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Ann Thorac Surg 2001;71:919-921
© 2001 The Society of Thoracic Surgeons


Original article: cardiovascular

Renewal of the Fontan circulation with concomitant surgical intervention for atrial arrhythmia

Youichi Kawahira, MDa, Hideki Uemura, MDa, Toshikatsu Yagihara, MDa, Yoshiro Yoshikawa, MDa, Soichiro Kitamura, MDa

a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan

Accepted for publication October 18, 2000.

Address reprint requests to Dr Kawahira, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka, 565-8565 Japan
e-mail: ykawahir{at}hsp.ncvc.go.jp


    Abstract
 Top
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 
Background. Atrial arrhythmia remains one of the major complications in the longer term after the Fontan procedure.

Methods. Conversion to total cavopulmonary connection was carried out concomitantly with surgical intervention for atrial arrhythmia in 4 patients undergoing the Fontan procedure by atriopulmonary connection and having continual atrial fibrillation or flutter in the longer term after the initial procedure.

Results. The surgical intervention restored sinus rhythm. Transient atrial fibrillation occasionally occurred after the reoperation in 1 patient in whom duration of preoperative arrhythmic period had been 6 years, and defibrillation was needed twice. In the other 3 patients, no episodes of paroxysmal arrhythmia have been noted. Subsequent to renewal of the Fontan circulation, cardiac index increased, with systemic venous pressure decreasing. All 4 patients are currently doing well with their functional status of New York Heart Association functional class I.

Conclusions. Combination of conversion to total cavopulmonary connection and concomitant surgical intervention for atrial arrhythmia is effective, when used appropriately and in a timely manner in patients with atrial arrhythmia in the longer term after the initial Fontan procedure by atriopulmonary connection.


    Introduction
 Top
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 
Atrial fibrillation or flutter remains one of the major complications in the longer term after the Fontan procedure by atriopulmonary connection, and often produces morbidity and mortality [1]. Conversion to total cavopulmonary connection is known to be efficient to treat atrial arrhythmia [27]. To make this approach more secure, surgical intervention for arrhythmia can be concomitantly used [8]. Our experience is herein described.


    Patients and methods
 Top
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 
Forty-four patients survived the Fontan procedure by conventional atriopulmonary connection before 1991 at our institute. Of these, 8 patients had episodes of atrial flutter or fibrillation in the longer term. One patient died of arrhythmia 12 years after operation. Medication has been so far effective in another 3 patients. In the remaining 4, medication did not prove very effective, and clinical symptoms, such as general fatigue and palpitation, were obvious with the patients in New York Heart Association functional status III. On catheterization, cardiac index was calculated as 1.5 ± 0.6 (1.2 to 1.7) L · min-1 · m-2, with systemic venous pressure being 16 ± 2 (15 to 18) mm Hg. We proceeded, therefore, to surgical intervention. All 4 patients had tricuspid atresia and pulmonary stenosis. Ventriculoarterial connections were concordant in 3 patients, and double outlet from the incomplete morphologically right ventricle in 1 patient. The Fontan circulation had been initially established by direct anastomosis between the pulmonary arteries and the right atrial appendage. Preoperative patients’ data were shown in Table 1. In 2 patients with atrial flutter, a macroreentry circuit had been detected around the terminal crest by preoperative electrophysiologic study.


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Table 1. Preoperative Characteristics of 4 Patients With Atrial Arrhythmias After Atriopulmonary Connection

 
Operative procedures were carried out through the median sternotomy. Initially, on cardiopulmonary bypass with the heart beating, the continuity previously constructed between the right atrium and the pulmonary arteries was divided. The superior caval vein was transected, and its distal stump was anastomosed to the right pulmonary artery in an end-to-side fashion. The proximal stump of the transected superior caval vein was oversewn. Greater parts of the free wall of the markedly enlarged right atrium were extensively resected for plication. Particular attention was paid to avoid injury to the sinus node. Subsequently, with the heart arrested, induced by crystalloid cardioplegic solution, the atrial septum was resected as much as possible. In patients with atrial fibrillation, a modification of the Maze procedure with the aid of cryoablation [9, 10] was used onto both of the atria. In those with atrial flutter, cryoablation of the atrial wall was carried out so as to ablate the common pathway of electrical circuits producing atrial flutter (Fig 1). In addition, the muscular floor between the coronary sinus and the dimple of the atretic tricuspid valve was cryoablated. Finally, a channel draining the inferior caval vein was constructed using a polytetrafluoroethylene tube graft. In patients 1 and 4 undergoing the renewal of the Fontan circulation before 1996, a 22-mm diameter tube was placed intraatrially [11]. We have not used the extracardiac Fontan procedure exclusively until 1997. The prosthesis was anastomosed to the very orifice of the inferior caval vein, and to the incised pulmonary arteries together with the incised right atrial appendage. Thus, no part of the atrial wall was exposed to the high-pressure venous channel, and the sinus node was placed within the low-pressure atrial chamber. In the other 2 patients (Nos. 2 and 3), the inferior caval vein was divided from the right atrium leaving a sleeve of musculature around its orifice, and a 24-mm diameter prosthetic tube was interposed between the interior caval vein and the pulmonary arteries in an extracardiac fashion. Aortic cross-clamping time and cardiopulmonary bypass time were 67 ± 34 minutes (range, 28 to 112 minutes) and 228 ± 68 minutes (range, 191 to 302 minutes), respectively.



