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Ann Thorac Surg 1998;65:149-154
© 1998 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Right-Sided Maze Procedure for Right Atrial Arrhythmias in Congenital Heart Disease

Division of Cardiovascular and Thoracic Surgery, Section of Pediatric Cardiology, and Division of Cardiovascular Diseases, Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA

Dr Danielson, Section of Cardiovascular Surgery, Mayo Clinic, 200 First St, SW, Rochester, MN 55905.

Presented at the Thirty-third Annual Meeting of The Society of Thoracic Surgeons, San Diego, CA, Feb 3–5, 1997.

	Abstract

Top Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Background. Atrial fibrillation and flutter, commonly associated with congenital heart anomalies that cause right atrial dilatation, may cause significant morbidity and reduction of quality of life, even after surgical repair of the anomalies.

Methods. In an effort to reduce the incidence of atrial tachyarrhythmias after repair of right-sided congenital heart disease, we performed a concomitant right-sided maze procedure.

Results. Eighteen patients with paroxysmal atrial fibrillation or flutter (n = 12) or chronic atrial fibrillation or flutter (n = 6) aged 10.9 to 68.4 years (mean 34.9 years) underwent a right-sided maze in association with repair of Ebstein’s anomaly (n = 15), congenital tricuspid insufficiency (n = 2), and isolated atrial septal defect (n = 1). There were no early deaths, reoperations, or complete heart block. Discharge rhythm was sinus (n = 16) or junctional (n = 2). Follow-up was complete in all 18 patients and ranged from 3.1 to 17.2 months (mean 8.1 months); all are in New York Heart Association class I. Early postoperative arrhythmias developed in 3 patients (all were converted to sinus rhythm by antiarrhythmic drugs). There were no late deaths or reoperations.

Conclusions. The inclusion of a right-sided maze procedure with cardiac repair in patients having congenital heart anomalies that cause right atrial dilatation and associated atrial tachyarrhythmias is effective in eliminating or reducing the incidence of those arrhythmias.

	Introduction

Top Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Atrial tachyarrhythmias, especially atrial fibrillation and atrial flutter, are commonly associated with congenital heart anomalies that cause right atrial dilatation [1][2][3][4]. We report our results in a group of patients undergoing surgical treatment of right-sided congenital heart disease in which a concomitant right-sided maze procedure, a modification of the Cox maze III operation [5], was performed for the correction of atrial fibrillation or flutter.

	Patients and Methods

Top Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Patients
Between February 21, 1995, and February 1, 1997, 22 patients with right-sided congenital heart disease associated with atrial fibrillation or flutter underwent a concomitant right-sided maze procedure at the time of cardiac repair. For reasons of length of follow-up, the first 18 patients (9 men, 9 women) were selected for this review; ages at operation ranged from 10.9 to 68.4 years (mean, 34.9 years). The right-sided maze procedure was combined with correction of Ebstein’s anomaly (n = 15), congenital tricuspid insufficiency (n = 2), and isolated atrial septal defect (n = 1). Diagnoses were based on clinical evaluation and echocardiography. All patients had severe right atrial enlargement demonstrated on preoperative chest radiography and echocardiography. Indications for operation included New York Heart Association functional class III or IV, progressive cyanosis, and progressive exercise intolerance. Associated cardiac defects included atrial septal defect (n = 12), accessory conduction pathway(s) (n = 2), and pulmonary artery stenosis (n = 2). Previous cardiovascular operations included tricuspid valve surgery (n = 2), pulmonary valvotomy (n = 2), closure of ventricular septal defect (n = 2), surgical ablation of accessory conduction pathway(s) (n = 2), and coronary artery bypass grafting, Blalock-Taussig shunt, and ligation of aneurysm of right coronary artery (n = 1). Three patients had undergone catheter ablation of accessory conduction pathways elsewhere.

Documented atrial tachyarrhythmias included paroxysmal atrial fibrillation or flutter (n = 12) and chronic atrial fibrillation or flutter (n = 6). Fourteen patients were receiving antiarrhythmic drugs preoperatively, including digoxin, ß-receptor antagonists, calcium-channel antagonists, quinidine, propafenone, procainamide, and amiodarone, and 7 had required multiple electrocardioversions. Preoperative electrocardiography showed sinus rhythm in 12, atrial flutter in 4, and atrial fibrillation in 2 patients. The duration of documented atrial fibrillation or flutter ranged from 6 months to 54 years (mean, 14.2 years).

Follow-up information was obtained from correspondence with the patients and referring physicians and from telephone interviews conducted with all of the patients.

