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J Thorac Cardiovasc Surg 1994;107:1291-1300
© 1994 Mosby, Inc.
SURGERY FOR CONGENITAL HEART DISEASE |
Le Plessis Robinson, France
From Marie Lannelongue Hospital, Le Plessis Robinson, France.
Received for publication May 28, 1993. Accepted for publication Sept. 16, 1993. Address for reprints: Claude Planché, MD, Marie Lannelongue Hospital, 133, avenue de la Résistance, 92350 Le Plessis Robinson, France.
Abstract
Absence of consensus persists regarding the optimal procedure and timing for the surgical treatment of young infants with symptomatic tetralogy of Fallot. From 1987 through 1992, 56 patients with tetralogy of Fallot were operated on at less than 6 months of age. Forty-one patients (median age 2.9 months) underwent primary repair and 15 (median age 2.4 months) underwent initial palliation. Mean follow-up was 24.2 ± 16.4 months. No strict protocol was used but patients who received initial palliation were younger, had a smaller pulmonary arterial tree, or had anomalous coronary artery. Two patients died (overall mortality 3.6%; 95% confidence limits 0% to 11%), one after initial palliation (6.7%), and one after primary repair (2.4%) (P = 0.47). Eight of the 15 patients who received initial palliation underwent repair and had an increase in pulmonary anulus size at the time of definitive repair (mean difference Z-value = 2.2 ± 1.6 standard deviation; p = 0.006). Transannular patch was required in 50% of patients who underwent repair (56% among patients having primary repair versus 13% for patients having initial palliation; P = 0.03). Five patients underwent reoperation. Early primary repair of symptomatic tetralogy of Fallot was achieved with a low mortality rate and is the preferred protocol. Initial palliation remains indicated in case of associated cardiac anomaly, very low weight, or severely hypoplastic pulmonary artery tree. (J THORAC CARDIOVASC SURG 1994;107:1291-300)
Repair of tetralogy of Fallot (TOF) in the first 6 to 12 months of life is now generally recommended, and even routine repair in the first month of life has been advocated.
1-6 The results of early repair, in reported series and in that reported herein, have been good, but some uncertainty continues about optimal management protocols. Certain matters with regard to transannular patching, its indications, potential disadvantages, and relative prevalence after a preliminary systemic–pulmonary artery shunting operation remain uncertain.
Repair early in life has for some time been the policy in this institution, but the practice has also included an initial shunting operation for some patients. The mix of procedures, completeness of objective studies, and the goodness of immediate-term follow-up make this experience suitable for intense analysis directed at some of the uncertainties remaining as to optimal treatment of patients with TOF.
MATERIALS AND METHODS
Patients.
Between Jan. 1, 1987, and July 1, 1992, 56 patients with TOF were referred for surgical treatment when less than 6 months of age. The number of patients under 6 months of age in whom surgical treatment was deferred is not available. Patients with pulmonary atresia, absent pulmonary valve, and complete atrioventricular canal defect were not included in this review. Three patients had Down's syndrome. Forty-six of the 56 patients had been receiving ß-adrenergic receptor blocking agents before the initial operation, in which cases this therapy was continued until operation.
Forty-one patients underwent primary repair. Fourteen patients underwent an initial modified Blalock-Taussig type of systemic-pulmonary artery shunt procedure with an interposed expanded polytetrafluoroethylene tubular graft (4 mm in 5 patients, 5 mm in 9); 12 on the right and 2 on the left. Eight of these patients subsequently underwent repair, and repair is planned for the remainder. One other patient with diminutive pulmonary arteries underwent palliative transannular patching without closure of the ventricular septal defect (which is still unclosed).
Among the 49 patients who underwent repair, the median age at repair was 3.4 months and 10% were less than 2 months old at repair; the median body surface area at repair was 0.31 m2. Median age at primary repair was 2.9 months and median age at an initial palliative operation was 2.4 months (Appendix Fig. 1, a); median body surface area at primary repair was 0.27 m2 and was 0.24 m2 at initial palliative operation (Appendix Fig. 1, b).
