Expanding the indications for pulmonary valve replacement after repair of tetralogy of fallot

BACKGROUND: Insertion of a competent pulmonary valve has been advocated to reduce right ventricular volume overload associated with pulmonary regurgitation (PR) after repair of tetralogy of Fallot. However the indications, proper timing, and long-term benefits of restoring pulmonary valve function remain controversial. METHODS: Thirty-six patients (aged 15.2 +/- 9.2 years) underwent pulmonary valve implantation (31 homografts, 5 heterografts) 12.2 +/- 6.9 years after tetralogy repair. Additional surgical procedures included pulmonary artery augmentation (n = 14), closure of septal defects (n = 10), and cryoablation and endocardial resection of ventricular tachycardia (n = 2). RESULTS: All patients have had clinical improvement in their exercise capacity. Preoperative and postoperative bicycle ergometry tests in 6 patients demonstrated significant improvement in the percent of predicted peak workload (68.5% +/- 19.8% to 80.7% +/- 17.4%, p < 0.015). One midterm death occurred in a 38-year-old patient with a history of ventricular tachycardia who died suddenly 2 years after pulmonary valve insertion. Postoperative echocardiographic measurements were available in 34 patients at a mean follow-up of 5 years. There was a 30% reduction in right ventricular end-diastolic diameter indexed to body surface area after surgery (30.1 +/- 10.2 to 18.6 +/- 6.0 mm/m(2), p < 0.0001). Two patients required conduit replacements at 1 and 9 years postoperatively. CONCLUSIONS: Timely insertion of a competent pulmonary valve in children, adolescents, and young adults with significant PR after tetralogy of Fallot repair results in subjective and objective improvement in exercise capacity and is associated with reduction in right ventricle size.     

Intracardiac repair of tetralogy of fallot

One-hundred and twenty-two patients underwent intracardiac repair for tetralogy of Fallot between the ages of four and twenty-one. Isolated infundibular stenosis was seen in 63.6 per cent combined infundibular and valvular stenosis in 17.3 per cent, diffuse hypoplasia of outflow tract and main pulmonary artery in 10 per cent and isolated valvular stenosis in 9 per cent. Narrowing of right pulmonary artery was seen in two patients. Outflow patch was necessary in seventy-seven. Nineteen patients died. The causative factors were transannular patch for diffuse hypoplasia, infection and inadequate repair. Residual right ventricular hypertension was an important cause for mortality and morbidity. Myocardial blood flow was also found reduced, especially when residual right ventricular pressure was high.     

Corrective surgery for tetralogy of fallot in adolescents and adults

One hundred and twenty three patients with tetralogy of Fallot over the age of thirteen years underwent total corrective surgery between July 1968 to December 1980 in the Hong Kong University, Cardiothoracic Centre at Grantham Hospital. The hospital mortality was 10.5 per cent. The number of late deaths was three. Eighty-three of the one hundred and six survivors were available for long-term follow-up. Significant residual ventricular septal defect was found in seven patients; four of these were successfully reoperated.     

Right heart reconstruction following repair of tetralogy of fallot

Ten patients in whom tetralogy of Fallot had been repaired underwent late reconstruction of the outflow tract of the right ventricle because of poor hemodynamic results. The major hemodynamic problems that necessitated right ventricular (RV) outflow tract reconstruction were severe pulmonary insufficiency in 9 patients and pulmonary stenosis in 1. Impaired RV contractility and RV aneurysm were the most important factors prompting valve replacement for severe pulmonary insufficiency. Seven patients received a Hancock prosthesis and 3, an aortic homograft. Among the 7 patients who underwent postoperative cardiac catheterization, the surgical results were hemodynamically excellent in 2, good in 3, and unsatisfactory in 2. The management of pulmonary insufficiency in such patients is discussed.     

