The population of patients with a systemic right ventricle (sRV) in biventricular circulation includes those who have undergone an atrial switch operation for destro-transposition of the great arteries (d-TGA) and those with congenitally corrected transposition of the great arteries (ccTGA). Despite the life expectancy of these patients is significantly increased, the long-term prognosis remains suboptimal due to late complications such as heart failure, arrhythmias, and premature death. These patients, therefore, need a close follow-up to early identify predictive factors of adverse outcomes and to implement all preventive therapeutic strategies. This review analyzes the late complications of adult patients with an sRV and TGA and clarifies which are risk factors for adverse prognosis and which are the therapeutic strategies that improve the long-term outcomes. For prognostic purposes, it is necessary to monitor sRV size and function, the tricuspid valve regurgitation, the functional class, the occurrence of syncope, the QRS duration, N-terminal pro B-type natriuretic peptide levels, and the development of arrhythmias. Furthermore, pregnancy should be discouraged in women with risk factors. Tricuspid valve replacement/repair, biventricular pacing, and implantable cardioverter defibrillator are the most important therapeutic strategies that have been shown, when used correctly, to improve long-term outcomes.

Advances in surgical techniques have led to the survival of most patients with congenital heart disease (CHD) up to their adulthood. During their lifetime, many of them develop atrial tachyarrhythmias due to atrial dilatation and scarring from surgical procedures. More complex defects and palliative repairs are linked to a higher incidence and earlier occurrence of arrhythmias. Atrial fibrillation (AF) is common in patients who have atrial septal defects repaired after age 55 and in patients with tetralogy of Fallot repaired after age 45. Patients with dextrotransposition of the great arteries who undergo Mustard or Senning atrial switch procedures have an increased risk of atrial flutter due to atrial baffle suture lines. Patients with Ebstein's anomaly are also prone to supraventricular tachycardias caused by accessory bypass tracts. Patients with a single ventricle who undergo Fontan palliation are at risk of developing persistent or permanent AF due to extreme atrial enlargement and hypertrophy. In addition, obtaining vascular access to the pulmonary venous atrium can present unique challenges during radiofrequency ablation for patients with a Fontan palliation. Patients with cyanotic CHD who develop AF have substantial morbidity because of limited hemodynamic reserve and a high viscosity state. Amiodarone is an effective therapy for patients with arrhythmias from CHD, but its use carries long-term risks for toxicity. Dofetilide and sotalol have good short-term effectiveness and are reasonable alternatives to amiodarone. Pulmonary vein isolation is associated with better outcomes in patients taking antiarrhythmic medications. Anticoagulants are challenging to prescribe for patients with CHD because of a lack of data that can be extrapolated to this patient population. Surgical ablation is the gold standard for invasive rhythm control in patients with CHD and should be considered at the time of surgical repair or revision of congenital heart defects. When possible, patients with complex CHD should be referred for care to an adult congenital heart disease center of excellence.

We describe the electrophysiological studies undertaken in four patients with atrioventricular nodal reentry tachycardia in the setting of concordant atrioventricular and discordant ventriculo-arterial connections (transposition). Radiofrequency ablation was attempted in three, all with success. Clear evidence of dual antegrade pathways through the atrioventricular node was present in only one of the four, but other characteristics of discrete fast and slow pathways into the atrioventricular node were present in all. Atrioventricular nodal reentry tachycardia was inducible in all. In the three patients in whom ablation was attempted, the application of radiofrequency energy to the low medial regions of the systemic venous atrium (morphologically left) consistently caused junctional accelerated rhythm, but these lesions were not successful in eliminating the tachycardia. Successful radiofrequency ablation required a retrograde approach to the region of the slow pathway in the pulmonary venous atrium (morphologically right).

Without intervention, babies born with transposed great arteries (TGA) are doomed to a rapid death. Jatene and coworkers deserve the credit for performing the first successful arterial switch operation (ASO) in a patient with TGA and ventricular septal defect (VSD) in 1975. Since then ASO has become the procedure of choice in most medical centers. This review article summarizes the historical aspects of arterial switch operation and assesses this procedure's outcomes.

To examine the relation between ventricular dysfunction and late clinical arrhythmia in adults who underwent the Mustard procedure for transposition of the great arteries.

Observational study based on periodic outpatient assessment of biventricular function.

Tertiary referral centre.

Analysis of data from 12 lead ECGs, echocardiography, exercise radionuclide ventriculography, and magnetic resonance imaging.

Clinical outcome and late onset clinical arrhythmia during follow up. ECG and ventricular function indices obtained before arrhythmia onset were used for analysis.

51 patients (mean (SD) age 25.7 (5.0) years) fulfilled entry criteria at a mean of 23.4 (4.0) years after the Mustard procedure. Late arrhythmia occurred in 11 (22%): sustained atrial flutter/fibrillation in 10, ventricular tachycardia in one. Compared with patients who remained arrhythmia free, patients with arrhythmia had longer QRS (129 (26) v 112 (16) ms, p = 0.01), greater QT dispersion (107 (28) v 51 (24) ms, p < 0.001), and increased ratio of right to left ventricular end diastolic diameter (2.4 (0.9) v 1.7 (0.7), p = 0.02), but no difference in wall thickness. Systemic ejection fraction was also reduced in the arrhythmia subgroup (at rest: 34.1 (13)% v 47 (16)%, p = 0.04; during exercise: 37.8 (12)% v 52 (17)%, p = 0.03). QRS duration correlated with right ventricular end diastolic diameter (r = 0.59, p < 0.001), suggesting a possible mechano-electric relation after the Mustard procedure. QT dispersion was the only predictor of clinical arrhythmia in multivariate analysis.

