Congenital and surgically acquired Wolff-Parkinson-White syndrome in patients with tricuspid atresia

OBJECTIVES: There are reports associating an increased incidence of Wolff-Parkinson-White syndrome with tricuspid atresia. Here we report on electrophysiologic studies in patients with tricuspid atresia and Wolff-Parkinson-White syndrome after the Fontan operation. In these patients the atrial arrhythmia often seen in patients undergoing the Fontan operation triggered atrioventricular re-entrant tachycardia or caused life-threatening arrhythmias. METHODS: Five patients with tricuspid atresia after palliation with a modified Fontan operation (atrioinfundibular connections) and Wolff-Parkinson-White syndrome are presented. RESULTS: Four of these patients had symptomatic paroxysmal orthodromic atrioventricular re-entrant tachycardia and a history of syncope; one of them additionally had atrial flutter with 2:1 conduction to the ventricle. A fifth patient presented with a life-threatening broad-complex tachycardia. In electrophysiologic studies an accessory pathway was localized in the right septal area in 3 patients. In 2 patients the accessory atrioventricular pathways were created by means of surgical intervention, connecting the right atrial appendage to the right ventricular outflow tract. All patients could be managed successfully by means of catheter ablation. CONCLUSIONS: In patients with tricuspid atresia, there are congenital and surgically acquired accessory pathways responsible for the increased rate of Wolff-Parkinson-White syndrome. Both types of accessory pathways can and should be treated by means of catheter ablation because atrial arrhythmia often seen in patients undergoing the Fontan operation can trigger atrioventricular re-entrant tachycardia or cause life-threatening tachycardia. Congenital accessory pathways should be excluded carefully before surgical intervention for total cavopulmonary anastomosis in patients with tricuspid atresia.     

A successful surgical treatment of WPW syndrome associated with tricuspid atresia

The occurrence of both tricuspid atresia and WPW syndrome is quite rare. To our knowledge, only two operated cases have been reported. In this report, a 8-year-old boy with this combination was reported. Tachycardia via the right side accessory pathway was for the first time pointed out after Kreutzer's operation. Epicardial mapping showed the earliest excitation at the right free wall. When fatty tissue around this area was removed by CUSA, a large cardiac vein appeared. By holding this vein with a forceps, the delta wave disappeared temporarily. ACP seemed to be present near this vein. Permanent disappearance of the delta wave was obtained after ligation and division. Four months after surgery, he is free from tachycardia attacks.     

Surgical treatment of Wolff-Parkinson-White syndrome combined with variant angina pectoris

Seven patients with coexistent Wolff-Parkinson-White (WPW) syndrome and variant angina pectoris underwent surgical interruption of an accessory atrioventricular conduction pathway. In two of these patients, perioperative coronary artery spasm occurred. One patient had a perioperative myocardial infarction and the other patient treated successfully had no infarction. Nitroglycerin and nifedipine were administered during and following operation in the other 5 patients without perioperative coronary artery spasm. No adverse effect was observed in the intraoperative electrophysiological study. Combined therapy of intravenous nitroglycerin administration and sublingual nifedipine administration are useful in the perioperative management of patients with coexistent WPW syndrome and variant angina pectoris.     

Pierre Robin syndrome combined with unilateral choanal atresia

A case of Pierre Robin syndrome (mandibular hypoplasia, cleft palate, and glossoptosis), associated with a unilateral choanal atresia in a full-term infant, is described. The pathogenesis of this combined anomaly is discussed through review of the embryologic development of the craniofacial structures. Diagnostic and management alternatives are reviewed.     

