Prophylactic Atrial Arrhythmia Surgical Procedures With Congenital ...

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Prophylactic Atrial Arrhythmia Surgical Procedures With Congenital Heart Operations: Review and Recommendations Constantine Mavroudis, MD, John M. Stulak, MD, Niv Ad, MD, Allison Siegel, MSSA, Alessandro Giamberti, MD, Louise Harris, MD, Carl L. Backer, MD, Sabrina Tsao, MD, Joseph A. Dearani, MD, Nihal Weerasena, MD, and Barbara J. Deal, MD

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Johns Hopkins Children’s Heart Surgery, Florida Hospital for Children, Orlando, Florida; Division of Cardiovascular Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota; Inova Fairfax Hospital, Cardiac Surgery Inova Heart and Vascular Institute, Falls Church, Virginia; Pediatric and Adult Congenital Heart Surgery, IRCSS Policlinico San Donato, San Donato M.se (MI), Italy; Division of Cardiology, Peter Munk Cardiac Centre, Toronto Congenital Cardiac Centre for Adults, Toronto, Ontario, Canada; Cardiovascular-Thoracic Surgery and Division of Cardiology and the Department of Pediatrics, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois; and Division of Cardiothoracic Surgery, The General Infirmary, Leeds, United Kingdom

Specific congenital heart anomalies significantly increase the risk for late atrial arrhythmias, raising the question whether prophylactic arrhythmia operations should be incorporated into reparative open heart procedures. Currently no consensus exists regarding standard prophylactic arrhythmia procedures. Questions remain concerning the arrhythmia-specific lesions to perform, energy sources to use, need for atrial appendectomy, and choosing a right, left, or biatrial Maze procedure. These considerations are

important because prophylactic arrhythmia procedures are performed without knowing if the patient will actually experience an arrhythmia. This review identifies congenital defects with a risk for the development of atrial arrhythmias and proposes standardizing lesion sets for prophylactic arrhythmia operations.

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technical considerations are paramount, including optimal prophylactic lesion sets, type of energy source, proximity of lesions to important structures, need for left atrial (LA) appendectomy, and application to 1 or both atria. These complex issues are balanced by the fact that prophylactic lesions are performed without advanced knowledge that arrhythmias will actually develop postoperatively. Invocation of bioethical principles of nonmaleficence, beneficence, patient autonomy, and justice are relevant and apply. The purpose of this article is to identify patients undergoing CHD repair who are at significant risk for the development of late atrial arrhythmias, review the literature of concomitant prophylactic and therapeutic arrhythmia ablation during CHD repair, and offer standardized lesion sets for future interprogram comparisons. A meeting, Arrhythmia Surgery in Patients with Congenital Heart Disease, was called in May 2013 with all the authors to present his or her vast experience in arrhythmia prevention and ablation in patients with CHD. After the presentations, a discussion took place to develop a set of lesions to be used in these patients in an attempt to prevent arrhythmias. It was determined that the anomalies to be discussed would include Ebstein’s anomaly

atients with congenital heart disease (CHD) are at increased risk for the development of late arrhythmia, which adversely impacts ventricular function, physical well-being, and long-term survival [1, 2]. Arrhythmias are the leading source of morbidity requiring hospitalization in adulthood after repair of CHD [3, 4]. Efficacy of catheter and surgically based treatment for specific atrial arrhythmia substrates has been well established; whether these therapeutic tenets can be extended to the prevention of arrhythmia development is unknown and untested. Prophylactic arrhythmia operations are proposed for patients with specific anatomic substrates as an effort to reduce the development of late arrhythmias. If effective, one would expect a significant decrease in late morbidity and perhaps a reduction in the risk of sudden death from arrhythmias. There is no unanimity of opinion as to what constitutes a standard therapeutic arrhythmia procedure, let alone a prophylactic intervention [5–9]. Application of therapeutic lesion sets with demonstrated efficacy may have unwanted consequences that pose concerns when used as prevention. Although the aim of the original Cox-Maze procedure was to treat atrial fibrillation (AF) with low morbidity, concerns for sinus node dysfunction led to modification of the lesion sets that was designed to minimize potential harm to the sinus node. Numerous

Address correspondence to Dr Mavroudis, 2501 N Orange Ave, Ste 540, Orlando, FL 32804; e-mail: constantine.mavroudis.md@flhosp.org.

Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier

(Ann Thorac Surg 2015;99:352–9) Ó 2015 by The Society of Thoracic Surgeons

Dr Ad discloses financial relationships with Atricure and Medtronic.

0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.07.026

Ann Thorac Surg 2015;99:352–9

REVIEW MAVROUDIS ET AL PROPHYLACTIC ARRHYTHMIA OPERATIONS AND CHD

Abbreviations and Acronyms AF AFL ASD AV CHD EA IVC LA RA RF SVC SVT TGA TOF

= = = = = = = = = = = = = =

atrial fibrillation atrial flutter atrial septal defect atrioventricular congenital heart disease Ebstein’s anomaly inferior vena cava left atrial right atrial radiofrequency superior vena cava supraventricular tachycardia transposition of great arteries tetralogy of Fallot

(EA), atrial septal defect (ASD), Fontan physiology, and tetralogy of Fallot (TOF). All authors provided their personal libraries and presentations for the purpose of providing guidance for physicians working with this patient population. After the meeting, a search was completed on PubMed for all articles published after 2000 using the following list of search terms: arrhythmia in CHD, prophylactic arrhythmia, sudden death in CHD, lesion sets, EA, TOF, ASD, single-ventricle physiology (Fontan, univentricular), atrial flutter (AFL), AF, surgical ablation, and energy sources with surgical ablation arrhythmias. Because the authors are from all over the world, communication continued through e-mail, telephone conferences, and in-person review as the article was developed and completed.

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development of AFL/AF presumably related to atrioventricular (AV) valve regurgitation and atrial dilatation (65% of SVT episodes), and an increasing awareness of the high risk of sudden death in this population (7%–15%) [16–18]. Catheter ablation of right-sided accessory connections in EA is associated with technical challenges and high arrhythmia recurrence owing to right atrial (RA) dilatation, AV annulus distortion, and muscle continuity between the right atrium and right ventricle [18–21]. In a large follow-up series of patients with EA after tricuspid valve repair, more than 60% were rehospitalized for cardiac causes at 20 years, the most common of which was arrhythmia [16]. The cause of the increased risk of sudden death is not clear and could be a consequence of rapid atrial arrhythmia resulting in low cardiac output or primary ventricular arrhythmia [18, 22–24]. Efforts to reduce the occurrence of atrial arrhythmias in patients with EA are thus critical [16–19].

Transposition of the Great Arteries Atrial switch repairs for TGA are associated with loss of sinus node function in 50% of patients at 20-year followup and the development of AFL in approximately one quarter of survivors [25, 26]. The atrial switch operation for TGA has been abandoned except in patients with congenitally corrected TGA in whom a double switch operation is performed. If future studies demonstrate prophylactic arrhythmia operation efficacy in other diagnostic substrates, incorporation of cavotricuspid isthmus ablation during the primary reparative procedure for TGA variants undergoing atrial baffle procedures may be considered.

Single-Ventricle Physiology

Ebstein’s Anomaly

Atrial Septal Defect

The arrhythmia concerns associated with EA are right-sided accessory connections (15%–30%), the late

As many as 50% of patients undergoing ASD repairs after 40 years of age [39, 40] were found to experience late

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Large clinical CHD database analyses with long-term follow-up have demonstrated the prevalence of late arrhythmias and identified the anatomic substrates at highest risk [2, 10–15]. Between 1985 and 2000, the prevalence of severe CHD in adults increased by 85%, and now approximately 49% of severe CHD exists in adults rather than children [2]. Bouchardy and colleagues [14] evaluated the impact of atrial arrhythmias in their adult database and found that in patients with no reported arrhythmias by 18 years of age, greater than 50% experienced atrial arrhythmias by 65 years. The impact of atrial arrhythmias increased the risk of early mortality by 50%, more than doubled the risk of stroke or heart failure, and caused a 3-fold increase in cardiac interventions. The anatomic diagnoses with the highest prevalence of supraventricular tachycardia (SVT) in the Bouchardy and colleagues study were EA (33%), transposition of the great arteries (TGA) status post atrial switch (28%); single-ventricle physiology (24%), ASD (18.9%), and TOF and truncus arteriosus (15.5%) [14].

