Risk factors for sudden death after repair of tetralogy of Fallot

BACKGROUND: Sudden cardiac death remains the most common cause of death after repair of tetralogy of Fallot. It has been suggested that sudden cardiac death is related to right ventricular hypertrophy or dilation. However, it is uncertain whether the preoperative patient status or operative techniques predispose for sudden cardiac death. METHODS: From 1958 to 1977, 658 patients underwent repair of tetralogy of Fallot at our institution at a median age of 12.2 +/- 8.6 years. One third had at least one previous palliative operation 4.6 +/- 2.5 years earlier. A total of 490 patients survived the first postoperative year and were analyzed for sudden cardiac death. During a follow-up period of 25.3 +/- 5.8 years (range, 1.0 to 35.5 years), 42 patients died, and 15 (36%) of those deaths were as a result of sudden cardiac death. RESULTS: Actuarial 10-year, 20-year, and 30-year survival rates were 97%, 94%, and 89%. Freedom from sudden cardiac death was 99%, 98%, and 95% after 10, 20, and 30 years. The risk of sudden cardiac death increased after 10 years from 0.06%/y to 0.20%/y. Univariate predictors (p < 0.1) of sudden cardiac death were use of an outflow tract patch (p = 0.068), male sex (p = 0.048), no previous palliation (p = 0.013), and higher preoperative New York Heart Association status (p = 0.014). Multivariate analysis confirmed these risk factors except use of an outflow tract patch. CONCLUSIONS: The most important risk factors for sudden cardiac death were higher preoperative New York Heart Association class and no previous palliation. Thus, early surgical intervention is recommended. The risk of sudden cardiac death increases with time, suggesting that long-term follow-up by specialized cardiologists or pediatricians should be intensified. However, all patients who died suddenly had at least two risk factors at the time of surgery.     

An unusual late complication following surgical repair of tetralogy of Fallot

A case is presented of extrusion of right ventricular outflow tract patch material through a mediastino-cutaneous fistula occurring five years after repair of tetralogy of Fallot. The focus of the mediastinal infection was an infected epicardial pacemaker electrode placed at the time of initial surgery. Subsequent echocardiographic studies showed minimal right ventricular outflow tract or pulmonary arterial dilatation, and, with no further surgery, the child is well after two and a half years follow-up.     

Transatrial-transpulmonary repair of tetralogy of Fallot

Classical tetralogy of Fallot was repaired by a transatrial-transpulmonary approach in 61 of 70 patients treated surgically on one surgical service between July 1981 and December 1985. Their ages ranged from 2.5 months to 57 years (mean 52.9 +/- 104.2 months). The ventricular septal defect was closed through the right atrium and tricuspid valve in 60 patients and via the pulmonary artery in one patient with a subpulmonary defect. A pulmonary arteriotomy was made in 38 patients to expose the stenotic pulmonary valve for valvotomy and to begin the infundibular mobilization and resection. It was extended through a small pulmonary anulus primarily in 15 patients and secondarily in one, a minimal distance upon the right ventricular infundibulum to enlarge the outflow tract to 2 standard deviations larger than the mean normal pulmonary valve anulus diameter. There were no hospital or late deaths or reoperations among the entire group. The mean postrepair peak right ventricular/left ventricular pressure measured in the operating room was 0.52 +/- 0.165 for the entire group of 61 patients which indicates similar relief of pulmonary stenosis to that obtained by the classical transventricular repair. At follow-up between 2.3 and 53.8 months (mean 22.6) each patient was in New York Heart Association Class I and without cardiac medication. Postoperative cardiac catheterization studies performed in nine patients between 4 and 28 months after operation showed no residual ventricular septal defects and a mean peak right/left ventricular pressure of 0.42 +/- 0.13. This series indicates that successful repair of tetralogy of Fallot can be accomplished in most patients by a transatrial-transpulmonary approach, which may provide improved right ventricular function over the long term.     

