Tetralogy of Fallot: Favorable Outcome of Nonneonatal Transatrial, Transpulmonary Repair Tom R. Karl, MD, Shunji Sano, MD, PhD, Samphant Pornviliwan, MD, and Roger B. B. Mee, FRACS Royal Children’s Hospital, Melbourne, Australia
This report describes our experience with 366 patients who had a transatrial, transpulmonary repair of tetralogy of Fallot between December 1980 and December 1991. Included in this group are patients with tetralogy of Fallot plus atrioventricular septal defect as well as patients displaying all degrees of aortic override (in the presence of subaortic ventricular septal defect and right ventricular outflow tract obstruction). Median age was 15.3 months and median weight, 12.3 kg. Of the 366 patients, 72% required a pericardial patch to reconstruct the main pulmonary artery or right ventricular outflow tract. Serious coronary anomalies were seen in 11 patients, without influencing surgical approach. There were two hospital deaths (0.5%; 70% confidence limits, 0.2% to 1.2%).Actuarial survival was 97.5% at 42 months (95% confidence limits, 95% to 99%) reflecting four late
deaths over 1,129 patient-years of follow-up. Postoperative cardiac catheterization studies were performed in 61 patients at a mean follow-up interval of 23 months. Mean right ventriculadleft ventricular systolic pressure ratio after repair was 0.46 (standard deviation, 0.281, and mean gradient across the right ventricular outflow tract was 15 mm H g (standard deviation, 24 mm Hg). Actuarial freedom from reoperation for any reason has been 95% (95% confidence limits, 92% to 97%) at 5-year and 10-year follow-up. These early and medium-term results encourage us to continue with transatrial, transpulmonary repair of tetralogy of Fallot. We believe that this approach has an operative risk similar to or lower than transventricular repair, and that it will result in better preservation of right ventricular function in the long term. (Ann Thoruc Surg 1992;54:903-7)
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early 40 years have passed since Lillehei and associates’ [l] first successful repair of tetralogy of Fallot (TOF) using cross-circulation. Since then, thousands of patients have undergone correction, and currently many pediatric cardiac centers are reporting excellent early results for repair of most TOF variants [2-61. A major unresolved issue is the timing of operation relative to early and late survival, freedom from reoperation, and late functional status. Ideally, a repair should be designed and timed to offer low operative and late mortality, minimal attrition rate for children awaiting operation, preservation of anatomy and ventricular function, good early and late hemodynamic profile, and freedom from reoperation. Hudspeth and associates [7] first reported the transatrial repair of TOF in 1963. Subsequently, it has been shown that TOF can be reliably repaired through a transatrial, transpulmonary approach [2, 5, 6, 8, 91. Documentation of right ventricular (RV) dysfunction, pulmonary insufficiency, and ventricular arrhythmia after transventricular repair of TOF [2, 10-161 led us to adopt the transatrial, transpulmonary approach in 1980, and the results are reported herein. Accepted for publication March 2, 1992. Dr Sano’s current address is Okayama University Medical Center, Okayama, Japan. Address reprint requests to Dr Karl, Victorian Paediatric Cardiac Surgical Unit, Royal Children‘s Hospital, Flemington Rd, Parkville, Victoria, Australia 3052.
0 1992 by The Society of Thoracic Surgeons
In the interval spanning December 1980 to December 1991, 366 patients underwent transatrial, transpulmonary repair of TOF in our unit. Patients with pulmonary atresia and ventricular septal defect and patients with absent pulmonary valve syndrome were excluded from this series, as we consider them different from both an anatomic and physiologic point of view. Included were all TOF patients with pulmonary artery (PA) anomalies (hypoplasia, stenosis, discontinuity), atrioventricular septal defect, anomalous origin or course of a coronary artery, and all degrees of aortic override (double-outlet right ventricle with subaortic ventricular septal defect and right ventricular outflow tract obstruction [RVOTO]). Patients with double-outlet right ventricle and aortomitral discontinuity were excluded. Four patients operated on early in this period underwent a transventricular repair and are not included in this study. Since March 1981, all patients having operation for TOF in our unit were repaired using the transatrial, transpulmonary approach (n = 366). The median age at the time of operation was 15.3 months. The age distribution is displayed graphically in Figure 1.Mean weight was 12.3kg (standard deviation, 10 kg). Associated cardiac anomalies were present in 72 of 366 patients (20%): Left superior vena cava Persistent ductus Secundum atrial septal defect
26 12 10 0003-4975/92/$5.00
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Age (months)
Fig 1 . Age distribution of 366 patients undergoing transatrial, transpulmonay repair of tetralogy of Fallot. Transannular patch use is indicated for each age stratum.