Perhaps the most challenging patients with CHD are those with Fontan physiology in whom atrial arrhythmias develop in more than 50% of cases [27–32], usually associated with significant RA dilatation and hemodynamically important lesions. Arrhythmias include multiple RA reentrant circuits, focal atrial tachycardia, and an increased incidence of AF. Modification of the Fontan operation has decreased the incidence of late atrial tachycardia from more than 60% in earlier atriopulmonary connection repairs [27, 28] to 13% to 60% in lateral tunnel connections [33–35] and approximately 8% to 15% in extracardiac connections [30–32]. Late arrhythmias in patients with modified connections can be expected to rise with longer follow-up. Catheter ablation in the patient with Fontan physiology has acute success rates of 50% to 70%, with at least 70% recurrence of tachycardia within 2 years [36–38]. Catheter access to the right atrium in patients with extracardiac connections is limited to the transhepatic or transthoracic approach, with attendant morbidity. Certainly patients with previous Fontan operations who are undergoing reoperations should be considered for prophylactic atrial lesion sets.

Lesion-Specific Literature Review

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postoperative AF/AFL compared with less than 10% of patients when repair was performed in childhood [41]. Murphy and coworkers [40] reported on 123 patients after surgical repair of ostium secundum or sinus venosus ASD, with follow-up extending 27 to 32 years. Patients 41 years or older had more frequent late cardiac failure, stroke, and AF. Age at operation was the most powerful independent predictor of long-term morbidity and mortality. Gatzoulis and associates [39] reviewed 213 adults who underwent surgical ASD closure, and 40 (19%) had preoperative AF/AFL. Patients with preoperative AF/AFL were older and had higher pulmonary artery pressure compared with those without preoperative arrhythmia. At mean follow-up of 3.8 years, 24 of the 40 patients (60%) with preoperative AF/AFL had persistent arrhythmia. De novo late AF/AFL was more common in patients older than 40 years at time of operation compared with those younger than 40 years in both series [39, 40]. Closure of ASDs asserts a remodeling effect thought to be beneficial for preventing arrhythmias in younger patients [41, 42]. However, older patients undergoing ASD closure should be considered for prophylactic arrhythmia procedures [43].

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Tetralogy of Fallot The development of arrhythmias in TOF occurs late after primary repair and is often associated with pulmonary regurgitation, tricuspid regurgitation, right ventricular dilatation, and myocardial dysfunction. Supraventricular tachycardia occurs in 15% to 35% of late postoperative patients with TOF [14, 44–48]. Prophylactic ablation at the time of primary TOF repair needs to be weighed against favorable transcatheter ablation accessibility and success rates. Karamlou and colleagues [46] demonstrated efficacy of concomitant atrial arrhythmia operations in patients with TOF with preexisting atrial arrhythmias undergoing reoperative pulmonary valve or tricuspid valve operations, or both. Thus, the older patient undergoing reoperation for pulmonary valve replacement or tricuspid valvuloplasty, or both, might be considered a candidate for prophylactic RA arrhythmia operations.

Evolution of Arrhythmia Surgical Nomenclature The original Cox-Maze procedures for AF were characterized as Cox-Maze I, II, and III [49]. Lesion sets were based on animal studies, with lines of block in both right and left atria designed to allow sinoatrial node impulse conduction to the AV node without allowing atrial reentry, which may trigger atrial reentry tachycardia/AF. Because original lesion sets were designed as “cut and sew,” energy ablative sources were introduced to shorten the procedure and limit bleeding. Subsequent efforts to minimize the pulmonary vein and LA lesions resulted in procedures labeled “mini”-Maze, “modified” Maze, “left atrial” Maze, and “biatrial” Maze. “Maze” became synonymous with any lesion set that was applied to the atria as therapy for reentrant atrial arrhythmias. Comparing the efficacy of Maze variations for AF is hampered by the


continuing evolution in lesion sets coupled with evolving energy sources. For therapeutic lesions designed to treat existing arrhythmias, the outcome can be assessed by tachycardia recurrence. However, to assess long-term prophylactic lesion set efficacy, it is especially important to adopt a uniform lesion set for specific anatomic substrates with standardized follow-up.

