and is reflected by the color scheme (red � yellow� green � blue � purple). A narrow conduction channel was found between the rightward edge of the outflow patch scar (gray area)and the superior rim of the tricuspid valve. A cluster of radiofrequency applications at this site (pink dots) closed off the channel andpermanently eliminated this VT circuit. LV indicates left ventricle; MPA, main pulmonary artery; and TV, tricuspid valve.

Figure 3. ECG (simultaneous leads I, aVf,and V1) from a 49-year-old woman whohad undergone uncomplicated closure ofan atrial septal defect many years prior,now presenting with a 6-day history ofintermittent palpitations. A, Regular rhythmwith a ventricular rate of 118 bpm is seen,which at casual first glance could be mis-taken for sinus tachycardia. After a Val-salva maneuver (B), the conduction patternbecame more irregular, which allowedclearer demonstration of the underlyingrapid atrial activity (arrows in lead V1) at arate of �236 bpm. The diagnosis of IARTcan sometimes be difficult during periodsof 2:1 conduction unless one is attuned toits high probability in CHD patients.




ownloaded from


Atrial FibrillationThe principle hemodynamic derangement and site of surgicalscarring in CHD tends to involve right heart structures, so thatIART arising from the right atrium is far and away the mostcommon form of atrial tachycardia. However, chronic hemo-dynamic stress is directed toward the left atrium in a subset ofCHD patients, and atrial fibrillation may occur as a result.The CHD lesions commonly associated with atrial fibrillationinclude aortic stenosis, mitral valve deformities, and unre-paired single ventricle.46 Management principles are similarto atrial fibrillation encountered in other forms of adult heartdisease, beginning with medical therapy for anticoagulationand ventricular rate control, followed by electrical or medicalcardioversion. As with IART, terminating an isolated atrialfibrillation episode in CHD is not difficult, but prevention ofrecurrence remains a challenge. Antiarrhythmic drugs mayoffer long-term protection against recurrence for some pa-tients, but like IART, pharmacological therapy has been onlymarginally successful for this purpose. Pacemaker implanta-tion may reduce atrial fibrillation recurrences in patients withsinus node dysfunction when fibrillation is part of thetachy-brady syndrome. Definitive elimination of atrial fibril-lation can be achieved with a combined right and left atrialmaze operation, which should be considered if a patientrequires surgery to address other hemodynamic issues.47 Tothe best of our knowledge, catheter ablation has not yet beenreported for atrial fibrillation in the setting of CHD.

Ventricular TachycardiaSerious ventricular arrhythmias are rare among CHD patientsduring their first decade or 2 of life, but once adulthood isreached, the potential for ventricular tachycardia (VT) andsudden death becomes a looming concern in select cases.Patients at greatest risk for developing VT appear to be thosewho have undergone a ventriculotomy and/or patching forcertain types of ventricular septal defects. In this scenario, themechanism for VT is reminiscent of the macroreentrantcircuits described earlier for IART, involving narrow conduc-tion corridors defined by regions of surgical scar48,49 inconjunction with natural conduction barriers such as the rimof a septal defect or edge of a valve annulus (Figure 4). Lesscommonly, ventricular arrhythmias can develop indepen-dently of direct surgical scarring whenever a long-standinghemodynamic overload causes advanced degrees of ventricledysfunction or hypertrophy. Examples of CHD lesions thatcan eventually lead to this myopathic variety of VT include(1) aortic valve disease,50,51 (2) L-TGA when the RV hasbeen recruited as the systemic ventricle,52 (3) severe Ebstein’s

anomaly, (4) certain forms of single ventricle, (5) Eisen-menger’s syndrome, and (6) unrepaired tetralogy of Fallot.53

The bulk of literature and clinical experience regarding VTin CHD has centered on tetralogy of Fallot. The prevalence ofVT after tetralogy repair has been estimated to be between3% and 14% in several large clinical series.2,3,6,54–56 Somepatients with slow VT may be hemodynamically stable atpresentation, but VT tends to be rapid for the majority,causing syncope or cardiac arrest as the presenting symptom.Even though rare cases of abrupt AV block or rapidlyconducted IART have been linked to catastrophic outcomesin tetralogy,57 sustained VT appears to be the single biggestcontributor to the 2% per decade incidence of sudden cardiacdeath (Table 2).6,55,58–60

Predicting VT events in tetralogy patients has been a topicof intense investigation for nearly 30 years. Selected articlesfrom the long list of studies* seeking risk factors aresummarized in Table 3. To date, no perfect risk-stratificationscheme has emerged, although several clinical variables withmodest prognostic value have been identified, including (1)older age at time of definitive surgery, (2) history of palliativeshunts, (3) high-grade ventricular ectopy, (4) inducible VT atelectrophysiological study, (5) abnormal RV hemodynamics,and (6) wide QRS width (�180 ms). The recently appreciatedcorrelation between QRS duration and VT54,55 is not surpris-ing when one considers that the most dramatic degree of QRSprolongation tends to be seen among tetralogy patients withhighly dysfunctional and dilated RVs (so-called mechano-electric interaction). When viewed in the aggregate, this longlist of variables helps to define a clinical profile for thetetralogy patient at risk, but no single item can be viewed ascompletely independent, and none provides perfect predictiveaccuracy.

If this generalized high-risk profile were applied to anycontemporary population of adult tetralogy patients, thenumber who fit the mold could be impossibly large. Tocompensate for lack of specificity, the practical approach toVT risk stratification in older tetralogy patients usuallyincorporates attention to symptom status. Obviously, anypatient who has survived a cardiac arrest or sustained VT istreated aggressively, usually with an ICD.5,82 But, in theabsence of a serious clinical event, careful inquiry for moresubtle symptoms is often relied on to determine whetheradditional testing or treatment is needed. At most centers,tetralogy patients who report concerning symptoms of palpi-tations, dizziness, or syncope usually undergo invasive eval-

*References 2, 3, 6, 14, 48, 49, 54–58, and 61–84.