Impaired ventricular function in adults with the Mustard procedure for transposition of the great arteries relates to clinical arrhythmia. Late atrial flutter/fibrillation may be a surrogate marker for ventricular dysfunction, and these patients may also be at risk of ventricular tachycardia.

The purpose of this study was to determine the efficacy and risks of radiofrequency ablation of various forms of supraventricular tachycardia after Mustard and Senning operations for d-transposition of the great arteries.

In this patient group, the reported success rate of catheter ablation of intraatrial reentry tachycardia is about 70% with a negligible complication rate. There are no reports of the use of radiofrequency ablation to treat other types of supraventricular tachycardia.

Standard diagnostic criteria were used to determine supraventricular tachycardia type. Appropriate sites for attempted ablation included 1) intraatrial reentry tachycardia: presence of concealed entrainment with a postpacing interval similar to tachycardia cycle length; 2) focal atrial tachycardia: a P-A interval < or =-20 ms; and 3) typical variety of atrioventricular (AV) node reentry tachycardia: combined electrographic and radiographic features.

Nine Mustard and two Senning patients underwent 13 studies to successfully ablate all supraventricular tachycardia substrates in eight (73%) patients. Eight of eleven (73%) patients having intraatrial reentry tachycardia, 3/3 having typical AV node reentry tachycardia, and 2/2 having focal atrial reentry tachycardia were successfully ablated. Among five patients having intraatrial reentry tachycardia (IART) and not having ventriculoatrial (V-A) conduction, two suffered high-grade AV block when ablation of the systemic venous portion of the medial tricuspid valve/inferior vena cava isthmus was attempted.

Radiofrequency catheter ablation can be effectively and safely performed for certain supraventricular tachycardia types in addition to intraatrial reentry. A novel catheter course is required for slow pathway modification. High-grade AV block is a potential risk of lesions placed in the systemic venous medial isthmus.

In patients with congenital heart disease who have undergone palliative surgical interventions postoperative arrhythmias frequently complicate the clinical course. Intraatrial reentrant tachycardias (IARTs) are one of the most common forms of postoperative arrhythmias in these patients and can lead to significant morbidity and even mortality. Drug therapy and/or antitachycardia pacing have been disappointing. Ablative therapy with radiofrequency energy offers a potential for cure for these patients but the conventional approach using multielectrode recordings and fluoroscopic guidance is technically difficult and provides limited success. Recent development of a novel nonfluoroscopic technology with electroanatomical mapping using the CARTO mapping/ablation system has shown promising results in defining the arrhythmia circuit, facilitating diagnosis, and guiding ablative therapy. Based on our preliminary experience, a systematic approach to postoperative IART using electroanatomical mapping is described. Further studies are needed to fully evaluate the impact of this new technology on the management and therapy of IART.

After surgery for complex congenital heart disease, clinically important atrial tachyarrhythmias have a higher than normal incidence if sufficiently large regions of conduction block occur within the atria, especially in the presence of hemodynamic alterations. Sinus bradycardia may result from direct damage to sinus node and its blood supply. Historical data have identified patients who have undergone the Mustard or Senning operations for dextrotransposition of the great vessels and the Fontan operation in cases of functional single ventricle as being at great risk for atrial tachyarrhythmias. These arrhythmias are especially poorly tolerated when there are co-existing hemodynamic alterations and are an important source of morbidity and mortality. Until recently, treatment strategies have been limited to antiarrhythmic drugs, bradycardia pacing, and--in suitable patients--antitachycardia pacing, often in combination. Amiodarone has been the most efficacious drug, but has only been of moderate value because of extracardiac side effects. Radiofrequency ablation of the atrial regions critical to reentrant circuits, which was discovered to be of value in patients with atrial flutter and a normal heart is being applied to this diverse group of patients. Early results are promising, but the Fontan operation patients are especially challenging because of early recurrences of apparently new reentrant circuits. Progress in this area will likely come from newer surgical techniques that prevent the milieu for atrial reentry and from multidimensional mapping systems for our current patients.

To assess the clinical, functional state, and complications late (15-20 years) after palliative Mustard operation.

Examination and evaluation of all patients presenting in adolescence and adult life after palliative Mustard operation for transposition of the great arteries and pulmonary vascular disease.

Grown-up Congenital Heart Unit specialised in the care of adolescents and adults with congenital heart disease, designated as having "quaternary" status within a tertiary referral centre for cardiac diseases.

Database searched for patients referred after palliative Mustard for classic transposition of the great arteries. Ten patients aged 18-31 (mean (SD) 25.9 (5.2)) years with a palliative Mustard operation performed at age 1.7-15 (mean (SD) 9 (4.6)) years were fully evaluated by echocardiography, exercise testing, Holter monitoring, and magnetic resonance imaging or radionuclide ventriculography, or both 15-20 years later.

One patient died aged 25 years with biventricular failure (ability index 3/4), haemoptysis, and atrial flutter, eight were well (ability index 2), and one disabled (ability index 3). Arterial oxygen saturation at rest was 85-98% (mean (SD) 93.8 (4)%) decreasing to 59-87% (mean 77 (9.5)%) after limited exercise. Symptomatic arrhythmias occurred in four patients, atrial flutter being the most common, and two had sinus node dysfunction. Significant tricuspid regurgitation occurred in four patients.