Surgical treatment of tricuspid atresia

Despite increasing experience with the surgical treatment of tricuspid atresia, controversy exists regarding the early and late results of the various surgical options. Between January 1, 1967, and September 1, 1982, 92 patients underwent 147 operations for tricuspid atresia. Eighty-five patients underwent 1 or more palliative operations (108 procedures), with 8 hospital deaths (9%; confidence limits [CL], 6 to 14%). Thirty-eight patients underwent 44 classic (Blalock-Taussig or Gore-Tex) shunts, with 3 early (7%; CL, 3 to 13%) and 3 late deaths. Actuarial survival at 5 years was 78%, but only 56% were alive and free from having to undergo further operation at 5 years. Thirty-seven patients underwent a Fontan procedure, with 5 hospital deaths (14%; CL, 8 to 22%). Of these 37 patients, 34 (92%) had a nonvalved connection between the right atrium and the right ventricular infundibulum or the pulmonary artery. Incremental risk factors for hospital mortality after the Fontan procedure in patients with tricuspid atresia as well as other cardiac anomalies include young age (p = 0.0003), diagnosis other than tricuspid atresia (p = 0.03), and complex associated procedures (p = 0.02). During the year 1983, hospital mortality was 7% (1 out of 14; CL, 1 to 22%) for the Fontan procedure without complex additional procedures. Actuarial survival after a Fontan procedure was 71% at 5 years, with 3 patients requiring late reoperation. Of the survivors, 96% are in New York Heart Association Functional Class I or II. The Fontan procedure without a valve offers good intermediate survival, good functional results, and few reoperations. In patients with diminished pulmonary blood flow, a classic shunt also provides good palliation and good intermediate survival, but there is a necessity for additional operations in many patients within 5 years.     

Surgical repair of tricuspid atresia

Surgical repair of tricuspid atresia has been carried out in three patients; two of these operations have been successful. A new surgical procedure has been used which transmits the whole vena caval blood to the lungs, while only oxygenated blood returns to the left heart. The right atrium is, in this way, `ventriclized', to direct the inferior vena caval blood to the left lung, the right pulmonary artery receiving the superior vena caval blood through a cava-pulmonary anastomosis. This technique depends on the size of the pulmonary arteries, which must be large enough and at sufficiently low pressure to allow a cava-pulmonary anastomosis. The indications for this procedure apply only to children sufficiently well developed. Younger children or those whose pulmonary arteries are too small should be treated by palliative surgical procedures.     

Surgical management of tricuspid atresia

Tricuspid atresia is one of the less common forms of congenital heart disease. The results of palliative surgery in 72 children are presented. Cardiac catheterization and angiocardiography are essential for precise definition of the anomaly. There is a 20% incidence of obstruction at atrial septal level. Closed atrial septostomy is of value in such cases in infancy. In most there is a reduced pulmonary blood flow. This may be increased by anastomosis of either the superior vena cava or a systemic artery to the pulmonary artery. The caval anastomosis, while having theoretical advantages, is not always possible, especially in small infants. In this series systemic arterial shunts have given at least comparable results, suggesting that both techniques are of value with this anomaly.     

Myocardial infarction after cryoablation surgery for Wolff-Parkinson-White syndrome

A 48-year-old woman with Wolff-Parkinson-White syndrome underwent surgical division of the accessory pathway in the left lateral wall. At 6 months after the procedure, she developed dyspnea and chest oppression. Coronary angiography revealed total occlusion in the left circumflex coronary artery (segment 13) at the exact site where cryoablation had been performed. The coronary occlusion was treated with an intracoronary bolus injection of urokinase (960,000 U) and subsequent percutaneous transluminal balloon angioplasty. No significant residual stenosis remained after the balloon angioplasty, and no further evidence of myocardial ischemia was noted for 13 years to date after the procedure.     