TABLE 2. Estimates of the Incidence of Sudden Death After Tetralogy ofFallot Surgery

Study Findings Incidence per Decade, %

Murphy et al6 6% of 163 cases followed up for 30 years 2.0

Nollert et al58 3% of 490 cases followed up for 25 years 1.2

Silka et al59 �2 Deaths per 1000 patient-years 2.0

Norgaard et al60 5.6% of 125 cases followed up for 25 years 2.2

Gatzoulis et al55 6% of 793 cases followed up for 21 years 3.0




ownloaded from


uation with hemodynamic catheterization and electrophysiol-ogy study. Programmed ventricular stimulation providesreasonably good predictive information on the risk of futureclinical VT events.83,85 A positive study may prompt implan-tation of a primary prevention ICD,5 or if monomorphic VTcan be induced and is tolerated long enough to permitmapping, catheter ablation of the VT circuit might be con-sidered.86–89 An electrophysiology study might also uncoverIART as a contributing or confounding factor for a patient’ssymptoms, which could be addressed with ablation at the

same setting. Correctable hemodynamic issues may also beidentified at catheterization that could shift therapy toward asurgical solution, such as relief of valve regurgitation com-bined with formal intraoperative VT mapping and ablation.79

The proper approach to an entirely asymptomatic adultwith repaired tetralogy remains unsettled. Most clinicians relyon a yearly evaluation with history and ECG, supplementedregularly with Holter monitoring or exercise testing to screenfor high-grade ventricular ectopy, along with periodic echo-cardiography or magnetic resonance imaging to monitor the

TABLE 3. Selected Investigations Into Potential Risk Factors for VT in Patients With Repaired Tetralogyof Fallot

Study Age at Repair Prior Shunt VEA EPS PS PR RV Function LV Function ECG

Gillette et al61 Yes

Garson et al62 Yes Yes

Horowitz et al48 Yes

Deanfield et al63 Yes

Garson et al64 Yes

Kugler et al65 Yes

Kavey et al66 Yes Yes Yes

Dunnigan et al67 Yes

Kobayashi et al68 Yes No No No

Burns et al69 Yes Yes

Deanfield et al53 Yes No

Garson et al70 Yes Yes Yes Yes

Deal et al71 Yes Yes

Walsh et al14 Yes

Chandar et al72 Yes Yes No Yes

Zimmerman et al73 Yes

Downar et al49 Yes Yes

Murphy et al6 Yes Yes

Cullen et al74 No

Jonsson et al75 Yes Yes

Roos-Hesselink et al2 Yes

Gatzoulis et al54 Yes Yes Yes

Balaji et al76 Yes

Nollert et al58 Yes Yes

Harrison et al3 No Yes Yes

Berul et al56 Yes

Daliento et al77 Yes Yes

Lucron et al78 Yes Yes Yes Yes

Gatzoulis et al55 Yes Yes Yes Yes

Therrien et al79 Yes Yes Yes

Hamada et al80 Yes

Ghai et al81 Yes Yes Yes Yes Yes

Dore et al82 No

Khairy et al83 Yes Yes Yes Yes

Russo et al84 No

Yes indicates study supports variable as predictive of malignant arrhythmias; No, study refutes variable as predictive of malignantarrhythmias; Age Repair, older age at time of definitive surgical correction; Prior Shunt, history of prior palliative shunt surgery; VEA,spontaneous high-grade ventricular ectopic activity on ECG or Holter monitoring; EPS, positive ventricular stimulation atelectrophysiology study; PS, residual pulmonary stenosis or other outflow obstruction; PR, pulmonary regurgitation; LV, left ventricular;and ECG, ECG findings (QRS duration, QT and JT dispersion).




ownloaded from


status of the RV. Should nonsustained VT be detected onsurveillance monitoring in an asymptomatic patient, or shouldRV function appear to be deteriorating, opinions still varywidely as to the appropriate response. Some clinicians wouldadvocate electrophysiology study to refine the arrhythmiarisk, some would recommend surgery for pulmonary valvereplacement, some would prescribe antiarrhythmic drugs,some would implant a primary prevention ICD, and somemight refrain from any treatment as long as the patientremains symptom free. Therapy continues to be individual-ized for asymptomatic patients depending largely on institu-tional experience and philosophy.

The lack of objective guidelines for VT prediction andtreatment in CHD patients is frustrating, but it would behazardous to adopt a dogmatic stance based on the data athand. With few exceptions,55,83 studies have been limited tosingle-center investigations with limited statistical power.Furthermore, because the event rate for sustained VT andsudden death in CHD is low compared with conditions suchas ischemic heart disease, the duration of prospectivefollow-up necessary to answer questions in the CHD fieldwould likely have to extend beyond 10 years. Now that thepopulation of adults with CHD at risk for VT has reachedsuch a substantial size, the opportunity may finally havearrived for an organized assessment of VT management. Theideal design for investigations of this type must involve closecollaboration between pediatric and adult CHD programs toensure continuity of data collection. It is incumbent oninterested clinicians to design and cooperate in long-termmulticenter studies of these arrhythmias.

Bradycardias in Adults With CHDSinoatrial Node Dysfunction in CHDDevelopmental defects involving the caval-atrial junction canbe associated with atypical anatomy and function for thesinoatrial node. This issue is most relevant to complex formsof heterotaxy syndrome in patients with a single ventricle. Inthe asplenia variety of heterotaxy, bilateral superior cavalveins often exist, each with its own sinoatrial node, whichresults in an interesting ECG pattern of fluctuation between 2discrete P waves at physiological rates. Apart from theunusual ECG, the coexistence of 2 sinus nodes is of minimalclinical consequence. In contrast, patients with the polysple-nia type of heterotaxy may lack a true sinus node altogether,which makes atrial depolarization dependent on slower atrialor junctional escape rhythms.90 Most patients with this rarecondition will ultimately require pacemaker implantation.

A more common cause of sinus bradycardia in adults withCHD is surgical trauma to the sinoatrial node or its artery, asmay occur during the Mustard, Senning, Glenn, and Fontanoperations.7,8,27,91 Chronotropic incompetence is poorly toler-ated in CHD patients with compromised hemodynamics,especially those with a single ventricle or AV valve regurgi-tation. The likelihood of a patient developing IART or atrialfibrillation is also increased significantly in this setting.7

Implantation of an AAIR or DDDR pacing system iscurrently recommended92 as a class I indication for anypatient with CHD and sinoatrial node dysfunction who has

symptoms directly attributable to slow heart rate. Pacemakerimplantation is also advised as a class IIb indication for CHDpatients with resting rates of �40 bpm or sinus pauses inexcess of 3 seconds, even in the absence of symptoms. Aswill be discussed, decisions regarding device implantationcannot be made casually in CHD patients because of the needfor epicardial implants and distorted venous pathways inmany cases.

AV Block in CHDThe AV conduction tissues may be congenitally abnormal interms of both their location and function in specific forms ofCHD, most notably L-TGA and endocardial cushion de-fects.93–95 In the former condition, the AV node and Hisbundle are displaced in an anterior direction away from theusual position in Koch’s triangle, whereas in the latter, theAV node and His bundle are displaced posterior to Koch’striangle. The functional properties of these displaced conduc-tion systems are often abnormal. In L-TGA, it is estimatedthat 3% to 5% of patients will have complete AV block atbirth, and an additional 20% will develop spontaneouscomplete block by adulthood.96,97 Even when intrinsic con-duction appears normal, these patients appear to be moresusceptible to traumatic AV block during surgical or catheterprocedures.