Surgical Technique
Cardiopulmonary bypass is instituted with standard cannulation of the ascending aorta for arterial inflow and direct cannulation of the superior and inferior venae cavae for venous drainage. The perfusate temperature is lowered to 25° to 30°C depending on the anticipated length of the procedure. A left ventricular vent is inserted through the right superior pulmonary vein and an aortic tack vent is placed.

Atrial septal defects are closed by direct suture or pericardial patch, and congenital tricuspid insufficiency is treated by valve repair, when feasible, or valve replacement. Our operative management of patients with Ebstein’s anomaly is described in detail in previous reports from our institution [1] and consists of (1) electrophysiologic mapping for localization of accessory conduction pathways in those patients having ventricular preexcitation, (2) excision of any attenuated atrial septum and pericardial patch closure of the septal defect for patients having an atrial septal defect or patent foramen ovale, (3) selective plication of the atrialized right ventricle, (4) reconstruction of the tricuspid valve, when feasible, or valve replacement, (5) correction of associated anomalies such as relief of pulmonary stenosis and division of accessory conduction pathways, and (6) excision of redundant right atrial wall (right reduction atrioplasty).

Our modification of the Cox maze III procedure [5] is illustrated in Fig 1Fig 2Fig 3Fig 4Fig 5Fig 6. The right atrial appendage is excised and the first maze incision is made from the base of the resected appendage inferolaterally to within 3 to 4 cm of the inferior vena cava (Fig 1Fig 2). The second maze incision is made anteriorly full thickness through the atrial myocardium to the anterior aspect of the tricuspid annulus. The area at the annulus is cryoablated or, more recently, lightly electrocoagulated to ensure all remaining muscle fibers are ablated, and the incision is closed (Fig 3). The third incision is made from the superior vena cava to the inferior vena cava parallel to the plane of the atrial septum (Fig 3). The inferior end of this incision is repaired for approximately 2 cm. A fourth incision is made perpendicularly from the caudal portion of incision number 3 and carried to the posterolateral aspect of the tricuspid valve annulus (Fig 4). The area at the annulus is lightly electrocoagulated and the incision is partially repaired. If not required earlier to facilitate exposure, the aorta is then cross-clamped and the heart is arrested with cold blood cardioplegia and topical hypothermia. A fifth incision is made from the midportion of incision number 3 across the atrial septum and fossa ovalis, down to the tendon of Todaro near the coronary sinus; this incision is extended into the left atrium laterally for 1 to 2 cm and then closed (Fig 5).

View larger version (91K): [in this window] [in a new window]   A surgeon’s view of the right atrium and right atrial appendage (RAA), superior vena cava (SVC), inferior vena cava (IVC), and the location of the sinus node (SN). (Reprinted with permission of the Mayo Clinic.)

View larger version (107K): [in this window] [in a new window]   Initial steps in the right-sided maze procedure. The right atrial appendage is excised and the first maze incision is made from the base of the resected appendage inferolaterally to within 3 to 4 cm of the inferior vena cava. (Reprinted with permission of the Mayo Clinic.)

View larger version (91K): [in this window] [in a new window]   The second maze incision is made anteriorly full thickness through the atrial myocardium to the anterior aspect of the tricuspid annulus. The area at the annulus is lightly electrocoagulated to ablate all remaining muscle fibers and the incision is closed. The third incision is made from the superior vena cava to the inferior vena cava parallel to the plane of the atrial septum. (Reprinted with permission of the Mayo Clinic.)

View larger version (107K): [in this window] [in a new window]   The inferior end of incision 3 is repaired for approximately 2 cm. A fourth incision is made perpendicularly from the caudal portion of incision No. 3 and carried to the posterolateral aspect of the tricuspid valve annulus. The area at the annulus is lightly electrocoagulated and the incision is partially closed. (CS = coronary sinus; FO = fossa ovalis; TV = tricuspid valve.) (Reprinted with permission of the Mayo Clinic.)

View larger version (127K): [in this window] [in a new window]   A fifth incision is made from the midportion of incision 3 across the atrial septum and fossa ovalis down to the tendon of Todaro near the coronary sinus; this incision is extended into the left atrium laterally for 1 to 2 cm and then closed. (Reprinted with permission of the Mayo Clinic.)

View larger version (122K): [in this window] [in a new window]   After concomitant cardiac procedures, the remaining right atrial maze incisions are closed. (SN = sinus node.) (Reprinted with permission of the Mayo Clinic.)

