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Ann Thorac Surg 2001;71:494-500
© 2001 The Society of Thoracic Surgeons
a Department of Cardiac Surgery, The General Hospital, Southampton, United Kingdom
b Department of Paediatric Cardiology, The General Hospital, Southampton, United Kingdom
Accepted for publication September 25, 2000.
Address reprint requests to Dr Monro, Department of Cardiac Surgery, The General Hospital, Tremona Rd, Southampton SO16 6YD, UK
e-mail: monro1711{at}aol.com
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Abstract |
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Methods. Between 1974 and 2000, 89 consecutive infants with a mean age of 6.3 ± 2.6 months (range, 15 days to 12 months) underwent repair of tetralogy of Fallot (ventricular septal defect and pulmonary stenosis) by one surgeon (J.L.M.). Three infants had previous palliative operations. Sixty-seven procedures were urgent or emergency. A transannular patch was inserted in 69 patients (77.5%). Follow-up was complete, averaging 13.4 ± 5.6 years (range, 0 to 25.4 years).
Results. There was one operative death (1.1%). Mean right ventricular to left ventricular pressure ratio postoperatively was 0.4 ± 1.1 (in 79 patients, < 0.5). Fourteen patients underwent reoperations or reinterventions. There were no reoperations for residual or recurrent ventricular septal defect. Kaplan-Meier freedom from reoperation or reintervention for any cause at 20 years was 85% ± 4.4%, for relief of right ventricular outflow tract obstruction it was 94% ± 3.1%, and for pulmonary valve replacement this was 95.4% ± 2.6%. Use of a transannular patch did not significantly affect the need for reoperation or reintervention. There was one late death (leukemia). Kaplan-Meier 20-year survival was 97.8% ± 1.9%. On latest echocardiography, 42 patients had moderate pulmonary regurgitation, 4 had a right ventricular outflow tract gradient more than 40 mm Hg, and 86 had good biventricular function. Twelve-lead electrocardiography was performed in all and 24-hour electrocardiography in 61 patients. One patient (1.1%) exhibited late recurrent ventricular tachycardia requiring implantation of a defibrillator. The remaining 86 patients are in New York Heart Association class I with none of them receiving antiarrhythmic medications.
Conclusions. These data strongly support the concept of early repair of tetralogy of Fallot. It is associated with an acceptable operative risk and a low incidence of significant arrhythmias, and provides long-term survival similar to that observed in the general population. Late complications may, however, develop, and long-term follow-up for their early recognition is essential.
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Introduction |
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The optimal age of repair of TOF has been controversial, but because of the reduced operative mortality in the current era, the realization of the benefits of early repair on the heart and other organ systems [7, 10, 16, 17], and the desire to avoid the risks and inconvenience of a palliative procedure, elective early repair of TOF is now increasingly being performed [7, 10, 15, 18, 19].
However, questions still remain regarding the long-term outcome after repair of TOF in infancy. The impact of the resultant pulmonary regurgitation, for instance, in those requiring reconstruction of the right ventricular (RV) outflow tract with a transannular patch (TAP), on their late hemodynamic condition is uncertain.
Since 1974, it has been our policy to favor primary repair, rather than palliation, of TOF in symptomatic infants requiring operation. Exceptions were infants having hypoplastic pulmonary arteries (PAs), small-sized left ventricle (LV), anomalous origin of the left coronary artery, and multiple ventricular septal defects (VSDs) in whom a two-stage repair was, mostly, preferred.
Encouraged by the results of this approach we have from 1988 also performed primary repair in asymptomatic infants. The evolution of our practice, regarding the age at repair, over the study period, resulted in a progressively higher proportion of patients undergoing definitive repair of TOF during the first year of life (Fig 1).
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Patients and methods |
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During the same period, 10 infants with TOF underwent palliative procedures. Nine of them survived and had definitive repair when they were more than 1 year of age.
Definitions
The terms emergency and urgent denote the performance of nonelective repair within 1 and 7 days, respectively, from the surgical referral by the pediatric cardiologists.
Operative mortality includes any death occurring within 30 days after the operation or during the same hospital admission.
Procedures performed by the cardiologists at recatheterization (balloon dilatation, stenting) are defined as reinterventions.
Clinical and pathologic features
Definitive diagnosis was, until around 1980, made by angiography. Thereafter a combination of echocardiography and angiography was used. Seventy-nine patients exhibited hypoxic spells at various intervals before their operation, 10 patients had no symptoms, and 66 were on ß-blockers preoperatively. Mean arterial oxygen saturation was 85.6% ± 10.6% (range, 31% to 100%).