Surgical repair of certain forms of CHD can result in directtrauma to the AV conduction tissues. Although improvedknowledge of the precise location for the AV node and Hisbundle in various forms of CHD15,94,95,98 has reduced theoccurrence, closure of some ventricular septal defects, sur-gery for left-heart outflow obstruction, and replacement orrepair of an AV valve may still be complicated by AV block.Fortunately, in more than half of cases, this injury is atransient affair related to myocardial stretch or edema ratherthan physical severing of the conduction tissues, and AVconduction recovers within 7 to 10 days of operation.4 But,for any patient with postoperative AV block that is notexpected to resolve or that persists at least 7 days after cardiacsurgery, permanent pacemaker implantation is advised92 as aclass I indication. A pacemaker may be considered by someas a class IIb indication when surgical AV block recovers butthe patient is left with permanent bifascicular block.57

Pacemaker and ICD Implantation in AdultsWith CHD

IndicationsAn important consideration when one interprets the AmericanCollege of Cardiology/American Heart Association/NorthAmerican Society of Pacing and Electrophysiology task forceguidelines for pacemaker implantation92 is that standardtransvenous systems may be contraindicated or difficult inmany CHD patients owing to complexities of venous anato-my or to the presence of significant intracardiac shunting thatcreates a thromboembolic risk from intravascular leads.Epicardial implantation is certainly possible in these circum-stances, although the surgery is more involved, and long-termlead performance may be inferior to transvenous systems.Additionally, epicardial lead placement in an older CHD




ownloaded from


patient who has undergone multiple prior cardiac operationspresents the surgeon with a highly scarred mediastinum;dissection down to the myocardial surface must be performedcarefully and deliberately to uncover sites with good sensingand pacing function. If a CHD patient is undergoing cardiacsurgery for other hemodynamic reasons and it appears highlylikely that epicardial pacing might be needed at some distantdate, the surgeon may want to take advantage of wideintraoperative exposure to place leads for future use. Fortu-nately, 86% of leads placed at the time of cardiac surgery arefound to function well when retrieved at a mean of 252 daysafter the operation.99

The indications for ICD implantation in CHD patients arestill evolving, but in general, they follow guidelines that aresimilar to other varieties of adult heart disease. The mostcommon type of CHD that requires ICD implantation istetralogy of Fallot, followed by transposition of the greatarteries and left-sided obstructive diseases.5,85,100

The experience with biventricular resynchronization pac-ing in CHD patients with depressed ventricular function islimited but promising.101 Clinical studies are now under wayto refine selection criteria, as well as to investigate the clinicalmerits of RV resynchronization to offset right bundle-branchblock after surgical closure of ventricular septal defects andtetralogy repair.

Technical ConsiderationsCareful preprocedural planning is essential for successfulpacemaker or ICD implantation in adults with CHD (Table4). Some of these patients can have anomalies of systemicvenous return and the coronary veins, the most common ofwhich is persistent left superior vena cava draining to thecoronary sinus.102 In this situation, the right superior venacava and innominate vein can vary in size from normal

caliber to completely absent. Implantation is still possiblethrough a left superior vena cava, although making thenecessary turn across the tricuspid valve from the coronarysinus is difficult and may require use of a long sheath or alarge right atrial loop to direct the tip toward the ventricle.103

If pacing within the coronary sinus is needed for cardiacresynchronization or any other purpose, anomalies of thecoronary sinus need to be considered, including ostial atre-sia,104 unroofed coronary sinus, and extreme dilation due topersistent left superior vena cava.

Venous occlusion is a recognized complication of perma-nent transvenous pacing leads.105,106 Risk factors for venousthrombosis after lead placement include the absence ofanticoagulant therapy, a history of prior venous thrombosis,use of female hormone therapy, and the presence of multiplepacing leads.107 Venous occlusion is therefore common inadult CHD patients with long-term pacing who may havemultiple leads in place for several decades. A contrastinjection into the antecubital vein(s) with follow-throughvenography is helpful to investigate acquired and congenitalabnormalities. If venous access is needed in the setting of acomplete venous occlusion, a variety of techniques forrecanalization and venous dilation are now available.108

Transvenous chamber access can be an issue if compli-cated atrial baffling has been used to redirect venous return.One major challenge in this regard occurs when ventricularpacing is needed after a Fontan repair. An epicardial approachis used in most of these cases, but successful transvenousventricular lead implantation after the Fontan operation hasnow been reported in several series, usually involving leadplacement into a coronary venous branch vessel.109–112

Combined challenges of surgical obstacles, chamber ac-cess, and unusual ventricular geometry are present in patientswith transposition of the great arteries after an atrial switch(Mustard or Senning) procedure.113 Extensive atrial bafflinglimits sites of atrial capture to small regions in the left atrialappendage (where phrenic nerve stimulation may be hard toavoid), anterior left atrial roof, or superior cava–right atrialjunction. Baffle obstruction is fairly common in these patientsand may require endovascular stent placement before leadplacement (Figure 5). In addition, the ventricular lead must beplaced in a morphological left ventricle that is thin andnontrabeculated, which requires close attention to tip fixationand sensing parameters.

TABLE 4. Special Considerations During TransvenousPacemaker and ICD Lead Implantation in Adults With CHD

Congenital venous anomalies

Acquired venous occlusion

Inability to access a chamber

Surgical obstacles (patches, conduits, and baffles)

Intracardiac shunts

Ventricular geometry and position

Figure 5. Chest radiograph demonstratingtransvenous ICD lead positions in a youngadult with L-TGA after a Mustard proce-dure (A, posteroanterior projection; B, lat-eral projection). Complete obstruction ofthe superior limb of the atrial baffle waspresent and required recanalization, dila-tion, and stent placement (arrow and inset)to open a path to the left ventricle. Theatrial lead was positioned in the superiorvena cava limb of the baffle proximal tothe stent. Note that the tip of the ICD leadis attached to the left ventricular apex.SVC/RA indicates superior vena cava andright atrial junction; LA, left atrium (whichbecame the neo-right atrium after surgery);and LAA, left atrial appendage.




ownloaded from


Intracardiac shunts can lead to embolic stroke via right-to-left shunting or inadvertent lead placement in the systemiccirculation.114 These shunts can occur at either the atrial orventricular level, ranging from a patent foramen ovale tolarge residual septal defects and patch leaks. In addition,patients with high central venous pressure (as often occursafter the Fontan repair) can develop direct connectionsbetween the supracardiac veins and pulmonary venous atriumthrough veno-venous collaterals.115,116 Trivial shunts that arepredominately left-to-right are probably not absolute contra-indications to transvenous leads, but larger shunts, particu-larly if right-to-left, need to be evaluated carefully by angiog-raphy or echocardiography before a final decision is made onthe route for lead implantation. If transvenous leads arestrongly preferred in such cases, shunt closure can be at-tempted beforehand with interventional techniques such asseptal occluders, covered stents, or even surgery.117,118 Ifintracardiac shunting cannot be eliminated satisfactorily,epicardial lead placement is probably the wisest alternative. Ifthere are still compelling indications for a transvenous sys-tem, anticoagulation is used after lead placement in patientswith residual shunting at most centers, although no firm datacurrently exist to verify the efficacy of this measure.