Discontinuous left pulmonary artery Interrupted inferior vena cava Aortopulmonary window Common atrium Mitral stenosidleft ventricular hypoplasia Straddling mitral valve Partially anomalous pulmonary venous drainage Totally anomalous pulmonary venous drainage Complete atrioventricular septal defect Anomalous coronary artery
4 3 1 1 1 1 1 1 9 11
One hundred thirty-five of 366 patients (37%) had undergone some form of previous palliation. Most of the palliative procedures (other than modified right BlalockTaussig shunt) were performed in other units, the patients having been referred to us for intracardiac repair. The operative technique was uniform throughout this 10-year period. Repairs were performed using hypothermic cardiopulmonary bypass with bicaval cannulation and cardioplegic arrest. Early in the series, circulatory arrest was used for parts of the repair in some patients. Systemic to PA shunts, if present, were divided after commencement of cardiopulmonary bypass. Working through the right atrium and tricuspid valve, we excised the parietal and septal extensions of the infundibular septum and carried dissection upward to the level of the pulmonary annulus. Other obstructing fibrous tissue and muscle bands were excised as well. Hegar dilators were passed through the tricuspid valve into the RV outflow tract to estimate its size. If the annular diameter at this point was less than the mean normal predicted by Rowlatt and colleagues [17] (as applied by Kirklin and associates [MI), then the main PA was incised. Valvotomy was performed through existing commissures whenever possible, and if necessary the incision was extended via the anterior commissure for 5 to 10 mm onto the RV free wall. The PA sizes were measured, and autologous pericardial patch enlargement was performed if required. Main PA and RV incisions were likewise repaired with a pericardial patch calculated to establish a diameter 2 to 3 mm larger than a normal main PA. Transannular patch use as a
Ann Thorac Surg 1992;54:90%7
function of age at operation is displayed in Figure 1. Ventricular septal defects were closed transatrially, using interrupted pledgeted sutures and a Dacron patch. Other surgical procedures were performed as indicated for the coexisting cardiac defects. Significant coronary anomalies were encountered in 11 patients, including anomalous origin of the left anterior descending artery from the right coronary artery (n = 7), right coronary artery from left coronary artery (n = 3), and circumflex from right coronary artery (n = 1). The presence of the anomalous coronary artery did not influence the surgical approach. Six of these 11 patients required a transannular patch for relief of RVOTO. Follow-up data were collected by ourselves or our referring cardiologists. Analysis of data was performed using Egret software (Statistics and Epidemiology Research Corp, Seattle, WA). Survival analysis employed the Kaplan-Meier technique, using 95% confidence limits (CL). Any patients lost to follow-up were censored from the analysis at the time of last follow-up. Proportion means are expressed with 70% CL with continuity correction for upper and lower limit.
Results There were two hospital deaths, for an overall operative The first occurred mortality of 0.5% (70%CL, 0.2% to 1%). in a 15-month-old child with TOF plus anomalous origin of the left PA from the descending aorta, mitral stenosis, and small left ventricle. This child died of low cardiac output 24 hours after operation. The second occurred in a 4-month-old patient with TOF plus aortopulmonary window. He died suddenly of unknown causes the day after operation. The 364 survivors have been followed up for a total of 1,129 patient-years (mean follow-up time, 37 months). Follow-up is complete for 92% of the patients. During this time there have been four late deaths documented. A 4-year-old patient with Down's syndrome and doubleoutlet right ventricle anatomy died of progressive pulmonary hypertension and pneumonia, probably on the basis of chronic upper airway obstruction. Postmortem examination showed grade 4 pulmonary vascular obstructive disease. A 6-month-old patient with Down's syndrome and complete atrioventricular septal defect plus TOF died suddenly at home 2 months postoperatively, 3 hours after administration of diphtheria/pertussis/tetanus vaccine. The third patient was a 21-month-old child with TOF plus bronchomalacia and spastic quadriparesis. He died 2 weeks after a reoperation for relief of left PA stenosis and pulmonary arteriopexy. His death is presumed to have been due to arrhythmia, and this was the only death after reoperation. The fourth patient was a 6-month-old baby who had been born prematurely and in whom bronchopulmonary dysplasia developed during a long period of ventilator dependence after birth. She had been on chronic oxygen therapy before and after TOF repair, which was done for relief of severe cyanotic spells. She died at home of respiratory failure related to infection, 6 months after repair.