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A short vertical infundibulotomy was made; the parietal band was transected but no muscle was resected. The ventricular septal defect was approached through the right ventriculotomy in all patients and was closed with a Dacron patch with a running suture. The decision to insert a transannular patch was made at the time of repair by measuring the internal diameter of the narrowest point in the pulmonary outflow tract with calibrated Hegar dilators. If the resulting figure was less than the minimum acceptable pulmonary valve ring diameter as proposed by Pacifico, Kirklin, and Blackstone, 7 the infundibulotomy incision was extended across the anulus into the main pulmonary artery and a transannular patch of autologous fresh pericardium was inserted. In one patient with a nonconfluent left pulmonary artery, an aortic allograft was used to reconstruct the right ventricular outflow tract and the divided left pulmonary artery was directly implanted on the allograft.
Any atrial septal defect present was closed. Before chest closure, right and left ventricular pressures were measured.
Selection of type of initial palliative operation.
No formal protocol was used for selecting patients for an initial palliative operation, and in six patients initial palliation rather than primary repair was done because of the preference of the referring cardiologist. However, all four patients with anomalous origin of the left anterior descending coronary artery from the right and one with discontinuity of the left pulmonary artery had an initial palliative operation. Those who had an initial palliative operation, compared with those who underwent primary repair, were younger (Appendix Fig. 1), had a smaller pulmonary valve anulus (Fig. 1), and smaller origins of the left and right pulmonary arteries (median Z-value –2.9 versus –1.5).
Follow-up.
Postoperative inquiry was done between January 1992 and February 1993. Four of the patients surviving at most recent follow-up could not be traced during the formal follow-up period. Two of these have not been available since they were discharged from the hospital after complete repair, one in 1987 and one in 1989 (29.8 months after repair).
Mean follow-up time for survivors was 24.2 ± 16.4 months. Fifty percent have been traced for at least 24.3 months: 25% for 34.0 months and 10% for 49.4 months.
Data analysis.
All dimensions were transformed to Z-values (standard deviation units). The equation is as follows:
where (1) is the observed dimension in the patient, who is of a known size (body surface area), and (2) and (3) refer to values in normal persons of the same body surface area as the patient, obtained from specific solutions of regression equations derived from the analyses of measurements made in individuals comprising a sample of normal persons. 7 The Z-values for 95% of the normal population, regardless of age or size, lie between –2 and +2.
The usual nonparametric analyses and estimations of P values were made. Multivariable logistic regression analysis was used to determine the risk factors (explanatory variables) for receiving a transannular patch. 8 Survival and freedom from other time-related events were computed with the Kaplan-Meier life-table method or the University of Alabama at Birmingham (UAB) time-related hazard function regression method, 9 or both methods. The infrequency of death disallowed multivariable analysis. Risk-adjusted comparisons were made between the number of several outcome events in this study and those from other studies with the use of formal statistical methods. 9
RESULTS
Survival.
Two of the 56 patients have died. One patient with multiple malformations (VACTERL syndrome), who was 3 months of age at the time of primary repair with a transannular patch, originally had a stenosis at the origin of the left pulmonary artery and in the operating room (OR) after repair had a postrepair right ventricular/left ventricular pressure ratio ([OR] PRV/LV) of 0.69. This patient had respiratory distress in relation to a tracheoesophageal fistula that necessitated an emergency tracheostomy and died 8 weeks after repair. The second patient, with a nonconfluent left pulmonary artery and severe hypoxic spells, underwent a right systemic–pulmonary artery shunt and died 2 weeks later without definitive repair. The small number of deaths precluded a multivariable analysis looking for risk factors for death.
Although the total mortality rate among the 15 patients with an initial palliative operation was 6.7% (70% CL* 0.9% to 21%; 95% CL 0.3% to 34%) and among the 41 patients who underwent primary repair was 2.4% (70% CL 0.3% to 8%; 95% CL 0.1% to 14%), the difference could well be due to chance alone (P = 0.47, Fisher's test). The two groups were sufficiently dissimilar that direct risk-adjusted comparisons of outcomes been them were not valid.
Indirect risk-adjusted comparisons were possible with the use of a two-institution study from the current era as an external standard (Boston Children's Hospital [BCH] and UAB). 5 The total mortality among the 56 patients was similar to that reported in that study. Also, with the use of a multivariable risk factor equation for death from that study (Appendix Table I),the small difference between the predicted (3.2) and actual (2) number of deaths among the 56 patients could have been due to chance alone (P value 0.5). The same is true for the 49 patients who underwent repair, either primarily or after an initial shunting operation (P value = 0.5). Also, the risk-adjusted predicted number of deaths among the 15 patients who underwent an initial palliative operation, had they instead undergone primary repair, was similar to the actual, with the difference being likely to be caused by chance alone (P value = 0.8); however, 7 of these 15 patients have not yet undergone repair.