Transvenous ICD lead implantation requires a thoroughunderstanding of the ventricular anatomy and chamber posi-tions to anchor the lead tip securely and ensure a suitablevector for defibrillation. When epicardial ICD leads arerequired, there has been a shift away from traditional patchesto novel configurations involving coils (Figure 6) placed in asubcutaneous or pericardial position.119 The experience withthis approach is limited, but it appears to offer increasedflexibility that can accommodate a wide variety of ventriculargeometries and heart sizes.

ConclusionsArrhythmias in adults with CHD are a growing problem thatrequires unique solutions. The number of clinicians trained toprovide electrophysiological care to this unusual patientgroup is still somewhat limited. Cardiology and electrophys-iology training programs need to consider devoting morecurriculum time to this topic in an effort to improve caredelivery into the future. The paucity of evidence-basedmanagement protocols for arrhythmias in adults with CHD is

also a concern, and a need clearly exists for larger collabo-rative studies involving both pediatric and adult CHD centersto generate more objective treatment guidelines.

DisclosuresNone.

References1. Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman

JI, Somerville J, Williams RG, Webb GD. Task force 1: the changingprofile of congenital heart disease in adult life. J Am Coll Cardiol.2001;37:1170–1175.

2. Roos-Hesselink J, Perlroth MG, McGhie J, Spitaels S. Atrial arrhyth-mias in adults after repair of tetralogy of Fallot: correlations withclinical, exercise, and echocardiographic findings. Circulation. 1995;91:2214–2219.

3. Harrison DA, Harris L, Siu SC, MacLoghlin CJ, Connelly MS, WebbGD, Downer E, McLaughlin PR, Williams WG. Sustained ventriculartachycardia in adult patients late after repair of tetralogy of Fallot. J AmColl Cardiol. 1997;30:1368–1373.

4. Weindling SN, Gamble WJ, Mayer JE, Walsh EP. Duration of completeatrioventricular block after congenital heart disease surgery.Am J Cardiol. 1998;82:525–527.

5. Alexander ME, Cecchin F, Walsh EP, Triedman JK, Bevilacqua LM,Berul CI. Implications of implantable defibrillator therapy in congenitalheart disease and pediatrics. J Cardiovasc Electrophysiol. 2004;15:72–76.

6. Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM,McGoon DC, Kirklin JW, Danielson GK. Long-term outcome inpatients undergoing surgical repair of tetralogy of Fallot. N Engl J Med.1993;329:593–599.

7. Fishberger SB, Wernovsky G, Gentles TL, Gauvreau K, Burnett J,Mayer JE, Walsh EP. Factors that influence the development of atrialflutter after the Fontan operation. J Thorac Cardiovasc Surg. 1997;113:80–86.

8. Flinn CJ, Wolff GS, Dick M, Campbell RM, Borkat G, Casta A, HordofA, Hougen TJ, Kavey RE, Kugler J. Cardiac rhythm after the Mustardoperation for complete transposition of the great arteries. N Engl J Med.1984;3310:1635–1638.

9. Garson A, Bink-Boelkens MTE, Hesslein PS, Hordof AJ, Keane JF,Neches WH, Porter CJ. Atrial flutter in the young: a collaborative studyof 380 cases. J Am Coll Cardiol. 1985;6:871–878.

10. Feltes TF, Friedman RA. Transesophageal echocardiographic detectionof atrial thrombi in patients with nonfibrillation atrial tachyarrhythmiasand congenital heart disease. J Am Coll Cardiol. 1994;24:1365–1370.

11. Rhodes LA, Wernovsky G, Keane JF, Mayer JE, Shuren A, Dindy C,Colan SD, Walsh EP. Arrhythmias and intracardiac conduction after thearterial switch operation. J Thor Cardiovasc Surg. 1995;109:303–310.

12. Cecchin F, Johnsrude CL, Perry JC, Friedman RA. Effect of age andsurgical technique on symptomatic arrhythmias after the Fontan pro-cedure. Am J Cardiol. 1995;76:386–391.

13. Stamm C, Friehs I, Mayer JE Jr, Zurakowski D, Triedman JK, MoranAM, Walsh EP, Lock JE, Jonas RA, Del Nido PJ. Long-term results of

Figure 6. Chest radiograph of an epicar-dial ICD system in a young adult inwhom a Kawashima-type Fontan opera-tion was done for heterotaxy anddouble-outlet RV (A, posteroanterior proj-ection; B, lateral projection). No trans-venous access to any of the cardiacchambers existed, so the ICD system isepicardial. Defibrillation was obtained via2 coils placed in the pericardial space. Ashort 5-cm coil is present (large arrow),typically used in the superior vena cava,now placed in the pericardial spacealong the right side of the spine. Thesecond long, 25-cm coil (small arrows),normally implanted subcutaneously, liesunder the heart and then ascends alongthe right of the spine.




ownloaded from


the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg. 2001;121:28–41.

14. Walsh EP, Rockenmacher S, Keane JF, Hougen TJ, Lock JE, CastanedaAR. Late results in patients with tetralogy of Fallot repaired duringinfancy. Circulation. 1988;77:1062–1067.

15. Anderson RH, Ho SY. The disposition of the conduction tissues incongenitally malformed hearts with reference to their embryologicaldevelopment. J Perinat Med. 1991;19:201–206.

16. Attenhofer Jost CH, Connolly HM, Edwards WD, Hayes D, Warnes CA,Danielson GK. Ebstein’s anomaly: review of a multifaceted congenitalcardiac condition. Swiss Med Wkly. 2005;135:269–281.

17. Levine J, Walsh EP, Saul JP. Catheter ablation of accessory pathways inpatients with congenital heart disease including heterotaxy syndrome.Am J Cardiol. 1993;72:689–694.

18. Oh JK, Holmes DR Jr, Hayes DL, Porter CB, Danielson GK. Cardiacarrhythmias in patients with surgical repair of Ebstein’s anomaly. J AmColl Cardiol. 1985;6:1351–1357.

19. Smith WM, Gallagher JJ, Kerr CR, Sealy WC, Kasell JH, Benson DW,Reiter MJ, Sterba R, Grant AO. The electrophysiologic basis and man-agement of symptomatic recurrent tachycardia in patients with Ebstein’sanomaly of the tricuspid valve. Am J Cardiol. 1982;49:1223–1234.

20. Reich JD, Auld D, Hulse E, Sullivan K, Campbell R; Pediatric Electro-physiology Society. The Pediatric Radiofrequency Ablation Registry’sexperience with Ebstein’s anomaly. J Cardiovasc Electrophysiol. 1998;9:1370–1377.

