Tricuspid atresia

Background: Tachyarrhythmia has been thought to be an absolute contraindication for the Fontan operation. Patients and methods: Three patients, 22, 9, and 11 years of age, diagnosed as having atrioventricular atresia combined with the Wolff-Parkinson-White syndrome underwent surgical treatment. Each had drug-resistant atrioventricular tachycardia that required direct cardioversion. Two patients with tricuspid atresia had an intermittent right-sided accessory pathway (ACP), and one with mitral atresia had a concealed left-sided ACP. The ACP was divided using an epicardial approach in two patients and an endocardial approach in one. Simultaneously, the Fontan operation was performed with atrioventricular connection (modified Fontan operation) in one patient, and a total cavopulmonary connection performed in another patient. In the remaining patient, ACP division was performed 3 years after the Fontan operation.

Results: There was no early death or other fatal complication, and the hemodynamic results were excellent. During the mean follow-up period of 68 months (range, 5 to 127 months), there has been no late death or recurrence of tachyarrhythmia.

Conclusion: Tachyarrhythmias caused by ACPs are not contraindications for the Fontan operation. Concomitant surgery is advocated, as excellent short- and long-term results may be expected in these patients. (Chest 1995; 107.669-73)

ACP=accessory pathway; AV=atrioventricular; AVRT= atrioventricular tachycardia; WPW syndrome=Wolff. Parkinson-White syndrome

Key words: Fontan operation; surgical treatment; tachycardia WPW syndrome

Since the first clinical success with total right heart bypass for treatment of tricuspid atresia was reported by Fontan and Baudet[1] in 1971, there have been numerous modifications of this procedure,[2-5] and this operation has been widely used as a functional corrective procedure not only in tricuspid atresia but also in other complex congenital heart diseases.[4,6,7] Choussat et al[8] provided technical guidelines for a relatively low-risk operation. One criterion is that sinus rhythm be present preoperatively, and tachyarrhythmia was formerly thought to be an absolute contraindication for the Fontan operation. We performed simultaneous Fontan operation with surgical division of the accessory pathway (ACP) in three patients with atrioventricular atresia associated with Wolff-Parkinson-White (WPW) syndrome. This report describes our successful surgical clinical experience with these patients.

METHODS

CASE 1

A 22-year-old man was referred to us for simultaneous surgery for tricuspid atresia and WPW syndrome (Table 1). He had been cyanotic since birth. His first episode of tachycardia with maximum heart rate of 175/min had occurred at the age of 15 years, and WPW syndrome of the right intermittent type had been diagnosed based on his electrocardiogram. The clinical diagnosis of tricuspid atresia without transposition of the great arteries, ventricular and atrial septal defects, and pulmonary stenosis (type 1b) had also been confirmed using catheterization and angiocardiogram. Thereafter he had been treated with multiple drugs, but atrioventricular tachycardia (AVRT) recurred despite medical treatment, and direct cardioversion was required to terminate the AVRT. At the age of 19 years, the patient required additional rapid atrial pacing therapy using an inductive radiofrequency atrial pacemaker (ATRICON, Sanei, Tokyo, Japan). This pacemaker functioned effectively for only 10 months, thereafter, atrial fibrillation with a rapid ventricular response (280 ms shortest R-R interval) was induced easily by activation of the pacemaker transmitter. Preoperative electrophysiologic study demonstrated the presence of right posterior ACP.

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A median sternotomy was performed while the patient was under general anesthesia. Using six paired bipolar electrodes, the epicardial earliest excitation area was detected in the right posterior atrioventricular (AV) groove between the atrium and the small rudimentary ventricular chamber (Fig 1). Via the endocardial approach, a right atriotomy was performed along the AV groove in the earliest excitation site, and the incision was extended to the crux. Cryocoagulation (2 min at 60[degrees]C) was performed around the edge of the coronary sinus. The tricuspid atresia was repaired using a right atrial and small right ventricular connection with a 25-mm Hancock porcine-valved conduit. Closure of the atrial septum (2.5X2.0 cm) and ventricular septal defect 3.OX2.5 cm) with a Dacron patch was performed concomitantly.

CASE 2

A 9-year-old boy was referred to us for surgical division of an ACP. He had been cyanotic since his birth and was incapacitated due to dyspnea on exertion. At the age of 5 years, the patient was clinically diagnosed as having tricuspid atresia with d-transposition of the great arteries, a ventricular septal defect, and pulmonary stenosis (type 2b) that were confirmed by catheterization and angiocardiogram. At the age of 7 years, he had undergone a Fontan operation, in which an atrial appendage was anastomosed directly to his own pulmonary artery at another institution. Two days after the Fontan operation, WPW syndrome of the right intermittent type was first identified electrocardiographically. Despite multiple drug therapy, AVRT with maximum heart rate of 170/min occurred because of hemodynamic deterioration. However, AVRT recurred despite medical therapy and direct cardioversion was required to terminate the AVRT. Preoperative electrophysiologic study demonstrated that the earliest excitation site was located on the right anterolateral wall.