Conotruncal repair of tetralogy of Fallot

A repair within the conotruncal portion of the right ventricle is introduced. It was used for the intracardiac repair of 30 consecutive patients with tetralogy of Fallot. The infundibular septum was totally resected to reduce the muscular outflow stenosis. Short patch infundibuloplasty with a large monocusp was then used. Instead of the tricuspid septal leaflet, the membranous flap was employed as the suture line for patching the ventricular septal defect (VSD) to avoid a conduction disturbance, residual VSD, and fixing of the tricuspid septal leaflet. The right ventricular (RV) to systemic arterial pressure ratio was 50.0 +/- 14.6% (N = 26) and right atrial pressure was 9.0 +/- 2.5 mm Hg (N = 26) one month after operation. RV end-diastolic volume was 93.0 +/- 30.5% of normal (N = 15) before operation and 96.7 +/- 29.0% of normal one month after operation in the same patients. These data suggest that a conotruncal repair can maintain good RV function with low right atrial pressure and with no increase of RV volume.     

Primary repair of tetralogy of Fallot in infancy

From June 1983 to April 1988, 100 consecutive infants with symptomatic tetralogy of Fallot (without pulmonary atresia) were operated on. Ages ranged from 0.5 to 12 months (mean 7.3 +/- 3.7). Twenty patients were 0.5 to 3 months, 21 were 3 to 6 months, and 59 were 6 to 12 months of age. Mean weight was 6.5 kg +/- 1.7. Seventy patients received a transannular patch. The hospital mortality rate was 3% and there were no late deaths. Cumulative follow-up was 180 patient-years. Causes of death included hypoplastic pulmonary arteries (4 and 5 months old) and right ventricular failure (4 months old). The most important factors influencing right ventricular outflow tract reconstruction were neither weight (p = 0.90) nor age (p = 0.05) but rather were the ratio between weight and pulmonary arterial outflow tract diameter (p = 0.0005) and the ratio between body surface area and pulmonary arterial outflow tract diameter (p less than 0.0001). The last 48 patients were operated on with no deaths. During this period, operative management differed essentially in myocardial protection with blood cardioplegia. The predicted 30-day survivorship after repair was 90% to 99% (95% confidence limits). No ventricular arrhythmias have been detected after repair (mean follow-up 22.2 months). Mean right ventricular/left ventricular end-diastolic dimension ratio was (0.53 +/- 0.10 with M-mode echocardiography. These early results encourage us to proceed with primary repair of infants with symptomatic tetralogy of Fallot thanks to improved surgical management and enhanced myocardial protection.     

Complete repair of tetralogy of Fallot in infancy

We reviewed our long-term results of complete repair of tetralogy of Fallot (TOF) in infant. One hundred and sixty infants diagnosed as TOF underwent complete repair between January 1990 and April 2002. Mean age at the operation was 8.1+/-2.6 months. Correction was accomplished through a short right ventriculotomy of less than 30% of the ventricular height in all patients. A transannular patch was used in 78 patients (49%). There were four early deaths and no late death. Follow-up was complete in all survivors. All patients are currently in New York Heart Association functional class I or II. Actuarial freedom from reoperation at 1 and 10 years were 94.0 and 87.5%, respectively. Echocardiographic studies at follow-up showed excellent right ventricular function in most patients. Our results suggest that early complete repair of TOF yielded acceptable results with low mortality and morbidity. Transventricular repair of intracardiac pathology can be safely applied to yield good postoperative right ventricular function.     