Patients with palliative Mustard operation have a low incidence of symptomatic ventricular dysfunction and despite exercise limitation by hypoxia, continue to live active, near normal lives until their thirties.

Atrial reentry tachycardia is common after surgical repair of congenital heart disease. The arrhythmia is often difficult to treat and is occasionally life-threatening. This study reports experience with atrial antitachycardia (AAIT mode) pacing for the management of atrial reentry tachycardia, with emphasis on the risks and benefits of automatic pacing therapy. Eighteen patients (2-32 years of age) with a variety of congenital heart lesions underwent atrial antitachycardia pacemaker placement for recurrent atrial tachycardia that was amenable to pace termination prior to the implantation procedure. An appropriate antitachycardia program was determined by repeated induction and termination of atrial tachycardia using the noninvasive programmed stimulation mode of the pacemaker. Over 4-30 months of follow-up, 6 patients had 189 episodes of tachycardia successfully converted with AAI-T pacing, 4 patients had 8 episodes of tachycardia detected but not successfully converted, and 8 patients had no episodes of tachycardia with antibradycardia pacing alone. The number of patients receiving pharmacological therapy other than digoxin or beta blockade fell from 12 to 6. Two subjects died suddenly, 1 while wearing a Holter monitor. In both, tachycardia was detected and pace cardioversion attempted.

Atrial antitachycardia pacing is a useful tool in the management of patients with congenital heart disease and atrial arrhythmias; however, in selected cases, it may not prevent and may even exacerbate the lethal complications of the tachycardia. Antitachycardia function evaluation is recommended under varying levels of autonomic stress prior to institution of automatic therapy.

Intraatrial baffling procedures such as the Mustard or Senning repair of transposition of the great arteries have been associated with a high incidence of cardiac arrhythmias. These abnormalities are thought to arise from trauma to the sinus node and atrial muscle during the procedure. In the arterial switch operation, there is little intraatrial manipulation other than the repair of the atrial septal defect. In theory, rhythm disturbances after the arterial switch operation should be less prevalent. From January 1, 1983, to December 31, 1990, 390 patients (230 with intact ventricular septum and 160 with a coexisting ventricular septal defect) underwent an arterial switch operation. Electrocardiograms and 24-hour Holter monitor studies were obtained in the 364 survivors at hospital discharge and during follow-up. Limited intracardiac electrophysiologic studies were performed 6 to 12 months after the operation.

Atrioventricular node function was preserved in most patients; seven patients (2%) had first-degree, two (0.7%) second-degree, and five (1.7%) had complete atrioventricular block (all with coexisting ventricular septal defect). All five patients with complete heart block received a permanent pacemaker. In those patients not having a permanent pacemaker, sinus rhythm was present in 96% on the surface electrocardiogram and 99% during 24-hour Holter monitor studies (1 month to 8.5 years, mean 2.1 years after the operation). Intracardiac electrophysiologic studies (n = 158) demonstrated normal corrected sinus node recovery times and AH intervals in 97% of patients. Atrial ectopy was present in 152 of 172 (81%) patients, with the majority (64%) of patients having only occasional premature beats without repetitive forms. Ventricular ectopy was a frequent finding during 24-hour monitoring. At hospital discharge 70% had ventricular ectopy; these values fell to 57% (in patients with intact ventricular septum) and 30% (in patients with a coexisting ventricular septal defect) at follow-up. In the early postoperative period, there were 25 episodes of supraventricular tachycardia (14 of which required therapy), 6 episodes of junctional ectopic tachycardia, and 9 episodes of ventricular tachycardia. The incidence of supraventricular tachycardia had fallen to 5% at follow-up, with no atrial flutter or fibrillation noted. Three patients had ventricular tachycardia on follow-up Holter studies. In summary, our results confirm the theoretical advantages of anatomic correction over atrial level correction of transposition of the great arteries with respect to preservation of sinus node function and low incidence of clinically significant tachyarrhythmias.

The purpose of this study was to define the incidence and risk factors for atrial tachyarrhythmias after the Fontan operation.

Atrial tachyarrhythmias cause morbidity after the Fontan operation. Causative factors may be affected by the type of systemic to pulmonary connection.

The Fontan operation was performed in 270 consecutive patients between 1982 and 1992. The mean age at operation was 7.0 +/- 4.3 years. Direct atriopulmonary connection was used in 138 patients (51%), total cavopulmonary connection in 94 (35%) and right atrial to right ventricular connection in 38 (14%).

Atrial tachyarrhythmias were seen early postoperatively in 55 patients (20%), preoperative atrial tachyarrhythmia being the only risk factor. Follow-up was achieved for 228 early survivors (97%) at a mean interval of 4.4 years. There were 20 late deaths. Late atrial tachyarrhythmias were noted in 29% of patients who received an atriopulmonary connection, 14% of those who received a total cavopulmonary connection and 18% of those who received a right ventricular connection (p < 0.02). Significant risk factors as determined by univariate and multiple logistic regression analysis were atriopulmonary connection type (odds ratio 0.40 for total cavopulmonary relative to atriopulmonary connection [p < 0.05] and 0.37 for right ventricular relative to atriopulmonary connection [p = 0.08]), longer follow-up interval (odds ratio 1.32 for each consecutive year [p < 0.002]) and atrial tachyarrhythmia in the operative period (odds ratio 6.31 [p < 0.0001]).

Early postoperative atrial tachyarrhythmias, length of follow-up and atriopulmonary connection are significant independent risk factors for the presence of late atrial tachyarrhythmias.