Under general anesthesia, the patient underwent reoperation through a median sternotomy. Epicardial mapping using six paired bipolar electrodes revealed that both the antegrade and retrograde earliest excitation area was located on the right anterolateral AV groove. Because of severe adhesion of the heart, the epicardial approach was selected. After epicardial fat pad dissection, a small vein crossing the AV grove of the earliest excitation area was observed. Lifting of this vein with a forceps led to a temporary cessation of the delta wave on the electrocardiogram. This finding let us to strongly suspect that the ACP might lie along this vein. Permanent cessation was attained with ligation and division of this vein, including the perivascular tissue.

CASE 3

An 11-year-old girl underwent simultaneous surgery, including Fontan procedure and ACP division. She had been brought to us at the age of 5 months with signs of progressive cyanosis. At the age of 3 years, she had undergone Glenn anastomosis (superior vena cava-right pulmonary artery anastomosis). At the age of 5 years, she experienced her first tachycardia attack, with maximum heart rate of 250/min. Since the age of 9 years, despite medical treatment with digitalis, propranolol, disopyramide, verapamil, and adenosine triphosphate, she had been admitted to the hospital more than 50 times for tachycardia and hemodynamic deterioration. Preoperative electrophysiologic study demonstrated the presence of concealed ACP. The clinical diagnosis of mitral atresia with a double-outlet right ventricle and pulmonary stenosis was confirmed by catheterization and angiocardiogram. Because the AVRT had became incessant and sufficient cardiac output could not be obtained, she required emergency surgery.

Under general anesthesia, she was operated on through a median sternotomy using the technique shown in Figure 2. The heart was exposed through a median sternotomy. A mat electrode composed of 24 pairs of bipolar electrodes with a 2-mm interelectrode distance was placed in the AV groove, and local electrograms were recorded simultaneously. A computerized mapping system (HPM-7100, Fukuda Denshi Co Ltd, Tokyo, Japan) was used to record the epicardial potentials and to process and display these data. The earliest excitation area was detected during AVRT on the left lateral wall. Temporary cessation was obtained by dissection of the AV goove of the earliest excitation area. Therefore, this area was cryoablated for 2 min at -- 100[degrees]C. Surgical correction was also achieved by total cavopulmonary connection. Right atriotomy was performed under cardiopulmonary bypass. The assessment of the intracardiac anatomy revealed mitral atresia with hypoplastic left atrium, double outlet of the right ventricle with rudimentary left ventricle, tricuspid type atrioventricular valve, coronary sinus defect, and pulmonary stenosis. The main pulmonary artery was transected and oversewn. Then, an 18-mm polytetraflorethylene (W. L. Gore & Associates, Elkon, Md) graft was sutured at the orifice of the inferior vena cava and the distal end of the main pulmonary artery. Tricuspid annuloplasty was also performed using DeVega's technique for treatment of tricuspid valve insufficiency (Fig 2).

RESULTS

Location of ACP

In each patient, the ACP was located in the AV groove between the atrium and the small rudimentary ventricular chamber (Fig 1). In the two patients with tricuspid atresia (patients 1 and 2), the ACP was located on the right posterior and right anterolateral walls. In patient 3, it was located on the left lateral free wall (Fig 3).

Surgical Result

There was no postoperative surgical death (Table 2). Weaning from extracorporeal circulation was uneventful, and there was no acute complication. During the follow-up periods of 10 years 7 months, 5 years 2 months, and 5 months, the patients have led normal lives without requiring any antiarrhythmic drug and have experienced no episode of tachycardia or other cardiac event.

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DISCUSSION

Combination of Atrioventricular Atresia and WPW Syndrome

Our three patients had atrioventricular atresia with associated WPW syndrome. At present, only a few other such patients who have undergone surgery[9,10] have been described in the literature. Dick et al[11] and Razzouk et al[12] each described a patient who had undergone surgery with tricuspid atresia with WPW syndrome. In all four patients with tricuspid atresia, the ACP was located on the right side (our patient 1, posterior wall; our patient 2, anterolateral wall; the patient of Dick et al had a posterior ACP; and the patient of Razzouk et al had an anterior ACP). Our patient with mitral atresia (patient 3) had a left lateral ACP. The observation that most patients with WPW syndrome associated with Ebstein's anomaly have an ACP on the right side[13,14] suggests that the ACP is related to the developmental tricuspid valve anomaly. The hypothesis that developmental anomalies of atrioventricular valves may be associated with ACP is further supported by the observation that patients with tricuspid atresia have an ACP on the right side while those with mitral atresia have an ACP on the left side.

Drug therapy for WPW syndrome associated with congenital heart disease is problematic because of the pharmacologic actions of antiarrhythmic agents and digitalis. Antiarrhythmic agents may induce heart failure because of negative inotropic effect, while digitalis shortens the antegrade refractory period in ACP. A simultaneous procedure for the WPW syndrome and congenital heart disease is curative for both disorders.

Fontan Operation and Tachycardia

The Fontan operation was successful in our two patients in whom the ACP was divided concomitantly. The same surgical success was reported previously only by Dick et al[11] in 1981.