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Appendix Table II), this one containing in addition the postrepair (OR) PRV/LV, the predicted and actual numbers of deaths among the 49 patients undergoing repair were again similar (P for difference = 0.4).
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Table I). Eight of the 15 patients undergoing an initial palliative operation have undergone complete repair, at varying times after the initial palliative operation (Fig. 2). The patients who had repair very early after the initial shunting operation had it because of shunt failure. The remaining seven patients are expected to undergo repair.
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Table II. A nomogram illustrating the strength of the variables is shown in Fig. 4.
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Reoperation.
Reoperation after repair was done in five patients (after one-stage repair in 4 and repair after initial shunt in 1) between 4 and 16 months after the repair. In two patients, retailoring of a transannular patch was done to correct a preoperatively present stenosis in the first portion of the left pulmonary artery. In two patients, an aneurysm of a right ventricular patch had developed and was corrected by retailoring the patch; in one of these there was an anomalous left anterior descending artery originating from the right coronary artery and residual infundibular stenosis necessitated additional myomectomy. In the fifth patient, a transannular patch was placed because of residual "annular" stenosis.
DISCUSSION
The most appropriate type and timing of operation for small children with symptomatic TOF has been controversial for a long time. Classically, if symptoms developed during infancy, initial shunting was done and correction was delayed until after 2 years of age. However, death or complications were not uncommon after aorta–to–pulmonary artery shunting, encouraging Barratt-Boyes and Neutze 10 and others 11,12 to introduce primary intracardiac repair of TOF in infancy, with good results. However, young age, as well as small size, were found to increase the risk of operative death. 13 Also, with the development of the modified Blalock-Taussig shunt morbidity and mortality of palliation decreased and the majority of surgeons continued to prefer a more conservative two-stage approach and obtained excellent results. 14-16 Although mortality of the modified Blalock-Taussig shunt is currently low, performance of a shunt in a very small patient is not always free of complications and pulmonary artery distortion remains a potential serious problem. 17,18 On the other hand, advances in the surgical management of congenital heart disease in young infants and neonates and awareness of the deleterious effects of chronic hypoxia and ventricular hypertrophy progressively led several groups toward earlier primary complete correction either selectively or routinely. 1-6
Mortality.
In this series, hospital and time-related survivals in patients who received one-stage repair were comparable to those recently reported by other groups including those who have adopted routine primary repair. 3-5 Hospital mortality after primary repair was 2.4% in this series and 6.7% after initial palliation. Although these rates are not significantly different, 7 of the 15 patients who received palliation have still not undergone definitive repair and the two groups are also sufficiently dissimilar to preclude comparisons.
Correlates for initial shunt.
The two-institution comparative study from BCH-UAB clearly demonstrated that there is no supremacy of a two-stage approach over a one-stage repair. 5 However, very young age (less than 3 months) was found to be a risk factor for death especially when associated with unfavorable patterns of the right ventricular outflow tract. 5,6 Usually in the series herein reported correction was postponed until after 1 month of age. The decision to repair or to do a modified Blalock-Taussig shunt procedure in the neonate with severe symptoms is taken on an individual case basis but smaller patients tended to receive initial palliation by shunting (Table I). Also, in this series, hypoplastic pulmonary arterial tree or anomalous coronary artery were correlates for initial palliation (Table I). Groh and colleagues 4 have recently shown that complete primary repair can be safely accomplished in infants with diminutive pulmonary arteries, because these arteries may dilate significantly after normal pulmonary flow is established. Probably more relevant than the size of the pulmonary arteries is the presence of severe hypoplasia of the distal main trunk and pulmonary anulus, rendering it technically difficult to place a geometrically perfect patch. Small size of these structures was associated with a lower survivorship in the BCH-UAB study. 5 Therefore we would advocate primary repair at the time of presentation regardless of age except in patients with very low weight (less than 3 kg), severely hypoplastic pulmonary arterial tree (Z-value less than –4), or an associated anomaly (multiple ventricular septal defects, anterior coronary artery descending from the right coronary artery).
Prevalence of transannular patching.