Impairment of sinus rhythm has been reported after the Mustard procedure for complete transposition. We observed two patients who developed severe bradycardia during or after exercise. The first had a 2.8 s sinus pause in the recovery period, the second had a drop in heart rate during exercise. Both eventually had a pacemaker implanted. Exercise testing can be helpful in detecting sick sinus syndrome.

We studied 60 patients who had survived the Mustard procedure for transposition of the great arteries, performed between the ages of 2 days and 24 months (mean 4.51 +/- 3.79). All patients were given a postoperative 24-hour dynamic electrocardiogram at 15 days, 1 year and 3 years and then every 2 years. Those who were found to have sinus node dysfunction during follow-up, were given a dynamic electrocardiogram every 3-6 months. The average follow-up period was 38.7 +/- 19.8 months, median 36. Sinus node dysfunction was detected during follow-up in 20 patients (33.3% of the total), 8 of whom had had a pacemaker inserted. Risk factors for late development of sinus node dysfunction were found to include prolonged cross-clamping of the aorta during surgery (P = 0.003), especially over 50 minutes (relative risk 3.5:1), and the presence of even transient sinus node dysfunction after surgery (P = 0.006). These observations suggest, first, that sinus node dysfunction may develop after the Mustard operation as a combined effect of extensive atrial surgery and a long period of myocardial ischemia and, second, that the presence of the disease immediately after the operation sometimes indicates that it will recur or persist during follow-up.

Esophageal electrocardiography (EsECG) is a useful adjunct in the diagnosis of dysrhythmias that are difficult to diagnose with a conventional ECG. This study was designed to evaluate which type of dysrhythmias required the EsECG for proper diagnosis and what factors produced the rhythm problems. Sixty-eight pediatric patients undergoing cardiac surgery were studied. After release of the aortic cross-clamp, the cardiac rhythm was analyzed by a standard limb-lead ECG at five-minute intervals. Twenty-six of 68 patients exhibited rhythms during reperfusion that required the EsECG for definitive diagnosis. This group of patients was younger, had longer durations of cardiopulmonary bypass and aortic occlusion, and required more time and interventions to achieve a normal sinus rhythm. The likelihood of difficult dysrhythmias was not related to the type of surgical procedure performed.

Electrophysiologic studies were performed at 12.7 +/- 4 months postoperatively in 20 patients who had the arterial switch repair at 1 to 120 days (mean 13 +/- 26 [SD]) for d-transposition of the great arteries. Preoperative electrocardiograms (ECGs) at rest revealed an ectopic atrial rhythm in one patient. Postoperative rest ECGs revealed transient postoperative ectopic atrial or junctional rhythms in two patients, intermittent ectopic atrial rhythms in two and right bundle branch block in nine with a normal QRS axis. In addition, ambulatory monitor recordings revealed infrequent premature ventricular complexes in five patients. Catheter endocardial mapping revealed sinus rhythm in 18 patients and ectopic atrial rhythm in 1 patient. Atrial activation after the switch repair was comparable with normal atrial activation. Activation of all low atrial sites was significantly earlier after the switch repair than after the Mustard repair. Mild abnormalities of sinus node function were present and consisted of slight prolongation of corrected sinus node recovery time in six patients and slightly increased sinoatrial conduction time in four. The ratio of sinus node recovery time to sinus cycle length was normal in all 20 patients. Atrial effective and functional refractory periods were normal in all patients and no atrial arrhythmias could be induced by programmed stimulation. Atrioventricular (AV) node conduction was excellent with normal AV node effective and functional refractory periods. The only electrophysiologic abnormality of His-Purkinje function was distal right bundle branch block in nine patients. Programmed stimulation of the ventricle produced repetitive ventricular responses in four patients: bundle branch reentry in three and intraventricular reentry in one.(ABSTRACT TRUNCATED AT 250 WORDS)

Atrial flutter is a common postoperative arrhythmia in patients with complete transposition of the great arteries (d-TGA) after the Mustard repair. Sixty patients with d-TGA who had the Mustard repair were evaluated by electrophysiologic studies. Thirty-three (55%) had inducible sustained atrial flutter; 17 of them developed spontaneous clinical episodes of atrial flutter (clinical atrial flutter group) and 16 did not (nonclinical atrial flutter group). In 6 of the 17 patients (35%) with clinical inducible atrial flutter, the condition was first documented in the electrophysiologic laboratory with subsequent development of spontaneous clinical episodes. Catheter endocardial mapping, used to determine atrial activation sequences and and conduction intervals, revealed intraatrial conduction delays with late activation of the low atrial sites in all patients. Abnormalities of atrial refractoriness were present, with a greater dispersion of atrial refractoriness found in the clinical atrial flutter group. Severe abnormalities of sinus nodal function appeared to a significantly greater degree among patients who had clinical episodes of atrial flutter. These electrophysiologic abnormalities and associated arrhythmias may predispose patients with d-TGA to sudden death.