21. Chetaille P, Walsh EP, Triedman JK. Outcomes of radiofrequencycatheter ablation of atrioventricular reciprocating tachycardia in patientswith congenital heart disease. Heart Rhythm. 2004;1:168–173.

22. Shah MJ, Jones TK, Cecchin F. Improved localization of right-sidedaccessory pathways with microcatheter-assisted right coronary arterymapping in children. J Cardiovasc Electrophysiol. 2004;15:1238–1243.

23. Lev M, Licata RH, May RC. The conduction system in mixed levocardiawith ventricular inversion (corrected transposition). Circulation. 1963;28:232–237.

24. Mönckeberg JG. Zur entwicklungsgeschichte des atrioventrikular-systems. Verh Deutsch Path Ges. 1913;16:228–249.

25. Epstein MR, Saul JP, Weindling SN, Triedman JK, Walsh EP. Atrio-ventricular reciprocating tachycardia involving twin atrioventricularnodes in patients with complex congenital heart disease. J CardiovascElectrophysiol. 2001;12:671–679.

26. Wu MH, Wang JK, Lin JL, Lai LP, Lue HC, Young ML, Hsieh FJ.Supraventricular tachycardia in patients with right atrial isomerism.J Am Coll Cardiol. 1998;32:773–779.

27. Ghai A, Harris L, Harrison DA, Webb GD, Siu SC. Outcomes of lateatrial tachyarrhythmias in adults after the Fontan operation. J Am CollCardiol. 2001;37:585–592.

28. Nakagawa H, Shah N, Matsudaira K, Overholt E, Chandrasekaran K,Beckman KJ, Spector P, Calame JD, Rao A, Hasdemir C, Otomo K,Wang Z, Lazzara R, Jackman WM. Characterization of reentrant circuitin macroreentrant right atrial tachycardia after surgical repair of con-genital heart disease: isolated channels between scars allow “focal”ablation. Circulation. 2001;103:699–709.

29. Kalman JK, Van Hare GF, Olgin JE, Saxon LA, Stark SI, Lesh MD.Ablation of “incisional” reentrant atrial tachycardia complicatingsurgery for congenital heart disease. Circulation. 1996;93:502–512.

30. Triedman JK, Jenkins KJ, Colan SD, Saul JP, Walsh EP. Intra-atrialreentrant tachycardia after palliation of congenital heart disease: char-acterization of multiple macroreentrant circuits using fluoroscopicallybased three-dimensional endocardial mapping. J Cardiovasc Electro-physiol. 1997;8:259–270.

31. Rhodes LA, Walsh EP, Gamble WJ, Triedman JK, Saul JP. Benefits andpotential risks of atrial antitachycardia pacing after repair of congenitalheart disease. Pacing Clin Electrophysiol. 1995;18:1005–1016.

32. Li W, Somerville J. Atrial flutter in grown-up congenital heart (GUCH)patients: clinical characteristics of affected population. Int J Cardiol.2000;75:129–137.

33. Wong T, Davlouros PA, Li W, Millington-Sanders C, Francis DP,Gatzoulis MA. Mechano-electrical interaction late after Fontan oper-ation: relation between P-wave duration and dispersion, right atrial size,and atrial arrhythmias. Circulation. 2004;109:2319–2325.

34. Collins KK, Love BA, Walsh EP, Saul JP, Epstein MR, Triedman JK.Location of acutely successful radiofrequency catheter ablation of intra-atrial reentrant tachycardia in patients with congenital heart disease.Am J Cardiol. 2000;86:969–974.

35. Triedman JK, Alexander ME, Berul CI, Bevilacqua LM, Walsh EP.Electroanatomic mapping of entrained and exit zones in patients withrepaired congenital heart disease and intra-atrial reentrant tachycardia.Circulation. 2001;103:2060–2065.

36. Mandapati R, Walsh EP, Triedman JK. Pericaval and periannular intra-atrial reentrant tachycardias in patients with congenital heart disease.J Cardiovasc Electrophysiol. 2003;14:119–112.

37. Delacretaz E, Ganz LI, Friedman PL, Walsh EP, Triedman JK, Sloss LJ,Landzberg MJ, Stevenson WG. Multiple atrial macroreentry circuits inadults with repaired congenital heart disease: entrainment mappingcombined with three-dimensional electroanatomic mapping. J Am CollCardiol. 2001;37:1665–1676.

38. Rhodes LA, Walsh EP, Saul JP. Conversion of atrial flutter in pediatricpatients using transesophageal atrial pacing: a safe, effective, and min-imally invasive technique. Am Heart J. 1995;130:323–327.

39. Triedman JK. Atrial reentrant tachycardias. In: Walsh EP, Saul JP,Triedman JK, eds. Cardiac Arrhythmias in Children and Young AdultsWith Congenital Heart Disease. Philadelphia, Pa: Lippincott Williams& Wilkins; 2001:137–160.

40. Stevenson EA, Casavant D, Tuzi J, Alexander MA, Law I, Serwer G,Strieper M, Walsh EP, Berul CI. Efficacy of atrial antitachycardiapacing using the Medtronic AT500 pacemaker in patients with con-genital heart disease. Am J Cardiol. 2003;92:871–876.

41. Triedman JK, Bergau DM, Saul JP, Epstein MR, Walsh EP. Efficacy ofradiofrequency ablation for control of intraatrial reentrant tachycardia inpatients with congenital heart disease. J Am Coll Cardiol. 1997;30:1032–1038.

42. Triedman JK, Alexander MA, Love BA, Collins KK, Berul CI,Bevilacqua LM, Walsh EP. Influence of patient factors and ablativetechnologies on outcomes of radiofrequency ablation of intra-atrialtachycardia in patients with congenital heart disease. J Am Coll Cardiol.2002;39:1827–1835.

43. Jais P, Shah DC, Haissaguerre M, Hocini M, Garrigue S, Le Metayer P,Clementy J. Prospective randomized comparison of irrigated-tip versusconventional-tip catheters for ablation of common flutter. Circulation.2000;101:772–776.

44. Triedman JK, Delucca JM, Alexander ME, Berul CI, Cecchin F, WalshEP. Prospective trial of electroanatomically guided, irrigated catheterablation of atrial tachycardia in patients with congenital heart disease.Heart Rhythm. 2005;2:700–705.

45. Mavroudis C, Backer CL, Deal BJ, Johnsrude C, Strasburger J. Totalcavopulmonary conversion and maze procedure for patients with failureof the Fontan operation. J Thorac Cardiovasc Surg. 2001;122:863–871.

46. Kirsh JA, Walsh EP, Triedman JK. Prevalence of and risk factors foratrial fibrillation and intraatrial reentrant tachycardia among patientswith congenital heart disease. Am J Cardiol. 2002;90:338–340.

47. Deal BJ, Mavroudis C, Backer CL Beyond Fontan conversion: surgicaltherapy of arrhythmias including patients with associated complex con-genital heart disease. Ann Thorac Surg. 2003;76:542–553.