Anaesthetic management of phaeochromo-cytoma associated with tricuspid atresia

The anaesthetic management of a patient with phaeochromocytoma, tricuspid atresia and pulmonary vascular stenosis is reported. The patient received no preoperative preparation with adrenergic blockers. Anaesthesia was induced and maintained with fentanyl, diazepam and sevoflurane. Intraoperative blood pressure was controlled with sodium nitroprusside, sevoflurane, phentolamine, and propranolol. For hypotension after resection of the tumour norepinephrine was required. This patient did not have a systemic to pulmonary shunt procedure performed, so the maintenance of pulmonary blood flow in the presence of haemodynamic instability during operation for phaeochromocytoma was a major concern. Monitoring of oxyhaemoglobin saturation (SpO2) with a pulse oximeter was considered to be useful because SpO2 may reflect pulmonary flow. During serious haemodynamic disturbances due to the manipulation of the tumour, the heart rate was inversely correlated with SpO2, but the relationship between mean arterial pressure and SpO2 was weak. Therefore, control of heart rate appeared to be more important than control of blood pressure in this case.     

Successful treatment of Wolff-Parkinson-White syndrome with concomitant mitral stenosis by simultaneous surgery

We report the case of a 61-year-old man with type B Wolff-Parkinson-White (WPW) syndrome associated with a right atrioventricular (AV) accessory pathway and concomitant mitral stenosis, who underwent successful operative treatment by simultaneous surgery. His preoperative course had been characterized by cardiac failure and repeated episodes of atrial tachyarrhythmia, in the form of fibrillation and flutter, which were difficult to control by conventional medication. Preoperative electrocardiograms (ECGs) had suggested that the accessory pathway was located in the right posterior to posteroseptal wall; however, at the time of surgery, epicardial electrophysiological mapping with sock electrodes revealed a preexcitation area in the AV groove at the lateral right margin of the heart. This discrepancy was thought to have been due to the presence of mitral stenosis or multiple accessory pathways. Thus, division and cryoablation of the accessory pathway by an endocardial approach, in addition to mitral valve replacement, were performed under cardiopulmonary bypass. His postoperative course was uneventful, and subsequent ECGs revealed that the delta waves had disappeared. The successful outcome of this patient demonstrates the effectiveness of simultaneous surgery for WPW syndrome associated with valvular disease.     

Coronary sinus septal defect associated with tricuspid atresia

Coronary sinus septal defects are unusual congenital anomalies that may assume particular clinical importance in patients undergoing a modified Fontan procedure. Two of 10 patients with tricuspid atresia who underwent a modified Fontan procedure between January, 1984, and December, 1984, were noted to have coronary sinus septal defects in the early postoperative period. A pathologic review of 159 specimens of tricuspid atresia revealed coronary sinus septal defects in four. Although infrequent, coronary sinus septal defects may permit significant persistent right-to-left interatrial shunting after a modified Fontan procedure. Ideally, preoperative recognition allows for selective direct closure or closure of the coronary sinus orifice. Alternatively, routine baffling of the coronary sinus into the left atrium obviates this potential source of interatrial communication.     

A case of tricuspid atresia with unroofed coronary sinus

Tricuspid atresia with unroofed coronary sinus is an extremely rare cardiac anomaly. A 15-year-old boy who was successfully operated was suspected this disease preoperatively. We speculated this disease by right atrial angiography and underwent the direct suture closure of a coronary sinus defect and Fontan's operation. When we perform Fontan's operation on tricuspid atresia, we should take the existence of unroofed coronary sinus into our consideration. The echocardiography, cardiac catheterization and angiography, especially, right atrial angiography had to be carried out preoperatively. During the operation, it is important to check over the influence of cardioplegic solution from the ostium of coronary sinus. If not, elaborate inspection have to be done on the back wall of the left atrium through ASD to search for abnormal communication directly or by inserting sound from coronary sinus.     

Wolff-Parkinson-White syndrome

A patient with Wolff-Parkinson-White syndrome, susceptible to tachydysrhythmias , presented for eye surgery under general anaesthesia. He developed a supraventricular tachycardia refractory to a variety of treatments. Phenylephrine consistently abolished the dysrhythmia, probably by acting directly to stimulate the arterial baroreceptors and hence vagal output. Vasopressor therapy should be considered in Wolff-Parkinson-White syndrome tachyarrhythmias, when circumstances permit.