This study describes the electrophysiologic effects of the Mustard repair of d-transposition of the great arteries on the sinus node and on conduction and refractoriness in the atrium, atrioventricular (AV) node and ventricle. Seventy-two electrophysiologic studies were performed on 64 patients after the Mustard operation. Standard rest and 24 hour ambulatory electrocardiograms were evaluated. Catheter endocardial atrial mapping, available in 67 of the 72 studies, revealed that sinus rhythm was present in the atria in 33 patients, ectopic atrial rhythm in 26 and junctional rhythm in 8. Intraatrial conduction delays with very late activation of the low medial, low lateral or low right atrium at the AV junction were present in 53 of 59 patients with atrial or sinus rhythm. Sinus node function was normal in only nine patients. Prolonged refractory periods were found in the right atrial portion of the new right atrium when compared with the left atrial portion of the new right atrium. Forty-one percent of the patients had prolongation of atrial refractoriness with shortening of the paced cycle lengths. Fifty-one percent developed sustained intraatrial reentry with programmed atrial stimulation. Forty-eight percent of these patients with inducible atrial tachycardia have since developed clinical episodes of atrial flutter. In summary, electrophysiologic studies revealed significant abnormalities of sinus node function and atrial conduction and refractoriness. Alone or in combination, these abnormalities, which result in severe bradycardia or rapid atrial arrhythmias, may lead to sudden death. The Jatene or arterial switch procedure may provide an appropriate alternative operation in this group of patients.

A selected group of 18 patients aged 15-27 years with transposition of the great arteries and a previous Mustard procedure were evaluated to determine their functional ability and clinical state. Arrhythmias were common, occurring at some time in 16/18 (89%). Arrhythmia was serious in four; two of them required pacing and two had cardiac arrests, one resulting in death. Seven (41%) had right ventricular dysfunction; this was progressive in three. Tricuspid regurgitation was present in seven (41%); it occurred in patients with normal and reduced right ventricular ejection fractions. Regurgitation became progressively worse as the right ventricle dilated. Left ventricular function was well preserved in most patients. Fourteen (82%) of this pioneer group were leading normal lives (ability index 1 or 2). Although these results are acceptable concern remains about the probability of deteriorating right ventricular function.

Between 1967 and 1976, 106 children with transposition of the great arteries (TGA) (55 simple, 51 complex) survived the Mustard procedure. Late death occurred in 8 patients (1 simple, 7 complex TGA). Cardiac arrhythmia developed in 31 patients, 6 of whom required a permanent pacemaker. Postoperative cardiac catheterization showed mild superior vena cava obstruction in 4 patients, mild pulmonary venous obstruction in 3, and baffle leak in 4. Only 1 of these patients underwent reoperation elsewhere for a baffle leak. Two other patients had reoperation for subpulmonary stenosis and 1, for tricuspid regurgitation. The actuarial survival at 18 years is 92 +/- 2.3%, and the event-free survival is 83 +/- 3.8% (95% confidence limits). Eighty-seven patients are in New York Heart Association Functional Class I, and 3 are in Class II. The results of this study show that the long-term survival and event-free survival have been satisfactory. Late death was significantly higher in patients with complex TGA (p = .027). Postoperative arrhythmia was common, but only 6 patients required permanent pacemakers and the incidence of late complications and reoperation has been low.

Transesophageal study was used for diagnosis and treatment of 51 episodes of tachycardia in 13 patients with complete transposition of the great arteries who had undergone atrial baffle procedure. At the time of atrial baffle procedure, patients were 6 to 36 months old (mean 23). Tachycardia (1 to 17 episodes per patient) first occurred 1 to 23 days (4 patients) or 1.8 to 12 years (9 patients) after atrial baffle. Transesophageal study was performed using a bipolar silicone rubber-coated catheter. Tachycardia conversion was accomplished with stimulation bursts using 4 to 10 stimuli 9.9 ms in duration at 20 to 28 mA and an interstimulus interval of 50 to 100 ms less than the atrial cycle length. All tachycardia episodes had regular atrial cycle lengths ranging from 200 to 350 ms. In 12 patients, second-degree atrioventricular (AV) block was observed during tachycardia, suggesting primary atrial tachycardia. However, in 1 patient, occurrence of AV block always resulted in tachycardia termination, suggesting the presence of AV reentrant tachycardia. Transesophageal stimulation converted 48 of 51 tachycardia episodes to sinus/junctional rhythm. Ten tachycardia episodes in 6 patients were transiently converted to atrial fibrillation lasting 3 seconds to 28 minutes before spontaneous conversion to sinus junctional rhythm. Conversion attempts were unsuccessful on 3 occasions. Acceleration of ventricular rate after stimulation necessitated DC cardioversion on 1 occasion. Conversion was not achieved in 2 tachycardia episodes using stimuli less than 10 mA. Transesophageal study is a safe and effective minimally invasive technique for diagnosis and treatment of tachycardia in infants and children who have had atrial baffle for transposition of the great arteries.

Since 1983 we have performed electrophysiologic studies in 6 patients who had previously undergone repair of an ostium primum atrioventricular septal defect. Information obtained during electrophysiologic studies was crucial in guiding appropriate pacemaker therapy in these patients. As judged from the resting electrocardiogram, sinus or junctional bradycardia was present in 3/6, atrial flutter / fibrillation in 2/6, and paced rhythm in 2 patients who had had ventricular pacemakers implanted for complete atrioventricular block. During maximal exercise testing 4 patients had reduced heart rates; 2 had sudden drops in heart rate at 1 min postexercise; 1 patient had exercise induced ventricular bigeminy; and 1 patient with atrial flutter and 2: 1-4: 1 block at rest developed 1: 1 conduction during Stage II with an effective ventricular rate of 220/min. During electrophysiologic studies, the maximum corrected sinus node recovery time was abnormal in five of the six, ranging from 410 to 5630 msec. There was no spontaneous atrial rhythm in the other patient. Complete atrioventricular block was present in 2 patients while the atrioventricular Wenckebach phenomenon occurred abnormally at atrial pacing cycle lengths greater than 450 msec in 2 others. Supraventricular tachycardia or atrial flutter/fibrillation, was either spontaneous or induced in 2/6 patients, while ventricular tachycardia was induced in 1/3 patients who underwent programmed ventricular stimulation. Electrophysiologic studies were important in unmasking severe sinus node disease in 3 patients and atrioventricular node disease in 2. We therefore recommend that electrophysiologic studies be strongly considered as part of the evaluation of conduction abnormalities following repair of ostium primum atrioventricular septal defect.