Early primary repair of tetralogy of Fallot

Young age, low weight, and the requirement for transannular patch reconstruction of the right ventricular outflow tract (RVOT) are thought to adversely affect intracardiac repair of tetralogy of Fallot. Forty patients underwent complete repair between January, 1984, and January, 1987. Only infants with pulmonary atresia, complete atrioventricular canal, or coronary artery anomalies were shunted initially. The malalignment ventricular septal defect was closed with a Dacron patch. Infundibular resection was minimized. All atrial communications were left open. Thirty-four patients (85%) had a transannular RVOT patch, and 2 patients (5%) had a nontransannular patch. All 10 infants weighing 3.4 to 9.6 kg had a transannular RVOT patch at 7 weeks to 12 months of age. An RVOT patch was used in 26 of 30 children operated on between 1 and 6 years of age (median age, 24 months). No patient undergoing intracardiac repair died. Postoperative RVOT pullback gradients were between 0 and 35 mm Hg (mean, 18.5 mm Hg). Postoperative pulmonary artery saturation (mean, 73%) did not reveal any residual ventricular septal defect. The right ventricular/arterial pressure ratio was always less than 0.6 (mean, 0.4). All children are doing well at follow-up from two to 37 months. Serial echocardiograms reveal no residual ventricular septal defect and only 1 moderate RVOT gradient. Follow-up cardiac catheterization in 15 patients revealed no residual ventricular septal defect and RVOT gradients between 5 and 35 mm Hg. The right ventricular/left ventricular pressure ratio was always less than 0.6 (mean, 0.48). The early and late results justify continued primary repair of tetralogy of Fallot in symptomatic children, regardless of age or weight.(ABSTRACT TRUNCATED AT 250 WORDS)     

Follow-up of tetralogy of Fallot after repair

Repair of tetralogy of Fallot (TOF) exists for more than 40 years. This repair results in a pulmonary regurgitation, which is usually well tolerated for two decades or so, but eventually this is injurious for the right ventricle (RV). The RV enlargement and severe RV dysfunction increase risk for ventricular tachycardia (VT) and sudden death in the long-term. The pulmonary valve replacement (PVR) is shifting earlier to preserve RV function before patients develop symptoms. Several parameters have to be considered to facilate correct timing for PVR (surgically of by catheterization)?: echocardiography, magnetic resonance imaging, electrocardiogram and cardiopulmonary exercise. All patients should have regular follow-up in a specialized grown-up congenital heart disease (GUCH) center to detect as soon as possible pathological signs of RV enlargement. Implantable cardioverter-defibrillator (ICD) implantation for primary prevention and programmed ventricular stimulation in repaired TOF remain controversal.     

Postoperative functional assessment of a modified surgical approach to repair of tetralogy of Fallot

Since February 1982, we have used a modified technique to repair tetralogy of Fallot with transatrial closure of the ventricular septal defect, a short infundibular incision with avoidance of muscle resection, and anterior expansion of the right ventricular outflow tract using a patch in every case. The pulmonary anulus is approached in the usual manner. Twenty-six patients have had repair with this technique without mortality, and 20 patients (Group 1) have been evaluated 1 to 3 years postoperatively. Their results are compared with those of all patients followed up in our division who had had repair by the traditional technique in the 5 years before February 1982, with the same evaluation within 4 years of operation (Group 2, n = 22). Ten of 20 patients in Group 1 and 14 of 22 in Group 2 required a transannular patch. At cardiac catheterization, right ventricular outflow tract obstruction was effectively relieved in both groups (right ventricular systolic pressure [mean +/- standard error], Group 1 versus Group 2, 45 +/- 5 versus 49 +/- 4 mm Hg). No patient with the modified technique had a residual ventricular septal defect. By M-mode echocardiography, right ventricular-left ventricular end-diastolic dimension ratio was significantly lower for Group 1 patients (0.58 +/- 0.03 versus 0.78 +/- 0.04; normal less than 0.45; p less than 0.001). With radionuclide ventriculography, right ventricular ejection fractions were significantly higher for Group 1 patients with the modified repair (38% +/- 2.2% versus 33% +/- 1.8%; normal greater than or equal to 45%; p less than 0.05). Because the incidence of obligatory pulmonary insufficiency caused by transannular patch is similar in both groups, these results cannot be directly attributed to the presence of pulmonary insufficiency alone. With ambulatory electrocardiographic monitoring, no patient in Group 1 had any ventricular premature contractions whatsoever, whereas six of 22 patients in Group 2 had demonstrable ventricular premature contractions. These results suggest that in children with tetralogy of Fallot, right ventricular outflow tract obstruction can be effectively relieved with this modified approach with improved early preservation of right ventricular function.     