The operative procedure was elective in 22 (24.7%) and urgent or emergency in 67 patients. The proportion of patients undergoing an elective repair before 1988 was 13%, and since then it rose to 43% (p = 0.002).
Three patients with hypoplastic PAs had a previous modified Blalock-Taussig shunt before surgical repair in the first year of life.
Surgical technique and operative data
Cardiopulmonary bypass with deep hypothermia and circulatory arrest, and surface or core cooling, were used until the late 1980s. Since then, repair of TOF under cardiopulmonary bypass alone became standard practice. Deep hypothermia and circulatory arrest and surface cooling are no longer in use.
Cardiopulmonary bypass with deep hypothermia and circulatory arrest was used in 70 patients, and hypothermic cardiopulmonary bypass alone in 19 patients. Surface cooling was used in 57 patients at a mean temperature of 18°C ± 2.7°C. Cold crystalloid cardioplegia (St. Thomas‘s solution) has been used since 1978, and recently, cold blood cardioplegia has been used. The mean duration of cardiopulmonary bypass was 47.1 ± 16.2 minutes, and the mean duration of circulatory arrest was 49.2 ± 9.1 minutes.
Before 1988 a transventricular approach was invariably used. A TAP was inserted if the diameter of the narrowest point of the RV outflow tract, measured with Hegar dilators, was less than the minimum acceptable pulmonary valve ring diameter for the patient’s age and weight, as suggested by Pacifico and coworkers [20]. In the event of uncertainty, use of a TAP was more likely if a bicuspid pulmonary valve was present. If a patch was necessary, a longitudinal incision was made through the pulmonary valve ring and continued to the bifurcation of the PA. If an outflow patch was required, a monocusp from an antibiotic-sterilized aortic homograft was used for preference before 1983. Since then, homografts have been more difficult to obtain, and autologous pericardium has mostly been used.
Since 1988, when from the preoperative investigations a TAP was thought not to be necessary using criteria as previously defined (PA to ascending aorta [AA] diameter ratio > 0.5 on preoperative angiogram) [21], a transatrial approach was used. Furthermore, when the insertion of a TAP was inevitable, the incision in the RV was shorter and the TAP smaller.
Branch PAs were assessed preoperatively with angiography supplemented more recently by echocardiography. If they were thought to be of adequate size, a complete repair was planned, otherwise a Blalock-Taussig shunt followed by a later repair was preferred. Hypoplastic PAs remain a contraindication for an early repair in our unit. At operation, the assessment of the adequacy of the size of branch PAs was initially based on the judgment of the operating surgeon. Subsequently, the McGoon ratio and the Nakata index were used [22, 23].
Ventricular septal defects were closed with a continuous polypropylene suture, using a polyethylene terephthalate or Gore-Tex (W.L. Gore & Associates, Flagstaff, AZ) patch, through a transventricular approach in 77 and a transatrial approach in 12 patients. Resection of infundibular muscle was carried out as required. Interatrial communications were routinely closed. The RV and LV pressures were routinely measured before chest closure.
Follow-up
After their discharge from the hospital the patients were followed up at regular intervals by the pediatric cardiologists, and echocardiography and 12-lead ECG were routinely performed.
Data were obtained through a detailed review of the hospital medical records. Additional information was sought from the referring physicians, family doctors, and the patients‘ families as appropriate.
Mean follow-up was 13.4 ± 5.6 years (range, 0 to 25.4 years). Twenty-four patients were followed for up to 10 years and 63 patients for 10.1 to 25.4 years. Follow-up information was complete within 12 months of the closing date of this study (March 31, 2000).
Statistics
Continuous data were expressed as mean (± standard deviation). Proportions were compared with 
2 or Fisher‘s exact test, and means with Student’s t test. Freedom from time-related events (± standard error from the mean) was calculated with the Kaplan-Meier method, and the resulting curves were compared with log rank test. A p value of less than 0.05 was considered significant. Analyses were performed with the SPSS PC version 8 (SPSS Inc, Chicago, IL).
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Results |
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A TAP was inserted in 69 patients (77.5%), a monocusp homograft in 33, pericardium in 31, and dura mater in 5. Until 1988 the incidence of the use of a TAP was 83.3% (45 of 54 patients), and since then it was 68.6% (24 of 35 patients; p = 0.1).
A PA/AA ratio of less than or equal to 0.5 (p < 0.00001) and presence of a bicuspid pulmonary valve (p = 0.0002) were significantly associated with the need for a TAP. A slightly higher proportion of infants up to 6 months of age required insertion of a TAP, but this may have been because of chance alone (p = 0.6; Fig 2).