48. Horowitz LN, Vetter VL, Harken AH, Josephson ME. Electrophysi-ologic characteristics of sustained ventricular tachycardia occurring afterrepair of tetralogy of Fallot. Am J Cardiol. 1980;46:446–452.

49. Downar E, Harris L, Kimber S, Mickleborough L, Williams W,Sevaptsidis E, Masse S, Chen TC, Chan A, Genga A. Ventriculartachycardia after surgical repair of tetralogy of Fallot: results of intra-operative mapping studies. J Am Coll Cardiol. 1992;20:648–655.

50. Wolfe RR, Driscoll DJ, Gersony WM, Hayes CJ, Keane JF, Kidd L,O’Fallon WM, Pieroni DR, Weidman WH. Arrhythmias in patients withvalvar aortic stenosis, valvar pulmonary stenosis, and ventricular septaldefect: results of 24-hour ECG monitoring. Circulation. 1993;87(suppl):I-89–I-101.

51. Keane JF, Driscoll DJ, Gersony WM, Hayes CJ, Kidd L, O’Fallon WM,Pieroni DR, Wolfe RR, Weidman WH. Second natural history study ofcongenital heart defects: results of treatment of patients with aorticvalvar stenosis. Circulation. 1993;87(suppl):I-16–I-27.

52. Gatzoulis MA, Walters J, McLaughlin PR, Merchant N, Webb GD, LiuP. Late arrhythmia in adults with the mustard procedure for transpositionof great arteries: a surrogate marker for right ventricular dysfunction?Heart. 2000;84:409–415.

53. Deanfield JE, McKenna WJ, Presbitero P, England D, Graham GR,Hallidie-Smith K. Ventricular arrhythmia in unrepaired and repairedtetralogy of Fallot: relation to age, timing of repair, and haemodynamicstatus. Br Heart J. 1984;52:77–81.

54. Gatzoulis MA, Till JA, Somerville J, Redington AN. Mechanoelectricalinteraction in tetralogy of Fallot: QRS prolongation relates to right




ownloaded from


ventricular size and predicts malignant ventricular arrhythmias andsudden death. Circulation. 1995;92:231–237.

55. Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C,Rosenthal M, Nakazawa M, Moller JH, Gillette PC, Webb GD,Redington AN. Risk factors for arrhythmia and sudden cardiac death lateafter repair of tetralogy of Fallot: a multicentre study. Lancet. 2000;356:975–981.

56. Berul CI, Hill SL, Geggel RL, Hijazi ZM, Marx GR, Rhodes J, WalshKA, Fulton DR. Electrocardiographic markers of late sudden death riskin postoperative tetralogy of Fallot children. J Cardiovasc Electro-physiol. 1997;8:1349–1356.

57. Wolff GS, Rowland TW, Ellison RC. Surgically induced right bundle-branch block with left anterior hemiblock: an ominous sign in postop-erative tetralogy of Fallot. Circulation. 1972;46:587–594.

58. Nollert G, Fischlein T, Bouterwek S, Bohmer C, Klinner W, Reichart B.Long-term survival in patients with repair of tetralogy of Fallot: 36-yearfollow-up of 490 survivors of the first year after surgical repair. J AmColl Cardiol. 1997;30:1374–1383.

59. Silka MJ, Hardy BG, Menashe VD, Morris CD. A population-basedprospective evaluation of risk of sudden cardiac death after operation forcommon congenital heart defects. J Am Coll Cardiol. 1998;32:245–251.

60. Norgaard MA, Lauridsen P, Helvind M, Pettersson G. Twenty-to-thirty-seven-year follow-up after repair for Tetralogy of Fallot. Eur J Cardio-thorac Surg. 1999;16:125–130.

61. Gillette PC, Yeoman MA, Mullins CE, McNamara DG. Sudden deathafter repair of tetralogy of Fallot: electrocardiographic and electrophysi-ologic abnormalities. Circulation. 1977;56:566–571.

62. Garson A Jr, Nihill MR, McNamara DG, Cooley DA. Status of the adultand adolescent after repair of tetralogy of Fallot. Circulation. 1979;59:1232–1240.

63. Deanfield JE, Ho SY, Anderson RH, McKenna WJ, Allwork SP,Hallidie-Smith KA. Late sudden death after repair of tetralogy of Fallot:a clinicopathologic study. Circulation. 1983;67:626–631.

64. Garson A Jr, Porter CJ, Gillette PC, McNamara DG. Induction ofventricular tachycardia during electrophysiologic study after repair oftetralogy of Fallot. J Am Coll Cardiol. 1983;1:1493–1502.

65. Kugler JD, Pinsky WW, Cheatham JP, Hofschire PJ, Mooring PK,Fleming WH. Sustained ventricular tachycardia after repair of tetralogyof Fallot: new electrophysiologic findings. Am J Cardiol. 1983;51:1137–1143.

66. Kavey RE, Thomas FD, Byrum CJ, Blackman MS, Sondheimer HM,Bove EL. Ventricular arrhythmias and biventricular dysfunction afterrepair of tetralogy of Fallot. J Am Coll Cardiol. 1984;4:126–131.

67. Dunnigan A, Pritzker MR, Benditt DG, Benson DW Jr. Life threateningventricular tachycardias in late survivors of surgically correctedtetralogy of Fallot. Br Heart J. 1984;52:198–206.

68. Kobayashi J, Hirose H, Nakano S, Matsuda H, Shirakura R, KawashimaY. Ambulatory electrocardiographic study of the frequency and cause ofventricular arrhythmia after correction of tetralogy of Fallot.Am J Cardiol. 1984;54:1310–1313.

69. Burns RJ, Liu PP, Druck MN, Seawright SJ, Williams WG, McLaughlinPR. Analysis of adults with and without complex ventricular arrhyth-mias after repair of tetralogy of Fallot. J Am Coll Cardiol. 1984;4:226–233.

70. Garson A Jr, Randall DC, Gillette PC, Smith RT, Moak JP, McVey P,McNamara DG. Prevention of sudden death after repair of tetralogy ofFallot: treatment of ventricular arrhythmias. J Am Coll Cardiol. 1985;6:221–227.

71. Deal BJ, Scagliotti D, Miller SM, Gallastegui JL, Hariman RJ, LevitskyS. Electrophysiologic drug testing in symptomatic ventricular arrhyth-mias after repair of tetralogy of Fallot. Am J Cardiol. 1987;59:1380–1385.

72. Chandar JS, Wolff GS, Garson A Jr, Bell TJ, Beder SD, Bink-BoelkensM, Byrum CJ, Campbell RM, Deal BJ, Dick M, Flinn CJ, Guam WE,Gillette PC, Hordorf AJ, Kugler JD, Porter CJ, Walsh EP. Ventriculararrhythmia in post-operative tetralogy of Fallot. Am J Cardiol. 1990;65:655–661.