Successful surgical repair of an elderly patient with tetralogy of Fallot: a case report.

We report on a rare case of a 61-year-old female patient with tetralogy of Fallot who had a late radical repair. The patient had no previous surgical interventions, and her postoperative course was uneventful. One year after the repair, the patient is doing well and has no symptoms of cardiac failure. Surgical treatment greatly benefited our patient by improving her functional status and extending her life expectancy.     

Long-term follow-up after repair of tetralogy of Fallot

One hundred sixty-six patients with tetralogy of Fallot including 15 extreme type-patients repaired since 1971 were analyzed with respect to their physical activities, school or social life, hemodynamics, ECG, reoperation and late mortality. Follow-up period ranged from 1 to 19 years with an average of 9.1. There were 9 late deaths including 4 sudden deaths. Actuarial survival rates at 10 and 19 years were 95.2% and 90.5%, respectively. Eighty-five patients (72.0%) of 118 replied were in NYHA functional class I, 31 (26.3%) in class II and 2 (1.7%) in class III. Thirteen patients (7.8%) demonstrated right ventricular/left ventricular systolic pressure ratio over 0.80 because of inadequate relief of the pulmonary stenosis or obstruction, hypoplasia of the pulmonary artery or external conduit stenosis. Although the left ventricular ejection fraction was maintained at normal range (0.65 +/- 0.09), that of the right ventricule was below normal level (0.52 +/- 0.09). Two patients developed complete heart block postoperatively and underwent pacemaker implantations. Complete right bundle branch block was observed in 85 patients including 6 with left axis deviation. It is concluded that early correction even in the early infancy, adequate enlargement of the right ventricular outflow tract and minimal right ventriculotomy should be applied for correction of tetralogy of Fallot.     

Outcome after one-stage repair of tetralogy of Fallot

AIM: The purpose of this study was to evaluate the outcome after one-stage repair of tetralogy of Fallot (TOF). METHODS: Between May 1997 and December 2002, 240 patients with a median age of 9 months (1 month-48 years) underwent one-stage repair of TOF. Closure of ventricular septal defect (VSD) was accomplished through the right atrium in 171 (71.3%) patients and through the right ventricle in 69 (28.7%) patients. For the reconstruction of the right ventricular outflow tract (RVOT), transannular repair was performed in 151 (62.9%) patients, and non-transannular repair was performed in 89 (37.1%) patients. Follow-up was complete, averaging 40+/-17.6 months (3 months-5.8 years). RESULTS: There were 2 (0.8%) operative deaths. Between early repair group (age under 6 months) and late repair group (age above 6 months), there were no differences in the method of RVOT reconstruction (transannular vs non-transannular) and the need for branch pulmonary artery angioplasty. Early repair group had more transventricular VSD closure than late repair group (46% vs 22%, P < 0.05). Duration of inotropic support and intensive care unit (ICU) stay were longer in the early repair group (P < 0.05). Five patients required reoperations due to RVOT obstruction (n = 3), and residual VSD (n = 2). Kaplan-Meier freedom from reoperation at 5 years was 98.3+/-1%. Nine patients underwent catheter intervention for branch pulmonary artery stenosis. Freedom from reintervention at 5 years was 95.4+/-1.5%. All survivors are currently asymptomatic. CONCLUSIONS: One-stage repair of TOF could be performed with low mortality and morbidity. Especially, early one-stage repair in symptomatic infant could be performed with low risk, eliminating the need for palliative procedures.     

Experience in surgical treatment of tetralogy of Fallot]

Corrective Surgery was done in 122 patients with tetralogy of Fallot between May 1981 and Oct. 1989. The surgical mortality was 7.4% (9 patients) and no late death were noted. Multiple stenoses of the RV out-flow tract were observed in 84 patients (69%). The rate of patching of RV out-flow tract was 85.2%. The operation was performed without preoperative cardiac catheterization or angiocardiography in 24 patients (19.6%). Selection of cases without angiocardiography, operative techniques, and management of anomalous coronary vessels were discussed.