The reduction in the prevalence of transannular patching in the most recent part of the experience at Marie Lannelongue Hospital may be related to greater awareness of this issue and acceptance of a smaller pulmonary "anulus" (93% transannular patching for Z = –2 in 1987 versus 35% in 1992; Fig. 4). It has been voiced that the benefits of earlier repair are overweighed by a higher prevalence of transannular patching. On the other hand, repair at an older age is associated with increased infundibular hypertrophy that necessitates extensive muscular resection, which may form the substrate for late ventricular dysfunction and arrhythmias. 12,19 In infants, muscular bands have not yet undergone secondary hypertrophic changes and therefore simple division of the obstructing parietal band is done. Transatrial repair has been done at Marie Lannelongue Hospital but is used in older patients when muscular hypertrophy instead of infundibular hypoplasia is the cause of obstruction. 20,21 Although transannular patching in this series was less prevalent for patients who underwent initial palliation (13% versus 56%, P = 0.03), the overall prevalence of transannular patching in patients undergoing primary repair was similar to that reported by other authors who used a one- or two-stage approach. 7,14,22
Growth of the pulmonary anulus.
Controversy remains regarding the influence of systemic–to–pulmonary arterial shunts on the development of the hypoplastic right ventricular–pulmonary arterial junction. In patients who underwent initial palliation in this study, the pulmonary "anulus" grew significantly during the interval between shunt placement and repair; this was also observed for the dimension of the pulmonary artery branches (Fig. 3). Several authors have reported pulmonary anulus enlargement between the initial shunt and repair procedures. 15,23 Pacifico, Kirklin, and Blackstone 7 reported a 70% prevalence of transannular patching after the initial shunt procedure and others have even reported the occurrence of acquired pulmonary atresia after shunting. 24 However, some of these observations were made in older patients and it is possible that annular enlargement may be greater in very young infants with TOF. If reduction of transannular patching is a reasonable objective, and knowing that in this series transannular patching was always used when the Z-value of the pulmonary anulus was less than -3 (Fig. 6), initial shunt could represent an alternative approach in this subset of patients and warrants further evaluation.
Reconstruction of the right ventricular outflow tract.
All reoperations in this series were necessary because of reconstruction problems of the right ventricular outflow tract, whether because of recurrent (or residual) left pulmonary artery stenosis or related to the type of material used for patching the outflow tract. Reoperation rate in this series was the same after one- or two-stage repair, but was still too high. Autologous fresh pericardium was used in this series because of its pliability and growth potential, as well as its hemostatic and geometric characteristics. Possibly use of glutaraldehyde-treated pericardium or some synthetic material would have been more appropriate and may have reduced the incidence of reoperations.
Long-term results of primary repair of tetralogy of Fallot in infancy are scarce, but some reports have suggested that early primary repair may improve late results. 25,26 Although the number of patients is small and follow-up is still short, this study has shed some light on some of the uncertainties regarding the treatment of patients with TOF. Symptomatic TOF without associated major anomalies can be safely repaired in early infancy. Very low weight associated with unfavorable patterns of pulmonary arterial anatomy remain in our view an indication for an initial shunt procedure. This policy may diminish the need for transannular patching at the time of complete repair and warrants further confirmation.
ADDENDUM
Since submission of this manuscript three patients who had undergone initial palliation underwent complete repair and none required a transannular patch.
We express our appreciation to Drs. John W. Kirklin and Eugene H. Blackstone of UAB for their assistance in data analysis and in the preparation of this manuscript.