Surgical Techniques: Atrial Arrhythmias Atrial Fibrillation The goal of surgical lesions to treat AF is the elimination of AF, preservation of sinus node conductivity to the AV junction, and maintenance of atrial transport function. Central to the efficacy of the LA Maze procedure are lesions designed to encircle the pulmonary veins and limit reentry circuits that would occur in the left AV valve isthmus, coronary sinus, and Bachmann’s bundle in the dome of the left atrium. The original Cox-Maze procedure included LA appendectomy with an incision to the confluence of the pulmonary vein encircling lesion or lesions. Resection of the LA appendage is thought important for removal of a source of thrombi known to occur in AF; it is not clear whether LA appendectomy plays a role in arrhythmia ablation. These tenets form the basis of subsequent modifications of surgical ablation, all developed to shorten the operation and allow epicardial approaches [50, 51]. In addition, because AF mostly originates in the left atrium, exclusion of right-sided lesions was introduced. The original cut-and-sew Cox-Maze III procedure resulted in long-term freedom from AF in more than 97% of patients [49, 52]. All subsequent modifications have achieved varying degrees of efficacy approaching 93% [53–55]. The superiority of a biatrial Maze procedure for AF prevention has been demonstrated in several studies [5, 54, 55]. Transcatheter AF ablative procedures have been designed to minimize the lesion sets still further owing to the intrinsic nature of transcatheter approaches that require complex catheter maneuvers, transseptal perforation, and extended fluoroscopy time. Complications not generally experienced with the surgical approach include esophageal perforation and pulmonary vein orifice stenosis.

Atrial Flutter The classic “cut-and-sew” right-sided Maze procedure [49] included a linear incision from the superior vena cava (SVC) to the inferior vena cava (IVC), RA appendectomy, incision from the base of the resected RA appendage to the midpoint of the RA anterior wall not in communication with the SVC-IVC incision, an incision posteriorly from the base of the RA appendage to the anterior tricuspid annulus, and a communicating incision from the SVC-IVC incision to the posterior tricuspid annulus. Importantly, these lesions were developed from animal models without CHD or previous operations. Subsequent electrophysiologic studies have demonstrated the key role of the RA cavotricuspid isthmus in


typical right AFL (cavotricuspid isthmus–dependent RA macroreentry) [56–59]. The RA cavotricuspid isthmus is the area between the tricuspid annulus and the coronary sinus and the IVC. Targeted ablation of this cavotricuspid isthmus region transforms the area of “slow conduction” to an area of “no conduction” and effectively terminates typical AFL [60]. In the CHD population, additional RA macroreentrant circuits have been identified and referred to as “non–isthmus-dependent” tachycardia [61]. These circuits may involve reentry around previous incisions (“incisional tachycardia”) or prosthetic material such as ASD patches. The lateral RA wall at the inferior aspect of the crista terminalis is often an area of unexcitable atrial tissue with low-voltage electrograms labeled as “scar,” which may contribute to additional macroreentrant circuits. Elimination of the isthmus of slow conduction between these incisions, patches, or electrical scars forms the basis of ablation strategies for non–isthmus-dependent RA tachycardia and are referred to as “modified RA Maze procedures.” Although effective for elimination of RA tachycardia in the setting of complex CHD [61, 62], the RA Maze may not be appropriate to use as prophylactic lesion sets, because the evidence for first-time arrhythmia occurrence favors a cavotricuspid isthmusdependent circuit. In light of the accumulated retrospective studies, the cavotricuspid isthmus lesion (Fig 1) for RA arrhythmia prophylaxis in these select diagnostic subgroups seems warranted. When there is a risk of AF such as in patients with ASD who are older than 40 years and in patients with EA with large left atria, addition of a prophylactic left-sided Maze procedure (Fig 2) should be considered.


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Fig 1. Right atrial view of potential prophylactic cavotricuspid isthmus ablative lesion set after aortobicaval cardiopulmonary bypass and cardioplegic arrest. One continuous cryoablation lesion is shown connecting tricuspid annulus at commissure of septal and posterior leaflets with inferior coronary sinus os and extending to posterior os of inferior vena cava (IVC). Additional lesion is placed to extend with cryoablation medial atriotomy to anterior os of IVC. Special care must be taken to place tricuspid-to-coronary sinus lesion away from conduction system that generally lies within triangle of Koch. This part of lesion set can be omitted if there is any question that conduction system may extend to tricuspid annulus at commissure of septal and posterior leaflets. (Ao ¼ aorta; PA ¼ pulmonary artery; SVC ¼ superior vena cava.)