The actuarial survival for all patients, including operative mortality, was 97.5% (95% CL, 95% to 99%) at 42 months with no deaths documented after that follow-up time (Fig 2). The great majority of survivors were in New York Heart Association class I at the time of most recent follow-up. Postoperative cardiac catheterization studies have been performed in 61 patients at a mean interval of 23 months. This population is possibly skewed toward patients with suspected residual problems detected noninvasively, who were more likely to undergo catheter study earlier in the series. Systolic RV/left ventricular pressure ratio range was 0.17 to 1.2 (mean, 0.46; standard deviation, 0.28), with a systolic RV/PA pressure gradient of 0 to 90 mm Hg (mean, 15 mm Hg; standard deviation, 24 mm Hg). There was no difference in RV/left ventricular pressure ratio between patients with and without a transannular patch ( p > 0.05). Although pulmonary insufficiency was detectable in a majority of patients, this was only rarely of important severity. Doppler echocardiographic studies were generally satisfactory in prediction of catheter findings, and are currently our main follow-up evaluation modality. Reoperations have been required in 15 patients over this follow-up period. The indications for reoperation were as follows: ventricular septal defect, 3 patients; RVOTO, 6; PA branch stenosis, 2; ventricular septal defect and PA branch stenosis, 1; ventricular septal defect and RVOTO, 1; and RV outflow tract aneurysm, 2. Reoperations for residual RVOTO were performed through a ventriculotomy in 2 patients, but otherwise repeat transatrial approach was satisfactory. There was one reoperation-associated death, as mentioned in the discussion of late mortality. Actuarial freedom from reoperation for any reason was 95% (95% CL, 92% to 97%)at 5 years and 95% (95% CL, 87% to 98%)at 10 years (Fig 3).
Comment The surgical risk for TOF repair is currently less than 5% in many centers [2, 5, 61. Late results have also been good,
0.90
1 Transatrial-Transpulmonary Repair
0.80 0.75 -
0.70
II
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KARLETAL TRANSATRIAL, TRANSPULMONARY TETRALOGY REPAIR
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.--CL
KM Probability I
I
I
I I
I
I
I
I
Probabilitv I
----------I 0.85
Transatrial-Transpulmonary Repair
0.80
0.75 0.70
- KM I
--..CL
Probability I
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I
I
I
1
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with 20-year survivial often exceeding 90% [13, 19, 201. These results have been marred, however, by a poor late hemodynamic status in some long-term survivors [2, 10, 11, 21-24]. Furthermore, sudden death from ventricular arrhythmia has been noted in 0.5% to 5% of patients within 10 years of operation [12-141. Both late RV dilatation/dysfunction and an increased risk of ventricular ectopic activity are related, at least in part, to the presence of a large RV incision [2, 10, 11, 15, 19, 21-23]. In an attempt to minimize or avoid these problems, we adopted our current approach in 1980, encouraged by the results obtained in other units. Transatrial tetralogy repair differs from the classic approach in that the RV incision, if any, is just long enough to relieve the RVOTO. Ventriculotomy is therefore considerably shorter than would be required for ventricular septal defect closure, which can always be done transatrially in TOF. Kawashima and associates [2] have demonstrated that the transatrial, transpulmonary approach results in lower RV end-diastolic index and higher RV ejection fraction during isoproterenol infusion, as well as a lower ventricular arrhythmia incidence relative to the transventricular approach. Other studies have also suggested that arrhythmia problems could be decreased if the ventriculotomy were limited in size, although there are conflicting data on this point [15, 19, 24, 251. Theoretically, with adequate relief of RVOTO, a competent or nearly competent pulmonary valve, and an intact right ventricle, patients without transannular patch (41%in our series) should have the maximum potential for good RV function. Even patients with a limited transannular patch should not develop severe pulmonary insufficiency, and in most cases we would predict a good probability of preserved RV function in the long term. As the transatrial, transpulmonary repair is suitable for all TOF variants (including cases with anomalous coronary arteries), we have used it exclusively since 1981. A major question in the care of babies with TOF is when to operate. The detrimental effects of TOF (and cyanotic heart disease in general) on the myocardium, brain, lungs, and other organ systems are at least in part
906