References
This article has been cited by other articles:
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  H. A. Vohra, L. Adamson, and M. P. Haw Is early primary repair for correction of tetralogy of Fallot comparable to surgery after 6 months of age? Interactive CardioVascular and Thoracic Surgery, August 1, 2008; 7(4): 698 - 701. [Abstract] [Full Text] [PDF]
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 A. Ooi, N. Moorjani, G. Baliulis, B. R. Keeton, A. P. Salmon, J. L. Monro, and M. P. Haw Medium term outcome for infant repair in tetralogy of Fallot: indicators for timing of surgery Eur. J. Cardiothorac. Surg., December 1, 2006; 30(6): 917 - 922. [Abstract] [Full Text] [PDF]
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 R. D. Stewart, C. L. Backer, L. Young, and C. Mavroudis Tetralogy of Fallot: Results of a Pulmonary Valve-Sparing Strategy Ann. Thorac. Surg., October 1, 2005; 80(4): 1431 - 1439. [Abstract] [Full Text] [PDF]
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J. R. Lee, J. S. Kim, H. G. Lim, H. Y. Hwang, Y. J. Kim, J. R. Rho, and C. Ahn Complete repair of tetralogy of Fallot in infancy Interactive CardioVascular and Thoracic Surgery, September 1, 2004; 3(3): 470 - 474. [Abstract] [Full Text] [PDF]
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 E. I. van Dongen, A. G. Glansdorp, R. J. Mildner, B. W. McCrindle, A. G. Sakopoulos, G. VanArsdell, W. G. Williams, and D. Bohn The influence of perioperative factors on outcomes in children aged less than 18 months after repair of tetralogy of Fallot J. Thorac. Cardiovasc. Surg., September 1, 2003; 126(3): 703 - 710. [Abstract] [Full Text] [PDF]
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C. Alexiou, Q. Chen, M. Galogavrou, J. Gnanapragasam, A. P. Salmon, B. R. Keeton, M. P. Haw, and J. L. Monro Repair of tetralogy of Fallot in infancy with a transventricular or a transatrial approach Eur. J. Cardiothorac. Surg., August 1, 2002; 22(2): 174 - 183. [Abstract] [Full Text] [PDF]
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R. G. Seipelt, J. F. Vazquez-Jimenez, J. S. Sachweh, M.-C. Seghaye, and B. J. Messmer Antegrade palliation for diminutive pulmonary arteries in Tetralogy of Fallot Eur. J. Cardiothorac. Surg., April 1, 2002; 21(4): 721 - 724. [Abstract] [Full Text] [PDF]
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E. A. Bacha, A. M. Scheule, D. Zurakowski, L. C. Erickson, J. Hung, P. Lang, J. E. Mayer Jr, P. J. del Nido, and R. A. Jonas Long-term results after early primary repair of tetralogy of Fallot J. Thorac. Cardiovasc. Surg., July 1, 2001; 122(1): 154 - 161. [Abstract] [Full Text] [PDF]
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R. Pretre, G. Gendron, D. Tamisier, F. Vernant, D. Sidi, and P. Vouhe Results of the Lecompte procedure in malposition of the great arteries and pulmonary obstruction Eur. J. Cardiothorac. Surg., March 1, 2001; 19(3): 283 - 289. [Abstract] [Full Text] [PDF]
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C. Alexiou, H. Mahmoud, A. Al-Khaddour, J. Gnanapragasam, A. P. Salmon, B. R. Keeton, and J. L. Monro Outcome after repair of tetralogy of Fallot in the first year of life Ann. Thorac. Surg., February 1, 2001; 71(2): 494 - 500. [Abstract] [Full Text] [PDF]
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M. Pozzi, D. B. Trivedi, D. Kitchiner, and R. A. Arnold Tetralogy of Fallot: what operation, at which age Eur. J. Cardiothorac. Surg., June 1, 2000; 17(6): 631 - 636. [Abstract] [Full Text] [PDF]
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J. Caspi, E. Zalstein, N. Zucker, A. Applebaum, L. H. Harrison Jr, N. A. Munfakh, H. A. Heck Jr, T. B. Ferguson Jr, A. Stopa, M. White, et al. Surgical management of tetralogy of fallot in the first year of life Ann. Thorac. Surg., October 1, 1999; 68(4): 1344 - 1348. [Abstract] [Full Text] [PDF]
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C. J. Knott-Craig, R. C. Elkins, M. M. Lane, J. Holz, C. McCue, and K. E. Ward A 26-year experience with surgical management of tetralogy of fallot: risk analysis for mortality or late reintervention Ann. Thorac. Surg., August 1, 1998; 66(2): 506 - 511. [Abstract] [Full Text] [PDF]
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F. Godart, S. A. Qureshi, A. Simha, P. B. Deverall, D. R. Anderson, E. J. Baker, and M. Tynan Effects of modified and classic Blalock-Taussig shunts on the pulmonary arterial tree Ann. Thorac. Surg., August 1, 1998; 66(2): 512 - 517. [Abstract] [Full Text] [PDF]
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 R. R. Chaturvedi, P. J. Kilner, P. A. White, A. Bishop, R. Szwarc, and A. N. Redington Increased Airway Pressure and Simulated Branch Pulmonary Artery Stenosis Increase Pulmonary Regurgitation After Repair of Tetralogy of Fallot: Real-Time Analysis With a Conductance Catheter Technique Circulation, February 4, 1997; 95(3): 643 - 649. [Abstract] [Full Text]
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