	Results

Top Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
A right-sided maze procedure was performed in all 18 patients. Concomitant cardiac procedures included tricuspid valve replacement (n = 16), closure of an atrial septal defect (n = 12), repair of pulmonary stenosis (n = 2), division of accessory conduction pathway (n = 2), and repair of tricuspid valve (n = 1). Cardiopulmonary bypass times ranged from 119 minutes to 307 minutes (median, 152 minutes) and aortic cross-clamp times ranged from 33 minutes to 100 minutes (median, 78 minutes). The patient with the longest bypass time had Ebstein’s anomaly and a previous procedure elsewhere for pulmonary valvotomy and closure of atrial septal defect and ventricular septal defect. She required tricuspid valve replacement, pulmonary valve replacement, pericardial patch enlargement of the main pulmonary artery, patch closure of recurrent ventricular septal defect, and the right-sided maze procedure. This patient also had the longest duration of chronic atrial fibrillation (8 years); she has been in normal sinus rhythm since the operation.

Most patients were paced atrially during weaning from cardiopulmonary bypass. There were no early deaths. No patient required intraaortic balloon pumping for hemodynamic support, and no patient had development of complete heart block or required early reoperation. Early postoperative complications included ventricular arrhythmia in 3 patients (multiple premature ventricular beats [n = 2]) and one run of nonsustained ventricular tachycardia) and prolonged (5 days) mechanical ventilation in 1 patient. An electrocardiogram at the time of hospital discharge showed sinus rhythm in 16 patients and junctional rhythm in 2 patients. The postoperative hospital stay ranged from 5 to 18 days (median, 7 days).

No late deaths have occurred in follow-up times ranging from 5.3 to 24 months (mean, 13 months). All patients are in functional class I and no patients have required late reoperation. One patient required placement of a dual-chamber rate-responsive pacemaker for chronotropic insufficiency with exercise. Late atrial arrhythmias developed in 3 patients at 3.0, 3.5, and 8.0 months postoperatively, respectively (mean 4.6 months). The rhythm was paroxysmal atrial flutter in 2 patients and paroxysmal atrial fibrillation in 1 patient. All three arrhythmias were converted to sinus rhythm by antiarrhythmic drugs with or without electrocardioversion. At last contact, 10 patients were receiving medications, including digoxin (n = 5), amiodarone (n = 2), ß-receptor antagonist (n = 2), and propafenone (n = 1). Late follow-up electrocardiograms ranging from 2.5 to 16 months (mean 9.3 months) showed 16 patients were in sinus rhythm (1 with intermittent pacemaker function) and 2 patients were in junctional rhythm.

	Comment

Top Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
Congenital heart anomalies in which right atrial dilatation occurs are frequently associated with atrial tachyarrhythmias, especially atrial fibrillation or flutter [1][2][3][4]. Acquired cardiac lesions that produce isolated right atrial dilatation, such as traumatic tricuspid insufficiency, are also frequently associated with atrial fibrillation or flutter [6]. Ebstein’s anomaly was the most frequent diagnosis in the present series. In this anomaly, deformity and displacement of the tricuspid valve cause severe tricuspid valve insufficiency and right atrial dilatation. There is also an attenuation and dilatation of the atrialized right ventricle and hypertrophy and dilatation of the functioning right ventricle. The pathology of this disorder provides an anatomic substrate that increases susceptibility to conduction abnormalities and both atrial and ventricular tachyarrhythmias [1][7]. An additional source of tachyarrhythmias in Ebstein’s anomaly is the presence of atrioventricular accessory conduction pathways, which were present in 14% to 15% of our surgical series [1][8]. Of 306 patients who underwent operation for Ebstein’s anomaly, 44 had concomitant surgical ablation of one or more accessory conduction pathways. The pathways were successfully ablated in all patients and survival was 100% at 10 years [8]. None of the atrial tachyarrhythmias in this review were related to accessory conduction pathways.

The frequency of atrial arrhythmias in patients with Ebstein’s anomaly varies among reported series, in part because of the ages of the patients in the series. The incidence of arrhythmias increases with age [7][9]. The mean duration of paroxysmal or chronic atrial fibrillation or flutter in the present series was 14.2 years. The mean age for patients in the present review (34.9 years) is considerably older than the mean age for our entire series of patients undergoing operation for Ebstein’s anomaly (19.1 years) [1]. In a report from our institution, Oh and associates [2] reviewed 52 consecutive patients who underwent operation for Ebstein’s anomaly. In this group, 18 patients (34.6%) had documented paroxysmal supraventricular tachycardia and 10 patients (19%) had paroxysmal atrial fibrillation or flutter preoperatively. Of the 10 patients with paroxysmal atrial fibrillation or flutter, one-third continued to have symptomatic atrial tachycardia when followed up for an average of 36 months after operation. A subsequent review at our institution [3, unpublished data] compared the incidence of preoperative and postoperative arrhythmias in 188 patients who underwent operation for Ebstein’s anomaly. When patients with accessory conduction pathways were excluded, supraventricular tachycardia was present in 40% of the patients preoperatively and in 28% postoperatively during an average follow-up of 6 years. In a subgroup of 81 patients followed up for more than 6 years, there was no significant difference in the incidence of preoperative and postoperative atrial arrhythmias (42% versus 37%, respectively). These results included both persistence of preoperative atrial fibrillation or flutter and the development of new atrial fibrillation or flutter during the follow-up period.