Sudden death may occur in children with supraventricular arrhythmias. Sick sinus syndrome, particularly if associated with tachycardia, may result in sudden death in children who have had open heart surgery and rarely in children with a normal heart. Children with supraventricular tachycardia rarely die. Only those with junctional automatic tachycardia or Wolff-Parkinson-White syndrome have died. Patients with a short anterograde refractory period may be at risk of sudden death. Surgical division of the accessory connection can prevent sudden death. Digitalis may accelerate atrioventricular (AV) conduction in patients with Wolff-Parkinson-White syndrome and, thus, should be used only after testing in the electrophysiology laboratory. Sudden death due to complete AV block should be preventable using pacemakers. Neonates with a ventricular rate less than 55 beats/min or children with a rate less than 45 beats/min should receive pacemaker therapy because of the statistical probability of death or syncope. Ventricular ectopic beats, particularly if frequent or multiform, may be an indication for pacemaker insertion. Patients with surgical complete AV block that persists for more than 7 to 10 days should receive physiologic pacemakers for the prevention of sudden death and hemodynamic benefit.

Earlier reports have suggested that the incidence of dysrhythmias after the Mustard procedure can be reduced if the sinoatrial node (SAN) is protected during surgery. To determine if these initial differences continue after longer follow-up, we examined all ECGs available for three groups of patients operated upon from January, 1965, through December, 1977. Group A included 37 patients who survived the operation prior to January, 1972, when surgical modifications were initiated to protect the SAN; group B included 44 patients available for follow-up who were operated upon from 1972 through 1974; and group C consisted of the 39 patients available for follow-up operated upon from 1975 to 1977. Dysrhythmias were classified as passive (failure of initiation or propagation of the SAN impulse), active (atrial flutter or supraventricular tachycardia), or atrioventricular (AV) conduction defects. Results were expressed as the incidence per number of different rhythms during follow-up intervals. The incidence of sinus rhythm in groups B and C (80%) was much greater than in group A (27%) during the first 2 years. However, after 8 years, less than 50% of the rhythms were sinus. Both brady- and tachydysrhythmias were common. Seven patients (6%) required pacemaker insertion for symptomatic sick sinus syndrome. Therefore despite efforts to protect the sinus node, late occurring dysrhythmias remain a significant problem in the postoperative Mustard patient.

The clinical, investigative, and surgical findings were reviewed in 47 patients with atrioventricular discordance who presented to the Brompton Hospital between January 1962 and June 1981. Although the unifying feature was the atria connecting to morphologically inappropriate ventricles, the hearts differed widely in other respects. In most cases there was the usual visceral and atrial arrangement, but six had a mirror image arrangement of the atria and viscera. Among those patients with usual atrial arrangement (solitus) the aorta was not always anterior and left sided, and ventricular "inversion" was not invariable. Only 35 of the 47 patients also had a discordant ventriculoarterial connexion, the majority of the remainder having a double outlet right ventricle. The specific diagnosis of atrioventricular discordance depended on echocardiographic and angiographic examination. The other investigations did not distinguish between different ventriculoarterial connexions and were not specific even for the presence of atrioventricular discordance. For those patients with the usual atrial arrangement the anticipated right heart border was present in only just over one third, and the reversal of Q wave progression in the precordial leads of the standard electrocardiogram was found in under a half. Many patients with atrioventricular discordance progressed normally to adult life. In 20 cases no surgery was performed. The results of total correction showed a mortality of 25% (three cases), including two deaths after a modified Fontan procedure for exceedingly complex associated lesions. The results of surgery in the survivors were excellent, and awareness of the disposition of the atrioventricular conduction tissue made it possible to avoid atrioventricular dissociation.

We compared several electrophysiological variables before and after Mustard and Senning procedures in 14 mongrel dogs and made the following three observations. First, after the Senning operation, the atrial activation in the areas of the anterior and middle internodal tracts is undamaged. After the Mustard procedure, however, conduction through the interatrial septum is practically abolished. Both techniques damage the area of the posterior internodal tract. On the whole, there is greater dispersion of atrial epicardial mapping with the Senning operation. Second, several degrees of depression in the sinus node automatism and intraatrial conduction were observed with both techniques. Third, atrioventricular block in various degrees was observed only after the Mustard procedure. The atrioventricular dissociation observed after the Senning operation was dependent on sinus pacemaker dysfunction only. We think that from the electrophysiological point of view, the Senning procedure is a valid alternative for repair of transposition of the great arteries.