The mean RV pressure at the end of the operation was 35 ± 7.7 mm Hg (range, 20 to 60 mm Hg), the mean LV pressure was 86 ± 8.2 mm Hg (range, 66 to 116 mm Hg), and the mean RV/LV pressure ratio was 0.40 ± 0.08 (range, 0.2 to 0.8). This was less than 0.5 in 79 patients, 0.5 to 0.6 in 8 patients, and 0.7 to 0.8 in 2 patients. The mean RV/LV pressure ratio among the patients who had a TAP was 0.39, and it was 0.41 in the 20 patients having a simple repair (p = 0.5).
Operative mortality and morbidity
One 8-month-old male infant with Down syndrome died 4 days after an uneventful procedure (1.1%). While in the intensive care unit, he became hypoxic and required high positive-pressure ventilation. He subsequently had bilateral pneumothoraces and respiratory distress syndrome. At postmortem examination, there was an unexplained generalized sloughing off of the tracheobronchial mucosa.
Thirteen patients (14.6%) had early postoperative complications as shown in Table 1.
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At latest 12-lead ECG, 81 patients were in sinus rhythm with right bundle-branch block pattern, 2 patients had intermittent first-degree and second-degree heart block not requiring treatment, 1 patient had occasional ventricular ectopic beats, and 2 patients were in normal sinus rhythm. No patient had a QRS complex equal to or longer than 180 ms.
A 24-hour Holter ECG was performed in 61 patients. Fifty-seven of these patients had sinus rhythm with right bundle-branch block pattern, 2 patients had intermittent first-degree and second-degree heart block already diagnosed by 12-lead ECG, and 1 patient had occasional ventricular ectopic beats, also diagnosed with 12-lead ECG. One patient had persistent recurrent tachycardia requiring antiarrhythmic medications and the implantation of an automatic defibrillator. This was the only patient to exhibit significant arrhythmia in this series (1.1%) and did so 16 years after his original operation.
There has been no case of a patient experiencing permanent neurologic deficit.
At the latest clinical evaluation all but 1 survivor, the patient with the defibrillator, were in New York Heart Association functional class I, leading a normal or nearly normal lifestyle with none of them receiving antiarrhythmic medications.
Outcome in neonates
There were 8 neonates (up to 30 days of age) in this series with a mean age of 24 ± 4.7 days (range, 15 to 30 days) and a mean body weight of 3.4 ± 0.9 kg (range, 2.1 to 4.2 kg). All had severe cyanosis preoperatively, and 5 had ductus arteriosus–dependent circulation and were receiving prostaglandins. None had undergone a previous operation. At a mean follow-up of 12.9 ± 6.1 years (range, 2 to 22.4 years), all 8 patients are well, with a sinus rhythm and right bundle-branch block pattern on their ECG.
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Comment |
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TopAbstractIntroductionPatients and methodsResultsCommentReferences |
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Early outcome
The immediate goal of repair of TOF is the restoration of a normal circulation by closing the VSD and relieving the RVOTO with the least possible operative mortality. Our experience in this respect, as evidenced by the low postoperative RV/LV pressure gradient and the lack of any reoperation for residual VSD, was rewarding. The operative mortality of 1.1% in this series, which extends over the last two and a half decades, was low and compares favorably with early mortality rates of 0% to 14.3% quoted in other series for one-stage or two-stage repair in infants, older children, and adults [1–15]. In their reports on children having repair during an era similar to ours, Kirklin and colleagues [9, 11] and Hammon and associates [4] found younger age (<3 months and <1 year) and use of a TAP to significantly increase the likelihood of an early death. None of the patients less than 3 months of age in our series died early or late, and the only operative fatality occurred, unexpectedly of pulmonary complications, in an 8-month-old infant with Down syndrome who underwent a simple repair.
Transannular patch and pulmonary regurgitation
The reported incidence of insertion of TAP varies widely. The relatively high overall incidence in this series (77.5%) reflects the efforts made to adequately relieve the often severe RVOTO according to the recommendations made by Pacifico and associates [20]. This has resulted in obtaining a mean postoperative RV/LV pressure ratio of 0.4, with the highest being 0.8. If we had accepted a higher postoperative RV/LV pressure ratio, the frequency of transannular patching would have been lower, and this may have reduced the incidence of regurgitation and the need for PVR. However, this would have most likely been achieved at the expense of an increase in the requirements for reoperations for residual or recurrent RVOTO. The decline in the use of TAP over time (83.3% until 1988 and 68.6% thereafter) was because of the change in our policy since 1988, favoring elective repair of TOF in asymptomatic infants with less severe RVOTO.