73. Zimmermann M, Friedli B, Adamec R, Oberhansli I. Ventricular latepotentials and induced ventricular arrhythmias after surgical repair oftetralogy of Fallot. Am J Cardiol. 1991;67:873–878.

74. Cullen S, Celermajer DS, Franklin RC, Hallidie-Smith KA, DeanfieldJE. Prognostic significance of ventricular arrhythmia after repair oftetralogy of Fallot: a 12-year prospective study. J Am Coll Cardiol.1994;23:1151–1155.

75. Jonsson H, Ivert T, Brodin LA, Jonasson R. Late sudden deaths afterrepair of tetralogy of Fallot: electrocardiographic findings associatedwith survival. Scand J Thorac Cardiovasc Surg. 1995;29:131–139.

76. Balaji S, Lau YR, Case CL, Gillette PC. QRS prolongation is associatedwith inducible ventricular tachycardia after repair of tetralogy of Fallot.Am J Cardiol. 1997;80:160–163.

77. Daliento L, Rizzoli G, Menti L, Baratella MC, Turrini P, Nava A, DallaVolta S. Accuracy of electrocardiographic and echocardiographicindices in predicting life threatening ventricular arrhythmias in patientsoperated for tetralogy of Fallot. Heart. 1999;81:650–655.

78. Lucron H, Marcon F, Bosser G, Lethor JP, Marie PY, Brembilla-PerrotB. Induction of sustained ventricular tachycardia after surgical repair oftetralogy of Fallot. Am J Cardiol. 1999;83:1369–1373.

79. Therrien J, Siu SC, Harris L, Dore A, Niwa K, Janousek J, WilliamsWG, Webb G, Gatzoulis MA. Impact of pulmonary valve replacementon arrhythmia propensity late after repair of tetralogy of Fallot. Circu-lation. 2001;103:2489–2494.

80. Hamada H, Terai M, Jibiki T, Nakamura T, Gatzoulis MA, Niwa K.Influence of early repair of tetralogy of Fallot without an outflow patchon late arrhythmias and sudden death: a 27-year follow-up study fol-lowing a uniform surgical approach. Cardiol Young. 2002;12:345–351.

81. Ghai A, Silversides C, Harris L, Webb GD, Siu SC, Therrien J. Leftventricular dysfunction is a risk factor for sudden cardiac death in adultslate after repair of tetralogy of Fallot. J Am Coll Cardiol. 2002;40:1675–1680.

82. Dore A, Santagata P, Dubuc M, Mercier LA. Implantable cardioverterdefibrillators in adults with congenital heart disease: a single centerexperience. Pacing Clin Electrophysiol. 2004;27:47–51.

83. Khairy P, Landzberg MJ, Gatzoulis MA, Lucron H, Lambert J, MarçonF, Walsh EP. Prognostic significance of electrophysiologic testing posttetralogy of Fallot repair: a multicenter study. Circulation. 2004;109:1994–2000.

84. Russo G, Folino AF, Mazzotti E, Rebellato L, Daliento L. Comparisonbetween QRS duration at standard ECG and signal-averaging ECG forarrhythmic risk stratification after surgical repair of tetralogy of Fallot.J Cardiovasc Electrophysiol. 2005;16:288–292.

85. Alexander ME, Walsh EP, Saul JP, Epstein MR, Triedman JK. Value ofprogrammed ventricular stimulation in patients with congenital heartdisease. J Cardiovasc Electrophysiol. 1999;10:1033–1044.

86. Goldner BG, Cooper R, Blau W, Cohen TJ. Radiofrequency catheterablation as a primary therapy for treatment of ventricular tachycardia ina patient after repair of tetralogy of Fallot. Pacing Clin Electrophysiol.1994;17:1441–1446.

87. Burton ME, Leon AR. Radiofrequency catheter ablation of right ven-tricular outflow tract tachycardia late after complete repair of tetralogyof Fallot using the pace mapping technique. Pacing Clin Electrophysiol.1993;16:2319–2325.

88. Gonska BD, Cao K, Raab J, Eigster G, Kreuzer H. Radiofrequencycatheter ablation of right ventricular tachycardia late after repair ofcongenital heart defects. Circulation. 1996;94:1902–1908.

89. Morwood JG, Triedman JK, Berul CI, Khairy P, Alexander ME,Cecchin F, Walsh EP. Radiofrequency catheter ablation of ventriculartachycardia in children, and in young adults with congenital heartdisease. Heart Rhythm. 2004;1:301–308.

90. Mullen MP, VanPraagh R, Walsh EP. Development and anatomy of thecardiac conduction system. In: Walsh EP, Saul JP, Triedman JK, eds.Cardiac Arrhythmias in Children and Young Adults With CongenitalHeart Disease. Philadelphia, Pa: Lippincott Williams & Wilkins; 2001:93–111.

91. Manning PB, Mayer JE, Wernovsky G, Fishberger SB, Walsh EP.Staged operation to Fontan increases the incidence of sinoatrial nodedysfunction. J Thorac Cardiovasc Surg. 1996;111:833–840.

92. Gregoratos G, Abrams J, Epstein AE, Freedman RA, Hayes DL, HlatkyMA, Kerber RE, Naccarelli GV, Schoenfeld MH, Silka MJ, Winters SL.ACC/AHA/NASPE 2002 guideline update for implantation of cardiacpacemakers and antiarrhythmia devices. J Am Coll Cardiol. 2002;40:1703–1719.

93. VanPraagh R, Papagiannis J, Grunenfelder J, Bartram U, Martanovic P.Pathologic anatomy of corrected transposition of the great arteries:medical and surgical implications. Am Heart J. 1998;135:772–785.

94. Anderson RH, Becker AE, Arnold R, Wilkinson JL. The conductingtissues in congenitally corrected transposition. Circulation. 1974;50:911–923.

95. Thiene G, Wenick ACG, Frescura C, Wilkinson JL, Gallucci V, Ho SY,Mazzucco A, Anderson RH. Surgical anatomy and pathology of the




ownloaded from


conduction tissues in atrioventricular defects. J Thorac CardiovascSurg. 1981;82:928–937.

96. Huhta JC, Maloney JD, Ritter DG, Ilstrup DM, Feldt RH. Completeatrioventricular block in patients with atrioventricular discordance. Cir-culation. 1983;67:1374–1377.

97. Connelly MS, Liu PP, Williams WG, Webb GD, Robertson P,McLaughlin PR. Congenitally corrected transposition of the greatarteries in the adult: functional status and complications. J Am CollCardiol. 1996;27:1238–1243.

98. Dick M, Norwood WI, Chipman C, Castaneda AR. Intraoperative re-cording of specialized atrioventricular conduction tissue electrograms in47 patients. Circulation. 1979;59:150–160.