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time-related [26-291. Consequently, neonatal repair (for symptomatic and nonsymptomatic patients) has been advocated [30-321. Success with neonatal repair of other lesions (for which delaying the operation is not an option) has provided some of the impetus [31]. In the long term, however, one must take into account attrition rate while awaiting operation, mortality after palliation, operative mortality, and late hemodynamic result. Although neonatal repair is attractive relative to the first two points, it remains to be seen whether the possibly higher operative mortality in this group will neutralize some of the (theoretical) benefits. Moreover, most neonatal repairs have been done using the transventricular approach, and therefore cannot solve the RV incision-related problems. The transatrial, transpulmonary repair can be performed in any size child, but is technically more satisfactory in those weighing more than 6 to 8 kg. We have attempted to strike a balance between time-related complications of the disease and the likelihood of achieving a good technical repair without ventriculotomy. The mean age at operation has been lower in recent years, and our current practice is repair at around 10 to 15 months of age. Anatomically good candidates for transatrial, transpulmonary repair might have correction earlier if symptomatic. We also prefer to delay complete repair for patients with hypoplastic branch pulmonary arteries. Infants in this latter group have usually come forward for palliation before our usual elective correction age. The right modified Blalock-Taussig shunt remains our palliative procedure of choice for smaller or younger patients who are symptomatic but are not ideal candidates for transatrial, transpulmonary repair. These palliated children will also have complete repair at about 12 to 18 months of age. Our nonneonatal one- or two-stage transatrial, transpulmonary repair has been satisfactory from the point of view of early and late mortality. Risk of palliation for TOF in our own unit (modified Blalock-Taussig shunt) currently cpproaches 0% (1 death in 10 years). The attrition rate of patients awaiting operation is difficult to know, as a substantial portion of our children are referred to us from other units (in and out of Australia) at the time of intracardiac repair. As the denominator in this group is unknown, the overall morbidity of waiting remains speculative. Certainly among patients followed up in our own cardiac unit from birth, the mortality is extremely low and mainly related to associated noncardiac problems. Although at this point it is impossible to prove statistically, our belief is that early and midterm resullts of our current program compare favorably with those of neonatal tetralogy repair in other units. As our follow-up is currently only approaching 10 years, we are unable to comment on the long-term outlook of transatrial repair relative to transventricular repair. We are aware that late hemodynamic deterioration (beyond 10 years' follow-up) that has been seen after transventricular repair is multifactorial and might occur with our approach as well. As a final point, the transatrial, transpulmonary approach allows TOF repair in the presence of anomalous coronary arteries, which may be present in 2% to 9% of patients [33, 341. Right ventricle to PA conduits were not
required in any of our 11 patients, and the coronary arteries were preserved in all cases. Among these 11 patients, there have been no operative deaths and thus far only one reoperation for residual RVOTO. In conclusion, we believe that this treatment program satisfies most of the criteria outlined in the introduction and will compare favorably with routine transventricular neonatal repair, in which a higher rate of RV dysfunction and late pulmonary insufficiency is projected, in addition to a (possibly) higher early mortality. We are aware that our follow-up time is limited and that the late hemodynamic deterioration based on other TOF-related factors may occur with our approach as well. Also, the overall mortality and morbidity for uncorrected patients in the first year of life remains somewhat speculative, although it has not been a major factor among patients followed up in our own unit. We, and many others, would therefore recommend nonneonatal transatrial, transpulmonary repair for children with TOF, as either a one-stage or, if necessary, a two-stage approach. Some of the TOF operations in the earlier part of this series were performed by Mr William Brawn, currently Consultant Cardiac Surgeon in Birmingham, UK. Also, we acknowledge the role of our cardiologists, anesthetists, intensivists, nurses, and perfusionists in the management and care of these children.