Arrhythmia Operations in Association With CHD Repairs

Technical concerns relative to the cut-and-sew Maze operation include length of the procedure and risk of perioperative bleeding. Khargi and colleagues [63] compared alternative sources of energy (radiofrequency [RF] microwave and cryoablation) (group 1) for treating AF with a classic Cox-Maze III procedure (group 2) [63]. Forty-eight studies reviewed 3,832 patients (2,279 in group 1 and 1,553 in group 2). There was no difference in mean duration of preoperative AF, LA diameter, and left ventricular ejection fraction. Freedom from AF was not statistically significant between groups (78% in group 1 versus 85% in group 2), implying no important difference in energy source efficacy. Besides cryothermy and bipolar RF energy, which are currently the most reliable intraoperative ablative techniques, high-frequency ultrasound, microwave energy, and laser energy sources have been used [5, 54]. New energy sources will continue to be introduced [55, 63–65]. Throughout the evolution of alternative energy sources, it has been understood that the transmurality of lesions is more important than the energy sources used to achieve results. Central to the application of these lesions is the importance of limiting collateral damage to the pulmonary vein ostia, coronary arteries, valve structures, and esophagus.

Fig 2. Right and left atrial views of potential prophylactic ablative lesions after aortobicaval cardiopulmonary bypass and cardioplegic arrest. Shown in left atrium are lesions sets that indicate circumferential isolation of pulmonary vein confluence, connection of pulmonary vein confluence with P3 location of posterior mitral valve annulus, and connection of pulmonary vein confluence with base of left atrial appendage (LAA). (Ao ¼ aorta; PA ¼ pulmonary artery; SVC ¼ superior vena cava.)

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Energy Sources

After early development of the Cox-Maze III as a standalone arrhythmia operation, AF surgical intervention was incorporated into mitral valve repairs and coronary

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artery bypass operations. Subsequently, arrhythmia operations were performed in association with repairs of simpler forms of CHD, followed by incorporation into surgical revisions in the univentricular heart [13]. Maze procedures in these populations showed freedom from AF/AFL ranging from 83% to 95% at hospital discharge and 79% to 93% during follow-up [7, 50, 66–68]. In addition, late functional outcome improved after concomitant Maze procedures in several studies [7, 66].

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Proposed Lesions Sets for Prophylactic Arrhythmia Operations Few studies have applied therapeutic lesion sets as prophylactic measures to prevent arrhythmias in humans [9, 69, 70]. Rodefeld and colleagues [71] in 1966 reported that a prophylactic atrial incision disrupted potential circuits around the atriotomy and prevented intraatrial reentrant tachycardia from developing in canines. Based on those animal studies, Collins and associates [72] applied a single incision in the anterior atrial flap to the anterior tricuspid annulus in patients with Fontan physiology. The short-term results failed to show efficacy, which was perhaps related to the small number of patients with limited duration of follow-up or, alternatively, related to a lesion set that did not address the RA cavotricuspid isthmus. Prophylactic arrhythmia operations with CHD have not been systematically tested by a prospective randomized clinical study owing to nonstandardized lesion sets, ill-defined patient populations, and lack of unanimity for the need of this additive procedure. Based on the limited clinical studies, retrospective surgical and transcatheter ablation results, and debated opinion of the authors, prophylactic arrhythmia lesion sets are offered for diagnostic subsets with predictive arrhythmia occurrence that are undergoing a primary or secondary therapeutic anatomic surgical intervention (Table 1; Figs 1, 2). We selected CHD lesions at highest risk for late development of AF or typical right AFL, taking into consideration the ease of subsequent transcatheter ablation. The efficacy of the lesion sets for preexisting atrial tachycardia was assessed and judgments offered for the minimal effective

prophylactic lesions without additional morbidity such as AV block, proarrhythmia, or collateral damage to adjacent structures.