Since October 1973, we have operated on 457 patients with WPW syndrome at Kanazawa University and Toyama Medical and Pharmaceutical University. Of these patients, 63 underwent concomitant surgery for another cardiac disease. Of course, prolongation of operation time is a concern; however, there was no operative death related to concomitant surgery.[15]

There was also a report that patients with WPW syndrome underwent preoperative ablation by radiofrequency techniques in the catheterization laboratory prior to Fontan operation, an approach that considerably simplified the operation.[16] However, in a patient such as patient 2 with mitral atresia who had Glenn anastmosis previously and low cardiac output state due to incessant tachycardia, it is difficult and dangerous to perform catheter ablation for anatomic and hemodynamic reasons. In addition, radiation exposure may be another disadvantage factor of catheter ablation in pediatric patients.17

We conclude from our surgical experience with atrioventricular atresia combined with WPW syndrome, tachyarrhythmia itself should not contraindicate the Fontan operation, Concomitant procedure of both Fontan operation and division of the ACP is indicated in selected patients and excellent long-term results may be obtained.

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REFERENCES

[1] Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971; 26:240-48 [2] Kreutzer G, Galindez E, Bono H, dePalma C, Laura JP. An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg 1973; 66:613-21 [3] Bjork VO, Olin CL, Bjarke BB, Thoren CA. Right atrial-right ventricular anastmosis for correction of tricuspid atresia. J Thorac Cardiovasc Surg 1979; 77:452-58 [4] Gale AW, Danielson GK, McGoon DC, Mair DD. Modified Fontan operation for univentricular heart and complicated congenital lesions. J Thorac Cardiovasc Surg 1979; 78:831-38 [5] de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations: experimental studies and early clinical experience. J Thorac Cardiovasc Surg 1988; 96:682-95 [6] Puga FJ, Chivarelli M, Hagler DJ. Modifications of the Fontan operation applied to patients with left atrioventricular valve atresia or a single atrioventricular valve. Circulation 1987; 76(suppl 3):53-60 [7] Matsuda H, Kawashima Y, Kishimoto H, Hirose H, Nakano S, Katoh H, et al. Problems in the modified Fontan operation for univentricular heart of the right ventricular type. Circulation 1987; 72(suppl 3);46-52 [8] Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H. Selection criteria for Fontan's procedure. In: Anderson RH, Shineboune EA, eds. Pediatric cardiology. Edinburgh:Churchill Livingstone, 1978; 559-66 [9] Misaki T, Iwa T, Kuzawa Y, Murakami S, Seki M, Miyahara K. Simultaneous surgery for Wolff-Parkinson-White syndrome combined with tricuspid atresia. J Jpn Assoc Thorac Surg 1986; 34:261-66 [10] Ohtake H, Iwa T, Misaki T, Watanabe G, Mukai A, Tsubota M, et al. A successful surgical treatment for WPW syndrome associated with tricuspid atresia. Jpn J Thorac Surg 1990; 43:57-60 [11] Dick M II, Behrendt DM, Byrum CJ, Sealy WC, Stern AM, Hess P, et al. Tricuspid atresia and the Wolff-Parkinson-White syndrome: evaluation methodology and successful surgical treatment of the combined disorders. Am Heart J 1981; 101:496-500 [12] Razzouk AJ, Gow R, Finley J, Murphy D, Williams WG. Surgically created Wolff-Parkinson-White syndrome after Fontan operation. Ann Thorac Surg 1992; 54:974-77 [13] Sealy WC, Gallagher JJ, Pritchett ELC, Wallace AG. Surgical treatment of tachyarrhythmia in patients with both an Ebstein's anomaly and a Kent bundle. J Thorac Cardiovasc Surg 1978; 75:847-53 [14] Iwa T, Mitsui T, Misaki T, Mukai T, Magara T, Kamata E. Radical surgical cure of Wolf-Parkinson-White syndrome: the Kanazawa experience. J Thorac Cardiovasc Surg 1986; 91:225-33 [15] Iwa T, Misaki T, Tsubota M, Ishida K. Surgical management of tachyarrhythmias. Am J Cardiol 1989; 64:86J-91J [16] Van Here GF, Lesh MD, Stanger P. Radiofrequency catheter ablation of supraventricular arrhythmias in patients with congenital heart disease: results and technical considerations. J Am Coll Cardiol 1993; 22:883-90 [17] Misaki T, Watanabe G, Iwa T, Matsunaga Y, Ohtake H, Tsubota M, et al. Surgical treatment of Wolff-Parkinson-White syndrome in infants and children. Ann Thorac Surg 1994; 58:103-07

(*) From the Department of Surgery 1, Toyama Medical and Pharmaceutical University (Drs. Misaki and G. Watanabe); the Department of Surgery 1, Kanazawa University School of Medicine (Drs. Iwa and Y. Watanabe); and the Department of Cardiothoracic Surgery, Kobe Children's Hospital (Dr. Yamaguchi), Japan. Manuscript received January 14, 1994; revision accepted June 2. Reprint requests: Dr. Misaki, Dept. of Surgery, Toyama Medical and Pharmaceutical University, Sugitani 2630, Toyama 930-01 Japan

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