Surgical repair of pulmonary arterial sling associated with tetralogy of fallot

Pulmonary artery sling associated with tetralogy of Fallot was successfully repaired in 2 patients. In 1 patient, extensive reconstruction was needed for severe hypoplasia in the left pulmonary artery, followed by definitive repair. In the other patient, surgical repair was achieved in a single-stage fashion.     

136例成人法乐四联症的外科治疗

目的探讨外科治疗成人法乐四联症的经验。方法从１９７５年４月至１９９７年１２月,对１３６例１４周岁以上成人法乐四联症施行根治术,最大年龄为５２岁。室间隔缺损修补材料中,１１２例采用涤纶补片,２４例采用自体心包补片。在１２６例使用补片扩大右心室流出道的病例中,９５例采用牛心包补片,３１例采用自体心包补片。结果住院死亡５例,住院病死率为３６８％。术后发生低心排综合征１７６％、心律失常１４０％、胸内出血需再次开胸止血１１８％、胸腔积液２１３％、心包积液５９％、肺部感染１２５％、感染性心内膜炎２２％。结论成人法乐四联症根治术有其自身特点,通过改进手术技术,加强术后监护处理,可以提高手术成功率     

Simultaneous repair of pectus excavatum and tetralogy of fallot: report of a case.

We report an 18-month-old boy with the association of pectus excavatum and tetralogy of Fallot (TOF). We successfully performed simultaneous pectus repair using sternal elevation without any prosthetic support and total correction of TOF after a prior modified Blalock-Taussig shunt. Retracting a divided costo-sternal complex with a rectus abdominal flap away from the operative field before the cardiac operation provided excellent surgical exposure. The modified Blalock-Taussig shunt prior to the combined repair prevented life-threatening hypoxic spells during dissection of the deformed sternum and costochondral cartilages before institution of cardiopulmonary bypass.     

Results of total correction of tetralogy of fallot performed in adults

Today, total correction of tetralogy of Fallot is rarely performed in adults. In a 10-year period, 40 patients aged 20 to 67 years underwent intracardiac repair in our institution. Twenty-eight of them had had a palliative procedure 11 to 30 years earlier. Preoperatively, 23 patients were in New York Heart Association (NYHA) Functional Class II, 14 were in Class III, and 3 were in Class IV.

Operative mortality was 2.5% (1/40). Follow-up ranged from 1 year to 11 years (average, 3 years). One patient died of a noncardiac cause 4 years after operation. Residual cardiac defects were observed in 4 patients. Postoperatively, 30 patients were in NYHA Functional Class I, 8 were in Class II, and 1 was in Class III.

Major ventricular arrhythmias were recorded in 7 (35%) of 20 patients. Radionuclide angiography demonstrated impaired right ventricular function in 8 patients. Left ventricular impairment was present in 2.

Total correction of tetralogy of Fallot can be performed safely in adults with low mortality and good functional improvement. The incidence of residual cardiac defects is low. The long-term importance of impaired ventricular function and arrhythmias remains to be ascertained.     

Ruptured sinus of valsalva presenting late following repair of tetralogy of fallot

Rupture of sinus of valsalva following repair of tetralogy of fallot (TOF) is very rare. It should be suspected as a cause of recurrent or prolonged pleural effusion and congestive cardiac failure in patients who have undergone repair of TOF. We report one such patient.     

Reoperation for residual defects following intracardiac repair for tetralogy of Fallot

Reoperation was carried out in two patients who had previously undergone correction for tetralogy of Fallot and presented with severe pulmonary hypertension. One patient who had undergone intracardiac repair five years earlier had a residual ventricular septal defect (VSD), right ventricular outflow (RVOT) obstruction, patent ductus arteriosus and severe pulmonary hypertension. The second patient, who had undergone intracardiac repair four years earlier, also had a residual VSD and RVOT obstruction. Both underwent VSD closure, RVOT resection and pulmonary valve replacement and did well postoperatively. We recommend aggressive surgical treatment in such patients even in the presence of pulmonary hypertension.     