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Fig 1. The sites of cryoablation through the incision to the right atrium were shown in this schema. For modification of the Maze procedure, cryoablation was added at the muscular floor between the coronary sinus and the dimple of the atretic tricuspid (1). In patients with atrial flutter, the common pathway of electrical circuits was cryoablated within the right atrium (2). Maneuver onto the muscular floor between the coronary sinus and the dimple of the atretic tricuspid was deemed essential also in this setting.

 
Postoperatively, warfarin and antiplatelet agent were administered for 1 year. The latter has been continued as anticoagulation medication also in the longer term.


    Results
 Top
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 
All patients were restored to sinus rhythm postoperatively. Paroxysmal atrial arrhythmia has not recurred in all except for patient 1, in whom transient atrial fibrillation occasionally occurred and defibrillation was carried out twice. The 3 patients with no postoperative arrhythmic episodes are currently taking no medication for arrhythmia. Digoxin and disopyramide has been continued to be administered to patient 1. Cardiac index increased postoperatively from 2.1 ± 0.6 (1.7 to 2.5) L · min-1 · m-2, with systemic venous pressure decreasing to 6 ± 2 (4 to 7) mm Hg. All 4 patients are currently doing well with the follow-up of 12 to 60 months (34 ± 25 months), and their functional status has improved to New York Heart Association functional class I.


    Comment
 Top
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 
Conversion of the Fontan circulation from the conventional atriopulmonary connection to total cavopulmonary connection has been used to improve atrial complications including dilatation or thrombosis of the atria. In some reports [3, 8], it was stated that the Fontan renewal was useful in association with cryoablation of arrhythmic circuits for treatment of atrial arrhythmia. Indeed, the mere conversion without direct surgical intervention for atrial arrhythmia provided an effective rate in 46% of patients (13 of 28 patients) as an antiarrhythmic therapy [37], whereas the procedure with cryoablation was effective for supraventricular tachyarrhythmia in 82% of patients (9 of 11) [8] (p = 0.045 by {chi}2 test). Atrial flutter with a known reentry circuit can be undoubtedly suppressed by ablation of the pathway. However, it may remain contentious, in contrast, whether atrial fibrillation can be justifiably treated with the Maze procedure or not in patients with cardiac malformations unsuitable for biventricular repair because of abnormal atrioventricular connections.

Because no clear indication for the use of the Maze procedure has been defined in patients undergoing the Fontan procedure, the general criteria previously proposed [10] were applied in our present series. We added cryoablation between the coronary sinus and the dimple of the atretic tricuspid valve. In patients with a normally structured heart, an electrical circuit around the tricuspid valve can be readily ablated at the vestibule of the tricuspid valve around the orifice of the coronary sinus. In those with tricuspid atresia, the right atrium has a muscular floor at the atretic atrioventricular junction without an orifice of the tricuspid valve. Accordingly, a reentry circuit, if any, is unlikely to be ablated until the maneuver is extended to the point of the dimple where atrial musculature is lacking. The surgeon must be careful of the atrioventricular node. Its location is not always clear in the setting of tricuspid atresia, particularly when the right atrium is very dilated.

Another surgical device for arrhythmia could be additional cryoablation onto the lateral wall of the right atrium crossing the terminal crest. An arrhythmic circuit is known to be present around the terminal crest, and, in 2 of our patients having atrial flutter, such a circuit had been identified by preoperative electrophysiologic study. Nonetheless, we resected extensively the free wall of the markedly enlarged right atrium, as well as the atrial septum. Because of this, cryoablation was needed just for a point around to the terminal crest. Extensive resection of the right atrial wall seemed ideal, as trabeculation of the atrium accommodated considerable thrombi in 2 patients. The alternative atrial cavity for the systemic circulation should not contain thrombi within it. The size of the alternative atrial chamber, furthermore, should not be very large. Wall tension of the atrium can be reduced by appropriate plication. Therefore, attention must be paid so as to avoid injury to the sinus node when resecting the free wall of the right atrium. As stated in our previous articles [11], it remains our policy, even at the time of the renewal of the Fontan circulation, to place the sinus node within the low-pressure atrial chamber, rather than into the high-pressure venous channel, preferably constructing an alternative extracardiac channel for the inferior caval venous drainage [12].