Ebstein’s Anomaly Some patients undergoing first-time EA repair do not have accessory connections or reentry-mediated atrial tachycardia. It is well known, however, that postoperative patients with EA have a high incidence of both atrial reentry tachycardia and AF owing to right and left atriomegaly, respectively [18, 19]. Our opinion is that a cavotricuspid isthmus lesion (Fig 1) be considered uniformly during repair of EA in older patients, with additional consideration for a left-sided Maze procedure (Fig 2) in the uncommon setting of marked LA dilatation.

Univentricular Hearts In patients with univentricular hearts undergoing primary intracardiac Fontan procedures, application of a limited “isthmus” lesion in the inferolateral right atrium poses the need for intracardiac access that might otherwise not be needed, carries the risk of injury to the AV node in the small heart, and poses specific anatomic landmark challenges [73–76]. For instance, patients with tricuspid atresia have no tricuspid valve; an isthmus lesion would therefore connect the coronary sinus with the IVC. Patients with right-sided heterotaxy have no coronary sinus and therefore an isthmus lesion would extend from the common AV valve annulus to the IVC orifice (away from the displaced AV node) and toward the extended right atriotomy. However, in patients who have undergone previous Fontan procedures who are now undergoing revision or conversion operations, based on the limited transcatheter approach and high incidence of postoperative atrial tachycardia, a modified RA Maze procedure is recommended.

Atrial Septal Defects Few studies compare ASD closures with and without a concomitant Maze procedure [43, 70]. Specifically, Kobayashi and colleagues [70] evaluated the efficacy and risk of the Maze procedure in 26 patients (mean age, 58

Table 1. Suggested Prophylactic Lesion Sets For Patients With Specific Congenital Heart Disease Congenital Heart Disease

Type of Arrhythmia at Risk

Prophylactic Lesion Set

Ebstein’s anomaly

ART, large right atrium

see Fig 1

ART, large right and left atria

see Fig 2

ART

see Fig 1

Atrial fibrillation ART

see Figs 1 and 2 see Fig 1

Univentricular hearts Atrial septal defect Tetralogy of Fallot ART ¼ atrial reentrant tachycardia.

Timing of Procedure Primary repair in patients without arrhythmias; most reparative operations performed in adolescents and adults Primary repair in patients without arrhythmias; not enough data to recommend prophylactic operations in neonates and infants Primary repair Fontan operation in patients without arrhythmias Patients > 40 y without arrhythmias Reoperation for older patients without arrhythmias


Tetralogy of Fallot Patients undergoing pulmonary valve insertion with no evidence of clinically sustained atrial arrhythmias are excellent candidates for prophylactic arrhythmia operations with ablation of the cavotricuspid isthmus area (Fig 1). Two clinical approaches can be used to apply the isthmus lesion set in these patients: all patients with TOF who are older than 18 years and are undergoing reoperation receive prophylactic isthmus ablation versus limiting the procedure to those with inducible atrial tachycardia. Although not widely practiced, preoperative electrophysiologic assessment could be performed to determine if RA isthmus-dependent arrhythmias can be induced. Patients with inducible atrial tachycardia could undergo “prophylactic” cavotricuspid isthmus ablation; patients without inducible atrial tachycardia could be followed without prophylactic arrhythmia operations.

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The question arises whether applying an isthmus lesion in patients without clinical atrial arrhythmias but with inducible atrial tachycardia is “prophylactic.” These issues require future contemplation. Regarding ablative lesions for ventricular tachycardia in TOF, there is consensus to avoid prophylactic ablative techniques for ventricular tachycardia. Ventricular lesions may be associated with proarrhythmia and risk of sudden death [79, 80].

Comment Prophylactic arrhythmia operations are warranted in certain select subsets of patients with CHD based on their high risk of experiencing important atrial arrhythmias. The authors’ consensus is that lesion sets should be standardized for therapeutic measures as well as for prophylactic applications for patients with CHD undergoing repair or repeated repair in an effort to minimize unwanted consequences. The principles of prophylactic procedures should be preserved: the prophylactic procedure should have demonstrated efficacy, should be simple to perform, and should be attended by minimal complications. Organized, prospective, randomized, multiinstitutional studies are necessary to assess outcomes of such a strategy.