To assess pre- and postoperative arrhythmias associated with anatomic correction of transposition of the great arteries, 34 patients had 145 standard electrocardiograms (ECGs) and 24 of these patients had 46 24-hour ECGs from 11 months before to 54 months after anatomic repair. Twenty-two patients underwent balloon atrial septostomy and 7 surgical atrial septectomy before the initial 24-hour ECG. Anatomic correction included repair of a large ventricular septal defect in 16 patients and an aortopulmonary window in 1 patient. The preoperative standard ECG showed sinus rhythm in every patient. Preoperative 24-hour tapes revealed sinus arrhythmias in 2, sinus bradycardia at a rate of less than or equal to 50 beats/min in 1, junctional rhythm in 1 and rare premature ventricular contractions (PVCs) in 1. The postoperative 24-hour ECG showed atrioventricular dissociation in 1 patient, rare premature atrial contractions in 7, rare PVCs in 8 and sinus bradycardia with junctional escape in 1. Eight patients had PVCs on the postoperative ECG that were not noted on preoperative tapes. No patient had prolonged bradycardia (less than or equal to 50 beats/min), life-threatening arrhythmias or sudden death. Except for 1 patient with atrioventricular dissociation believed to be secondary to a preventable cause, 34 patients followed for 890 patient-months after anatomic correction for transposition of the great arteries had no significant arrhythmias.

This study reports the 2-dimensional echocardiographic appearance of the caval and pulmonary venous pathways after the Senning procedure in 28 patients and establishes normal values for the caval and pulmonary venous pathway dimensions. Eighteen patients had no caval or pulmonary venous obstruction or tricuspid regurgitation at catheterization; 2 had isolated superior vena caval obstruction, 3 had isolated pulmonary venous obstruction, 4 patients had severe tricuspid regurgitation, and 1 had a large residual ventricular septal defect. The caval and pulmonary venous pathways were imaged in modified 4-chamber and transverse views, and the narrowest dimension of each pathway in each view was measured by 2 independent observers. Dimension measurements were then normalized to the cube root of body surface area. Caval and pulmonary venous pathway "dimension products" were obtained by multiplying the normalized dimension in the 4-chamber view by the normalized dimension in the transverse view. All patients with catheterization-proven caval or pulmonary venous obstruction or tricuspid regurgitation had caval or pulmonary venous pathway dimension products outside the normal range, defined by our measurements in the 18 patients with no caval or pulmonary venous obstruction or tricuspid regurgitation. Thus, 2-dimensional echocardiography can provide both quantitative and qualitative information about the caval and pulmonary venous pathways after the Senning procedure.

A retrospective study was done in 50 patients after Mustard's operation (group A) and in 204 patients after closure of a secundum atrial septal defect (group B) to analyze the postoperative dysrhythmias and to relate them to surgical factors. Forty-two percent of the patients in group A had dysrhythmias at the end of the follow-up, compared to 23% of group B patients. There was a high late mortality in group A (16%) significantly related to AF and AVJ. In group A a significant correlation was found between dysrhythmias and age at operation, use of cardioplegia, perfusion time, and the type of cannulation. In group B there was a significant relation between the location of the defect and the presence of abnormal pulmonary venous drainage. After ASD closure using hypothermia instead of cardiopulmonary bypass, the incidence of dysrhythmias was significant lower. Damage to the sinus node by cannulation and by suturing in the sinus node area is the main cause of the high incidence of dysrhythmias after atrial surgery. This high incidence should be a factor in the consideration of new types of operations.

Pre- and postoperative cardiac catheterization data and cinenangiocardiograms of 82 patients who survived the Mustard operation for transposition of the great arteries (TGA) were reviewed. The post-operative catheterizations were performed 20 days to 10 years after operation (mean 2.5 years). Forty-six patients (56%) had no or insignificant associated cardiac lesions, whereas 36 (44%) had ventricular septal defect, pulmonary stenosis, or both, and required surgical intervention at the time of the Mustard operation. Postoperatively, 11 patients (13%) had significant systemic venous obstruction. Of the 11 patients, 6 required reoperation, and 2 patients had evidence of restenosis or complete obstruction in the superior vena cava after reoperation. In most patients, superior vena caval obstruction was well tolerated even in the presence of high pressure in the superior vena cava. Pulmonary venous obstruction occurred in 5 patients (6%), 3 of whom had no clinical symptoms despite severe pulmonary venous obstruction, although all had radiographic evidence of pulmonary venous congestion. The incidence of obstruction was drastically reduced after the Mustard operation was modified to include routine enlargement of the pulmonary venous atrium. Tricuspid regurgitation was uncommon (10%), but did occur in patients who had transatrial closure of a ventricular septal defect. Preoperatively, left ventricular outflow obstruction occurred in 38%. In 12 patients an attempt was made to relieve the obstruction at surgery. The 6 patients who had localized obstruction had a good result, but patients with more diffuse narrowing of left ventricular outflow had little or no relief of obstruction. Mild to moderate left ventricular outflow gradients regressed spontaneously in most patients after the Mustard operation.

Disorders of rhythm or conduction in patients with transposition of the great arteries (TGA) after the Mustard operation have been widely reported. This study provides a systematic evaluation of the electrophysiologic function of 87 survivors of the Mustard operation at a single institution. Surface electrocardiograms were reviewed in all 87 patients, Holter monitoring data in 26 patients, exercise electrocardiograms in 21 patients, and invasive electrophysiologic data in 61 patients. Surface electrocardiograms showed normal sinus rhythm in 52%, sinus node dysfunction in 27%, and atrioventricular block in 16%. Holter monitoring was obtained in an unselected subgroup of 26 patients who had a mean age of 12 years and a mean interval from operation of 9 years. Sinus node dysfunction was found in 58%, atrioventricular block in 27% ventricular ectopy in 50%, supraventricular ectopy in 27%, and no abnormalities in only 8%. Intracardiac electrophysiologic evaluation showed a high frequency of abnormal sinus node recovery times and suboptimal response of the atrioventricular-conduction system to rapid atrial pacing. When all modalities used in this study were considered, sinus node dysfunction occurred in 47%, ectopy in 34% and atrioventricular block in 23%. Although only 30% of patients had no evidence of arrhythmia, symptoms of rhythm or conduction disturbances were rare.