References 1. Lillehei CW, Cohen M, Warden HE, et al. Direct vision intracardiac surgical correction of tetralogy of Fallot and pulmonarv atresia defects. Report of first ten cases. Ann Surg i955;142:41845. 2. Kawashima Y. Kitamura S. Nakano S. Yaeihara T. Corrective surgery for tetralogy of Failot without or with minimal right ventriculotomy and with repair of the pulmonary valve. Circulation 1981;64(Suppl 2):147-53. 3. McGrath LB, Gonzalez-Lavin L. Tetralogy of Fallot repair with minimal or no ventriculotomy. J Cardiac Surg 1987;2: 27-47. 4. Pacific0 AD, Sand ME, Bargeron LM, Calvin EC. Transatrial transpulmonary repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 1987;93:919-24. 5. Dieth CA, Torres AR, Cazzaniga ME, Favaloro RG. Right atrial approach for surgical correction of tetralogy of Fallot. Ann Thorac Surg 1989;47546-52. 6. Touati GD, Vouhe PR, Amodeo A, et al. Primary repair of tetralogy of Fallot in infancy. J Thorac Cardiovasc Surg 1990;99:396-403. 7. Hudspeth AS, Cordell AR, Johnston FR. Transatrial approach to total correction of tetralogy of Fallot. Circulation 1963;27:79&800. 8. Edmunds LH Jr, Saxena NG, Friedman S, Rashkind WJ, Dodd PF. Transatrial repair of tetralorn of Fallot. Surgery 1976;80:681-8. 9. Falkovskii GE, Ilin VN, Churkin VM, et al. Initial experience in the correction of the tetralogy of Fallot in infants ;sing an approach through the right atrium and pulmonary artery. Grudn Serdechnososudistais Khir 1990;12:3541. 10. Horowitz LN, Vetter VL, Harken AH, Josephson ME. Electrophysiologic characteristics of sustained ventricular tachycardia occurring after repair of tetralogy of Fallot. Am J Cardiol 1980;46:446-52. 11. Harkin AH, Horowitz LN, Josephson ME. Surgical correction of recurrent sustained ventricular tachycardia following complete repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 1980;80:779-81.
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,
"
v_
-
I
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12. Foster V, McGoon DC, Kennedy MA, et al. Long term evaluation (12-22 years) of open heart surgery for tetralogy of Fallot. Am J Cardiol 1980;46:625-82. 13. Katz NM, Blackstone EH, Kirklin JW, et al. Late survival and symptoms after repair of tetralogy of Fallot. Circulation 1982;65:40>10. 14. Rosing DR, Borer JS, Kent KM, et al. Long term hemodynamic and electrocardiographic assessment following operative repair repair of tetralogy of Fallot. Circulation 1978;58: (Suppl 1):209-17. 15. Basagoitia AM, Iturralde P, Galvan 0, et al. Disorders of the rhythm and conduction in patients operated on for a total correction of tetralogy of Fallot. Arch Inst Cardiol Mex 1991;61:27-32. 16. Zimmermann M, Friedli B, Adamec R, Oberhansli I. Ventricular late potentials and induced ventricular tachycardia after surgical repair of tetralogy of Fallot. Am J Cardiol 1991;67: 873-8. 17. Rowlatt JF, Rimoldi HJA, Lev M. The quantitative anatomy of the normal child’s heart. Pediatr Clin North Am 1963;10:499. 18. Kirklin JW, Barratt-Boyes BG. Ventricular septa1 defect and pulmonary stenosis or atresia. In: Kirklin JW, Barratt-Boyes BG. Cardiac surgery. New York Wiley, 1986:701-99. 19. Kawashima Y, Kobayashi J, Matsuda A. Long term evaluation after correction of tetralogy of Fallot. Kyobu Geka 1990; 43:66&5. 20. Miyamura H, Eguchi S. Long term follow-up (20-25 years) of tetralogy of Fallot after correction. Kyobu Geka 1990;43: 6404. 21. Korns ME, Schwarz CJ, Lillehei CW, Edwards JE. Sequelae and complications of ventriculotomy. A pathologic study. Circulation 1969;39(Suppl 3):124-7. 22. March HW, Ross JK, Weirich WL, Gerbode F. The influence of the ventriculotomy site on the contraction and function of the right ventricle. Circulation 1961;24572-7. 23. Stirling GR, Stanley PH, Lillehei CW. The effects of cardiac bypass and ventriculotomy upon right ventricular function. Surg Forum 1957;8:43>8.