References 1. Koyak Z, de Groot JR, Mulder BJ. Interventional and surgical treatment of cardiac arrhythmias in adults with congenital heart disease. Expert Rev Cardiovasc Ther 2010;8:1753–66. 2. Khairy P, Ionescu-Ittu R, Mackie AS, Abrahamowicz M, Pilote L, Marelli AJ. Changing mortality in congenital heart disease. J Am Coll Cardiol 2010;56:1149–57. 3. Verheugt CL, Uiterwaal CS, van der Velde ET, et al. Mortality in adult congenital heart disease. Eur Heart J 2010;31:1220–9. 4. O’Leary JM, Siddiqi OK, de Ferranti S, Landzberg MJ, Opotowsky AR. The changing demographics of congenital heart disease hospitalizations in the United States, 1998 through 2010. JAMA 2013;309:984–6. 5. Gillinov AM. Choice of surgical lesion set: answers from the data. Ann Thorac Surg 2007;84:1786–92. 6. Cox JL, Jaquiss RD, Schuessler RB, Boineau JP. Modification of the maze procedure for atrial flutter and atrial fibrillation. II. Surgical technique of the maze III procedure. J Thorac Cardiovasc Surg 1995;110:485–95. 7. Stulak JM, Dearani JA, Puga FJ, Zehr KJ, Schaff HV, Danielson GK. Right-sided Maze procedure for atrial tachyarrhythmias in congenital heart disease. Ann Thorac Surg 2006;81:1780–5. 8. Khargi K, Keyhan-Falsafi A, Hutten BA, Ramanna H, Lemke B, Deneke T. Surgical treatment of atrial fibrillation: a systematic review. Herzschrittmacherther Elektrophysiol 2007;18:68–76. 9. Theodoro DA, Danielson GK, Porter CJ, Warnes CA. Rightsided maze procedure for right atrial arrhythmias in congenital heart disease. Ann Thorac Surg 1998;65:149–54. 10. Nieminen HP, Jokinen EV, Sairanen HI. Late results of pediatric cardiac surgery in Finland: a population-based study with 96% follow-up. Circulation 2001;104:570–5. 11. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation 2007;115:163–72. 12. Zomer AC, Vaartjes I, Uiterwaal CS, et al. Circumstances of death in adult congenital heart disease. Int J Cardiol 2012;154: 168–72.

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years) with ASD and AF. These patients were compared with a control cohort of 45 patients (mean age, 55 years) without AF who underwent ASD closure alone. Incidence of late AF was significantly higher in the control group (18%) than in the Maze group (0%). Considering the 40-year-old patient with secundum ASD who presents for transcatheter or surgical closure, the question arises whether to include concurrent transcatheter or surgical prophylactic right-sided and left-sided arrhythmia procedures. Alternatively, transcatheter ablation could be performed late after the anatomic procedure for an arrhythmia indication [77, 78]. A number of prophylactic surgical ablative lesions can be considered for the patient with ASD who is older than 40 years of age. The most inclusive is a full Cox-Maze lesion set with special attention to the interatrial lesion (between the fossa ovalis and the posterior atrial incision). The SVC-IVC lesion may be placed in a more posterior position to avoid injury to the sinoatrial node. The least invasive prophylactic lesion sets in this setting are the left lesion set (Fig 2) and the cavotricuspid isthmus lesion in the right atrium (Fig 1). These lesion sets have historical significance, require the smallest number of lesion applications, and are less likely to cause arrhythmias, leading the way for a prospective study that is likely to have more universal acceptance than the more extensive lesion set. In the unusual setting of a patient older than 40 years undergoing primary surgical repair for ostium primum ASD (partial AV canal), SVC sinus venosus ASD, IVC sinus venosus ASD, or single atrium, additional technical concerns arise. The AV node in patients with ostium primum ASD (partial AV canal) is displaced closer to the coronary sinus; a cavotricuspid isthmus prophylactic lesion would jeopardize the AV node and is not recommended. A prophylactic lesion in older patients should be placed to connect the lateral right AV annulus to the IVC distant from the coronary sinus as described for patients with single-ventricle heterotaxy [74]. Prophylactic cavotricuspid isthmus lesions can be placed in older patients with sinus venosus ASD without the probability of AV node dysfunction.


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