Some surgeons may omit the operative maneuver of the Maze procedure on the left atrium when treating atrial arrhythmia with the Fontan circulation. Marked dilatation of the right atrium is deemed the major cause of atrial fibrillation subsequent to the Fontan procedure. The left atrium, nonetheless, can also be dilated and involved in the circuit for atrial fibrillation [10, 13]. We have made it our policy, therefore, to carry out the Maze operative maneuver not only on the right atrium but also on the left atrium. Even with the entire Maze procedure, atrial arrhythmia could recur postoperatively. In our particular patient, conversion to total cavopulmonary Fontan connection was carried out 6 years after commencement of atrial arrhythmia. In the other 3 patients with no recurrence of atrial arrhythmia, duration was shorter from the initial arrhythmic event to conversion. Other indicators proposed for better outcome after the Maze procedure [10] did not differ between our patients. Reoperation should have taken place earlier in patient 1.

Some physicians may recommend catheter ablation before surgical renewal of the Fontan circulation. Such a strategy, although we have not attempted it, may be similarly effective, and even less invasive. Our preference, nonetheless, is the surgical conversion to total cavopulmonary connection concomitantly with surgical ablation for atrial arrhythmia, as the dilated right atrium could contain thrombi, and extensive resection of such atrial wall can be part of the surgical intervention for atrial arrhythmia. In addition, if the Maze procedure is undertaken, it should be completed not only for the right atrium, but also for the left atrium. The entire Maze procedure is undoubtedly difficult to achieve by catheter ablation.

In conclusion, the efficacy of conversion from atriopulmonary connection to total cavopulmonary connection in patients with atrial fibrillation or flutter can be augmented by concomitant surgical intervention for treatment of atrial arrhythmia. To provide better results for the combined procedures, reoperation should occur within a short time period.


    References
 Top
 Abstract
 Introduction
 Patients and methods
 Results
 Comment
 References
 

  1. Gewillig M., Wyse R.K., de Leval M.R., Deanfield J.E. Early and late arrhythmias after the Fontan operation: predisposing factors and clinical consequences. Br Heart J 1992;67:72-79.[Abstract/Free Full Text]
  2. Pearl J.M., Laks H., Stein D.G., et al. Total cavopulmonary connection versus conventional modified Fontan procedure. Ann Thorac Surg 1991;52:189-196.[Abstract]
  3. Kao J.M., Alegos J.C., Gran P.W., Williams R.G., Shannon K.M., Laks H. Conversion of atriopulmonary to cavopulmonary anastomosis in management of late arrhythmias and atrial thrombosis. Ann Thorac Surg 1994;58:1510-1514.[Abstract]
  4. McElhinney D.B., Reddy V.M., Moore P., Hanley F.L. Revision of previous Fontan connections to extracardiac or intraatrial conduit cavopulmonary anastomosis. Ann Thorac Surg 1996;62:1276-1283.[Abstract/Free Full Text]
  5. Kreutzer J., Keane J.F., Lock J.E., et al. Conversion of modified Fontan procedure to lateral atrial tunnel cavopulmonary anastomosis. J Thorac Cardiovasc Surg 1996;111:1169-1176.[Abstract/Free Full Text]
  6. Van Son J.A., Mohr F.W., Hambsch J., Schneider P., Hess H., Haas G.S. Conversion of atriopulmonary or lateral atrial tunnel cavopulmonary anastomosis to extracardiac conduit Fontan modification. Eur J Cardiothorac Surg 1999;15:150-157.[Abstract/Free Full Text]
  7. Vitullo D.A., DeLeon S.Y., Berry T.E., et al. Clinical improvement after revision in Fontan patients. Ann Thorac Surg 1996;61:1797-1804.[Abstract/Free Full Text]
  8. Mavroudis C., Backer C.L., Deal B.J., Johnsrude C.L. Fontan conversion to cavopulmonary connection and arrhythmia circuit cryoablation. J Thorac Cardiovasc Surg 1998;115:547-556.[Abstract/Free Full Text]
  9. Kosakai Y., Kawaguchi A.T., Isobe F., et al. Modified maze procedure for patients with atrial fibrillation undergoing simultaneous open heart surgery. Circulation 1995;92(Suppl 2):359-364.[Abstract/Free Full Text]
  10. Kobayashi J., Kosakai Y., Nakano K., Sasako Y., Eishi K., Yamamoto F. Improved success rate of the maze procedure in mitral valve disease by new criteria for patients’ selection. Eur J Cardiothorac Surg 1998;13:247-252.[Abstract/Free Full Text]
  11. Uemura H., Yagihara T., Kawashima Y., et al. What factors affect ventricular performance after a Fontan-type operation?. J Thorac Cardiovasc Surg 1995;110:405-415.[Abstract/Free Full Text]
  12. Uemura H. The Fontan type procedure in patients with visceral heterotaxy. Cardiol Young 1998;8:419-422.[Medline]
  13. Sanfilippo A.J., Abascal V.M., Sheehan M., et al. Atrial enlargement as a consequence of atrial fibrillation; a prospective echocardiographic study. Circulation 1990;82:792-797.[Abstract/Free Full Text]



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