An alternative technique for intracardiac exposure during transatrial repair of tetralogy of fallot

The commonly used technique to facilitate intracardiac exposure during transatrial repair of tetralogy of fallot involves considerable retraction of the tricuspid valve using retractors. We describe an alternative surgical technique in which it is possible to dispense away with the retractors. The advantages of such a technique are discussed.     

A bicuspid pulmonary valve associated with tetralogy of fallot

Congenitally bicuspid pulmonary valves are uncommon. When they occur, it is usually in association with other congenital cardiac lesions, most often a tetralogy of Fallot. We present a rare case of a patient with a congenitally bicuspid pulmonary valve who had pulmonary valve and RVOT reconstruction. The patient did well for 17 years, but needed further reconstruction when the pulmonary valve started getting stenosed and RV pressures went up significantly. The pulmonary valve showed fibroses, thickening, and focal calcification.     

Total intracardiac repair for tetralogy of Fallot in adults

The anatomic and clinical features of 47 patients who were 18 years of age or older at the time of total intracardiac repair for tetralogy of Fallot are reviewed. Twenty (43%) patients had had previous palliative surgery. Of 14 pulmonary-systemic shunts, 9 (64%) remained patent. The location of the ventricular septal defect was infracristal in 90% of patients. The predominant right ventricular outflow tract obstruction was at the infundibulum in 30%; another 64% of patients had combined valvular and infundibular obstruction. Total intracardiac repair was achieved; hospital mortality was 8.5%. Morbidity was minor, and hemorrhage was a significant problem in only 2 patients. Thirty-five patients have been followed from 11 months to 15 years after surgery. There were 4 late deaths; the actuarial 10-year survival rate was 82%.     

Atrioventricular septal defect with tetralogy of fallot: results of surgical correction

Background. The outcome of surgical correction of complete atrioventricular septal defect with tetralogy of Fallot has improved in recent years. Controversy exists about the optimal approach to this complex lesion. Our experience over the past 8 years with a single technique is reviewed. The important anatomic features of this lesion are discussed in relation to our method of repair.

Methods. Between 1988 and 1996, 11 consecutive patients underwent correction of complete atrioventricular septal defect with tetralogy of Fallot. Nine patients had undergone prior palliative shunts. The two-patch technique for atrioventricular septal defect was used. The ventricular septal defect was closed through a right ventriculotomy in each case. The commissure between the superior and inferior bridging leaflets of the left portion of the common atrioventricular valve was closed in each patient. Management of the right ventricular outflow tract was individualized.

Results. There was one mortality in the early postoperative period. One patient required reoperation for closure of a dehiscent left atrioventricular valve cleft. All survivors are currently in New York Heart Association functional class I or II at follow-up ranging from 2 to 101 months.

Conclusions. Atrioventricular septal defect with tetralogy of Fallot can be corrected with low mortality using the two-patch technique and closure of the ventricular septal defect through a combined approach using a right ventriculotomy and right atriotomy. Routine closure of the commissure of the left portion of the atrioventricular valve results in a low incidence of regurgitation. A good functional result can be achieved in most patients at intermediate-term follow-up.tetralogy of Fallot, atrioventricular septal defect, repair     

Surgical management of tetralogy of fallot with idiopathic thrombocytopenic purpura

The association of cyanotic heart disease with Idiopathic Thrombocytopenic Purpura (ITP) may accentuate the hemorrhagic complications of cardiac surgery under cardiopulmonary bypass. A-5-year-old girl with Chronic Steroid Refractory Idiopathic Thrombocytopenic Purpura with Tetralogy of Fallot (TOF) underwent successful intracardiac repair under normothermic cardiopulmonary bypass and warm blood potassium cardioplegia. Presurgery she received immunoglobulins and perioperatively she was administered inj methylprednisolone, inj aprotinin, platelets and fresh frozen plasma.