Corrected sinus node recovery time (CSRT) has been found unreliable in identifying all cases of sick sinus syndrome. Since other factors than sinus node dysfunction might add to the pathologic significance of the CSRT, we assessed it in 15 patients (nine group I patients with "prolonged" CSRT max = 3,196 + 2,740 msec and six group II patients with "short" CSRT max = 367 + 79 msec) at 0800, 1100, 1400, 1700, 2000, and 2300 hours with atrial overdrive stimulation rates (AST) of 90, 110, 140, 170, and 200 bpm on three consecutive days using a loop-mounted stable atrial electrode. Only with AST greater than or equal to 140 beats per minute (bpm) did all CSRTI values prove prolonged (greater than or equal to 560 msec). CSRTI values at corresponding time intervals were reproducible with AST greater than or equal to 140 bpm (day 1 vs 2 vs 3, P greater than .05), but not at AST 90 bpm and 110 bpm (P less than .05); CSRTII results, however, varied from day to day (less than .05) due to less scatter of single results. CSRTI results increased progressively with AST 90, 110, and 140 bpm from 301 + 256 msec by 60% for each pacing rate up to 785 + 848 msec. With AST greater than or equal to 140 bpm, the pattern of CSRT changes was inconsistent; this was also reflected by the distribution of the mean maxima of CSRTI: For 0800 hours at AST 140 bpm = 822 + 937 msec; for 1100 hours at AST 200 bpm = 824 + 1446 msec; for 1400 hours at AST 140 bpm = 780 + 814 msec; for 1700 hours at AST 170 bpm = 1,099 + 1,008 msec; for 2000 hours at AST 200 bpm = 1,156 + 1,280 msec; and for 2300 hours at AST 170 bpm = 1,021 + 1,102 msec. We conclude therefore that the optimal diagnostic yield for sick sinus syndrome testing is influenced by the time of the day and the AST used for CSRT testing.

Postoperative arrhythmias may occur in any patient who undergoes intracardiac surgery for a congenital heart defect. The correction of certain intracardiac heart defects predisposes to a large incidence of cardiac arrhythmias. Ventricular arrhythmias and conduction disturbances are seen after correction of tetralogy of Fallot, ventricular septal defect and atrioventricular canal defect. Supraventricular arrhythmias and sinus nodal dysfunction may be seen after surgery for transposition of the great arteries or atrial septal defect. The identification, evaluation and treatment of these patients are discussed.

During atrial extrastimulation, split His potentials, prolonged His-to-ventricular (H2V2) intervals and block distal to the His bundle deflection were observed in both preoperative and postoperative children with heart defects. His-Purkinje responses and refractoriness were identified in 31 of 78 (40%) pediatric patients (20 of 51 preoperative and 11 of 27 postoperative) during atrial extrastimulation coupled to sinus and/or paced cycle lengths. Split His potentials were found in 14 patients (eight preoperative and six postoperative) and His bundle relative refractory periods ranged from 250--490 msec. Prolonged H2V2 intervals were found in these and in an additional 16 patients (11 preoperative and five postoperative) and the relative refractory period of the His-Purkinje system ranged from 230--500 msec. Block distal to the His deflection occurred in seven patients (five preoperative and two postoperative) and the effective refractory period ranged from 230--510 msec. Split His potentials, long H2V2 intervals and block distal to the His bundle deflection produced by atrial extrastimulation were found in peroperative as well as postoperative children. These responses probably represent functional electrophysiologic characteristics of the pediatric cardiac conduction system.

Thirty patients with ventricular septal defect and d-transposition of the great arteries or double outlet right ventricle underwent placement of an intraventricular baffle to achieve physiologic correction. Eighteen of these patients also required an external conduit to establish continuity between the right ventricle and the pulmonary artery. There was an overall 22 percent early mortality rate, although the rate was 63 percent in patients who weighed less than 10 kg. There was an additional 20 percent late mortality rate, primarily related to conduction disturbances and pulmonary vascular obstructive disease, problems that have not occurred in patients operated on since 1974. Of the survivors, all are asymptomatic and as a group have achieved a significant weight gain. Many have residual hemodynamic abnormalities that required cardiac catheterization for precise assessment. Four patients have been reoperated on, with no additional mortality.

The clinical and electrocardiographic findings in five children with the sick sinus syndrome and an otherwise normal heart are described. There were three boys and two girls. Their age at onset of either bradycardia or symptoms ranged from 1 day to 7 years. In one patient, the youngest ever reported with this syndrome, bradycardia was noted before birth. Four children presented with neurological symptoms--attacks of dizziness, fainting spells, or syncope. One boy, treated for epilepsy before the underlying arrhythmia ws diagnosed, died suddenly while playing. One child had near-fatal syncope caused by ventricular tachycardia. Continuous 24-hour electrocardiographic monitoring is the best method of assessing the severity of the condition. Sinus bradycardia, sinuatrial block, and periods of sinus arrest up to 4.8 seconds were recorded. Two patients had associated atrioventricular block and were therefore presumed to have binodal disease. Atrial fibrillation or flutter occurred in three patients. Isolated sick sinus syndrome may be a life-threatening condition in childhood for which, in selected cases, the insertion of a permanent pacemaker is indicated.