Atrial tachyarrhythmias are poorly tolerated by patients with unrepaired congenital heart disease, and they may be a source of considerable morbidity and even mortality after successful cardiac repair [2][7]. The mechanism of death is presumably atrial fibrillation or flutter with rapid ventricular conduction or degeneration into ventricular fibrillation [7]. Treatment may require one or more antiarrhythmic drugs, most of which have a negative inotropic effect that may further depress myocardial function in patients with congenital heart disease. A pacemaker may also be required in combination with antiarrhythmic drugs for complete control of atrial tachyarrhythmias. For these reasons, although the incidence of postoperative atrial tachyarrhythmias is decreased by the standard repairs for the Ebstein and other congenital cardiac anomalies, we planned to institute a policy of performing a concomitant antiarrhythmia procedure in patients undergoing repair of congenital heart disease in whom atrial fibrillation or flutter was present on the basis of right atrial dilatation.

The Cox maze III operation is currently the surgical procedure of choice for management of medically refractory atrial fibrillation [5][10]. In addition to being used in patients with atrial fibrillation or flutter and no underlying structural disease, the maze procedure has been added to the operation for patients requiring mitral valve repair, closure of atrial septal defect, septal myectomy for hypertrophic obstructive cardiomyopathy, and coronary artery bypass grafting [11][12][13]. There have been two recent reports in which atrial incisions in the maze procedure have been applied to the left atrium only, with some beneficial reduction in late atrial fibrillation [14][15].

We modified the standard Cox maze III procedure by limiting the incisions to the right atrium and atrial septum. If effective, the right-sided maze would have several advantages over the standard maze operation including shorter operating time, minimizing the dissection necessary when dense adhesions are present, limiting the number of atrial suture lines, eliminating suture lines behind the heart where control of bleeding can be difficult, avoiding a possible postoperative noncontractile left atrium, and possibly decreasing the risk of systemic embolization. In addition, the decreased number of suture lines appears to decrease the tendency for postoperative fluid retention caused by a decrease in atrial natriuretic peptide. In this small series, the operation did not add to the operative or late mortality (both were zero), and there was a gratifying decrease in the incidence of atrial fibrillation or flutter from 100% to 17% (83% decrease). This decrease in preoperative supraventricular tachyarrhythmias, excluding those as a result of accessory conduction pathways, is greater than the reduction from 40% to 28% (30% decrease) that has been noted with the standard repair for Ebstein’s anomaly [3] and other congenital lesions with right atrial enlargement in our experience.

For the standard maze procedure, early (<3 months) arrhythmias are common, ranging from 40% to 50% [10]. With the passage of time and further resolution of postoperative changes, this incidence decreases. Because all three of the arrhythmia occurrences in our review were early (mean of 4.6 months), this incidence may decrease in the months ahead. It remains to be seen how much improvement the patients in this review will experience and what the incidence of late arrhythmias will be. In the meantime, this experience has been encouraging enough that we plan to continue with concomitant right-sided maze procedures in patients with atrial fibrillation or flutter associated with a large right atrium and structural heart disease.

	Acknowledgments

Top Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 
We thank Merrilee S. Rogotzke and Susan L. Burns for excellent secretarial support and Betty J. Anderson for outstanding data management support in the preparation of the manuscript.

	References

Top Abstract Introduction Patients and Methods Results Comment Acknowledgments References

 

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3. Cordes TM, Porter CJ, Danielson GK Ebstein’s and arrhythmias: the outcome after surgical repair. J Am Coll Cardiol 1994;23:104A.
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6. Van Son JAM, Danielson GK, Schaff HV, Miller FA Traumatic tricuspid valve insufficiency: experience in thirteen patients. J Thorac Cardiovasc Surg 1994;108:893-898.[Abstract/Free Full Text]
7. Olson TM, Porter CJ Electrocardiographic and electrophysiologic findings in Ebstein’s anomaly. Pathophysiology, diagnosis, and management. Prog Pediatr Cardiol 1993;2:38-50.
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