99. Cohen MI, Rhodes LA, Spray TL, Gaynor JW. Efficacy of prophylacticepicardial pacing leads in children and young adults. Ann Thorac Surg.2004;1:197–202.

100. Dore A, Santagata P, Dubuc M, Mercier LA. Implantable cardioverterdefibrillators in adults with congenital heart disease: a single centerexperience. Pacing Clin Electrophysiol. 2004;1:47–51.

101. Dubin AM, Janousek J, Rhee E, Strieper MJ, Cecchin F, Law IH,Shannon KM, Temple J, Rosenthal E, Zimmerman FJ, Davis A,Karpawich PP, Al Ahmad A, Vetter VL, Kertesz NJ, Shah M, Snyder C,Stephenson E, Emmel M, Sanatani S, Kanter R, Batra A, Collins KK.Resynchronization therapy in pediatric and congenital heart diseasepatients: an international multicenter study. J Am Coll Cardiol. 2005;46:2277–2283.

102. Minniti S, Visentini S, Procacci C. Congenital anomalies of the venacavae: embryological origin, imaging features and report of three newvariants. Eur Radiol. 2002;8:2040–2055.

103. Ratliff HL, Yousufuddin M, Lieving WR, Watson BE, Malas A,Rosencrance G, McCowan RJ. Persistent left superior vena cava: casereports and clinical implications. Int J Cardiol. 2006;113:242–246.

104. Khairy P, Triedman JK, Juraszek A, Cecchin F. Inability to cannulatethe coronary sinus in patients with supraventricular arrhythmias: con-genital and acquired coronary sinus atresia. J Interv Card Electro-physiol. 2005;2:123–127.

105. Spittell PC, Hayes DL. Venous complications after insertion of a trans-venous pacemaker. Mayo Clin Proc. 1992;67:258–265.

106. Bracke F, Meijer A, van Gelder B. Venous occlusion of the access veinin patients referred for lead extraction: influence of patient and leadcharacteristics. Pacing Clin Electrophysiol. 2003;26:1649–1652.

107. van Rooden CJ, Molhoek SG, Rosendaal FR, Schalij MJ, Meinders AE,Huisman MV. Incidence and risk factors of early venous thrombosisassociated with permanent pacemaker leads. J Cardiovasc Electro-physiol. 2004;15:1258–1262.

108. McCotter CJ, Angle JF, Prudente LA, Mounsey JP, Ferguson JD,DiMarco JP, Hummel JP, Mangrum JM. Placement of transvenous

pacemaker and ICD leads across total chronic occlusions. Pacing ClinElectrophysiol. 2005;28:921–925.

109. Shah MJ, Nehgme R, Carboni M, Murphy JD. Endocardial atrial pacinglead implantation and midterm follow-up in young patients with sinusnode dysfunction after the Fontan procedure. Pacing Clin Electro-physiol. 2004;27:949–954.

110. Hansky B, Blanz U, Peuster M, Gueldner H, Sandica E, Crespo-Martinez E, Mathies W, Meyer H, Koerfer R. Endocardial pacing afterFontan-type procedures. Pacing Clin Electrophysiol. 2005;28:140–148.

111. Adwani SS, Sreeram N, DeGiovanni JV. Percutaneous transhepatic dualchamber pacing in children with Fontan circulation. Heart. 1997;77:574–575.

112. Ermis C, Zadeii G, Gupta M, Benditt DG. Trans-aortic His bundleablation with permanent ventricular pacing via the coronary sinus inL-transposition of great arteries with classic Fontan procedure. J IntervCard Electrophysiol. 2002;3:257–260.

113. Khairy P, Landzberg MJ, Lambert J, O’Donnell CP. Long-termoutcomes after the atrial switch for surgical correction of transposition:a meta-analysis comparing the Mustard and Senning procedures.Cardiol Young. 2004;14:284–292.

114. Van Gelder BM, Bracke FA, Oto A, Yildirir A, Haas PC, Seger JJ,Stainback RF, Botman KJ, Meijer A. Diagnosis and management ofinadvertently placed pacing and ICD leads in the left ventricle: a mul-ticenter experience and review of the literature. Pacing Clin Electro-physiol. 2000;23:877–883.

115. Weber HS. Incidence and predictors for the development of significantsupradiaphragmatic decompressing venous collateral channels followingcreation of Fontan physiology. Cardiol Young. 2001;11:289–294.

116. Sugiyama H, Yoo SJ, Williams W, Benson LN. Characterization andtreatment of systemic venous to pulmonary venous collaterals seen afterthe Fontan operation. Cardiol Young. 2003;13:424–430.

117. Knauth AL, Lock JE, Perry SB, McElhinney DB, Gauvreau K,Landzberg MJ, Rome JJ, Hellenbrand WE, Ruiz CE, Jenkins KJ. Trans-catheter device closure of congenital and postoperative residual ventric-ular septal defects. Circulation. 2004;110:501–507.

118. Khairy P, O’Donnell CP, Landzberg MJ. Transcatheter closure versusmedical therapy of patent foramen ovale and presumed paradoxicalthromboemboli: a systematic review. Ann Intern Med. 2003;139:753–760.

119. Stephenson EA, Batra AS, Knilans TK, Gow RM, Gradaus R, Balaji S,Dubin AM, Rhee EK, Ro PS, Thogersen AM, Cecchin F, Triedman JK,Walsh EP, Berul CI. A multicenter experience with novel implantablecardioverter defibrillator configurations in the pediatric and congenitalheart disease population. J Cardiovasc Electrophysiol. 2006;17:41–46.

KEY WORDS: ablation � atrial flutter � heart defects, congenital� arrhythmia � pacemakers � electrophysiology � pediatrics




ownloaded from


Edward P. Walsh and Frank CecchinArrhythmias in Adult Patients With Congenital Heart Disease

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 2007 American Heart Association, Inc. All rights reserved.

is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231Circulation doi: 10.1161/CIRCULATIONAHA.105.592410

2007;115:534-545Circulation.

http://circ.ahajournals.org/content/115/4/534World Wide Web at:

The online version of this article, along with updated information and services, is located on the

http://circ.ahajournals.org//subscriptions/

is online at: Circulation Information about subscribing to Subscriptions:

http://www.lww.com/reprints Information about reprints can be found online at: Reprints:

document. Permissions and Rights Question and Answer this process is available in the

click Request Permissions in the middle column of the Web page under Services. Further information aboutOffice. Once the online version of the published article for which permission is being requested is located,

can be obtained via RightsLink, a service of the Copyright Clearance Center, not the EditorialCirculationin Requests for permissions to reproduce figures, tables, or portions of articles originally publishedPermissions:



ownloaded from

http://circ.ahajournals.org/content/115/4/534

http://www.ahajournals.org/site/rights/

http://www.lww.com/reprints

http://circ.ahajournals.org//subscriptions/


congenital heart disease for the adult cardiologist · y the time patients with congenital heart...

Documents