Whether repair at a younger age increases the need for use of a TAP remains uncertain. In a report from Toronto [12], it has been suggested that younger age at repair is associated with an increased need for transannular patching. However, in a two-institutional study comparing the results of a protocol of early repair on the establishment of diagnosis (Boston Children‘s Hospital) versus palliation in infancy and repair during the second year of life (University of Alabama), age at repair did not influence the incidence of use of TAP [11]. Although this question would be best answered with a prospective randomized study, our experience would suggest that the severity of the RVOTO, rather than age at repair, is the most important determinant of the frequency of use of TAP (Fig 2).
Transannular patching is often necessary to relieve the RVOTO, but it induces pulmonary regurgitation, which may be poorly tolerated, in the all-important immediate postoperative period. To reduce its early occurrence we have by preference used monocuspid homografts, accepting that they will become regurgitant later.
Long-term pulmonary regurgitation, on the other hand, may lead to RV dilatation and dysfunction, increase the need for reoperations, and provoke malignant ventricular arrhythmias [24]. Late pulmonary regurgitation invariably developed in the patients who required a TAP in this series, being severe in 6 patients who required replacement of a pulmonary valve and moderate in 42 patients. Its hemodynamic effect, however, was mostly benign, with 86 of the 87 late survivors having a good RV and LV function and being in New York Heart Association functional class I. Similar hemodynamic outcomes have been observed after repair of TOF during infancy elsewhere [7, 10].
Reoperations and reinterventions
The 20-year freedom from reoperation or reintervention for any cause of 85% (94% for RVOTO and 95.4% for PVR) would appear to be encouraging.
Use of a TAP was not a significant factor for any further operations or interventions for PVR in this series. Its clinical relevance, nevertheless, is apparent: 6 patients requiring PVR were all among those who received a TAP, and it is probable that more of these patients will need a PVR in time.
In a large series from the University of Alabama [9] comparing the outcome after repair of TOF with or without use of TAP with an up to 20-year follow-up, TAP was not a risk factor for reoperation in general, but it significantly increased the need for a PVR because of pulmonary regurgitation. The 20-year freedom from PVR in that series was 88% in the group receiving a TAP and 100% in the group not receiving a TAP [9].
The 91.8% freedom for PVR in the group receiving a TAP in our series is good, but it should be noted that 3 patients needed PVR more than 20 years after their operations (Fig 5).
There was no serious morbidity or mortality at reoperation, and the implantation of a homograft in the RV outflow tract restored good hemodynamics and reversed the process of the ongoing RV dilatation in all 6 patients, although 1 patient went on to exhibit recurrent ventricular tachycardia. This stresses the need for a close, long-term follow-up, so that a PVR can be performed before irreversible RV damage is established.
Late arrhythmias and survival
Previous studies have reported good early and late hemodynamic outcomes after repair of TOF in older children and adults but described a disappointing incidence of late arrhythmias and a late sudden death rate of up to 6% [7, 25]. The identification of the patients likely to experience catastrophic events late after repair of TOF remains a difficult problem, but Gatzoulis and coworkers [26] found that a QRS complex equal to or more than 180 ms, on the 12-lead ECG, is a good predictor of the development of ventricular arrhythmias and sudden death. The 1.1% incidence of significant arrhythmias and the lack of sudden death in our patients, none of whom had a QRS complex duration equal to or more than 180 ms on the 12-lead ECG, with a mean follow-up of 14.3 years, are most gratifying features of this study.
Low incidence or no arrhythmias and no sudden deaths have also been quoted from elsewhere after repair of TOF during infancy, although with somewhat shorter follow-up. Walsh and colleagues [7] described an incidence of ventricular arrhythmia of 1% after repair of TOF in 220 infants (mean follow-up of 60 months), whereas Touati and associates [10] and Caspi and coworkers [19] did not encounter ventricular arrhythmias in their series of 100 and 82 infants, respectively (mean follow-up, 22 to 24 months). Combined, these results appear to support the view that ventricular arrhythmias may be a consequence of endomyocardial fibrosis related to long-term hypoxemia, and that repair at an early age may reduce its prevalence and minimize the risk of sudden death [17, 27].
The long-term survival after repair of TOF with a variety of protocols at different ages is known to be very good, although not identical with that of the general population [7, 9, 11, 14, 28]. The actuarial 20-year survival of 97.8% (Fig 6) in this series, with the only late death occurring because of leukemia, is most rewarding and demonstrates that a normal survival expectancy after repair of TOF in infancy can be reasonably anticipated.
In conclusion, the results of this study strongly support the concept of early repair of TOF. In addition to low operative risk, repair of TOF in infancy is associated with a low incidence of significant arrhythmias and provides long-term survival similar to that observed in the general population. Late complications may, however, develop, and consistent long-term follow-up for their early recognition and management is essential.
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