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24. Kato H, Nakano S, Matsuda H, et al. The influence of pulmonary regurgitation on left ventricular function after repair of tetralogy of Fallot. Nippon Kyobu Geka Gakkai Zasshi 1990;38:2257-63. 25. Abe T, Komatsu S. Long term followup studies in 211 patients with tetralogy of Fallot after corrective surgery over ten years. Kyobu Geka 1990;43:598-604. 26. Yamaki S. Pulmonary vascular disease in shunted and nonshunted patients with tetralogy of Fallot. Tohoku J Exp Med 1990;162:109-19. 27. Hausdorf G, Hinrichs C, Nienaber CA, Keck EW. Left ventricular contractile state after surgical correction of tetralogy of Fallot: risk factors for late left ventricular dysfunction. Pediatr Cardiol 1990;11:61-8. 28. Chandar JS, Wolff GS, Garson A Jr, et al. Ventricular arrhythmias in postoperative tetralogy of Fallot. Am J Cardiol 1990;65:55-61. 29. Li RK, Mickle DA, Weisel RD, et al. Effect of oxygen tension on the antioxidant enzyme activities of tetralogy of Fallot ventricular myocytes. J Mol Cell Cardiol 1989;21:567-75. 30. Guirgis NH, Losay J, Serraf A, et al. Complete repair of tetralogy of Fallot in infants under the age of six months. Arch Ma1 Coeur 1991;84:679-83. 31. Di Donato RM, Jonas RA, Lang P, et al. Neonatal repair of tetralogy of Fallot with and without pulmonary atresia. J Thorac Cardiovasc Surg 1991;101:12&37. 32. Gay F, Guarnera S, Tamisier D, et al. Results of the surgical treatment of tetralogy of Fallot before six months of age. A consecutive series of 62 cases with 49 complete repairs. Arch Ma1 Coeur 1990;83:511-6. 33. Fellows ICE, Freed MD, Keane JF, Van Praagh R, Bernhard WF, Castaneda AL. Results of routine preoperative coronary angiography in tetralogy of Fallot. Circulation 1975;51:561-6. 34. Hurvitz RA, Smith W, King H, Girod DA, Caldwell RL. Tetralogy of Fallot with abnormal coronary artery. J Thorac Cardiovasc Surg 1980;8:12%34.
This report describes our experience with 366 patients who had a transatrial, transpulmonary repair of tetralogy of Fallot between December 1980 and December 1991. Included in this group are patients with tetralogy of Fallot plus atrioventricular septal defect as well as patients displaying all degrees of aortic override (in the presence of subaortic ventricular septal defect and right ventricular outflow tract obstruction). Median age was 15.3 months and median weight, 12.3 kg. Of the 366 patients, 72% required a pericardial patch to reconstruct the main pulmonary artery or right ventricular outflow tract. Serious coronary anomalies were seen in 11 patients, without influencing surgical approach. There were two hospital deaths (0.5%; 70% confidence limits, 0.2% to 1.2%).Actuarial survival was 97.5% at 42 months (95% confidence limits, 95% to 99%) reflecting four late
deaths over 1,129 patient-years of follow-up. Postoperative cardiac catheterization studies were performed in 61 patients at a mean follow-up interval of 23 months. Mean right ventriculadleft ventricular systolic pressure ratio after repair was 0.46 (standard deviation, 0.281, and mean gradient across the right ventricular outflow tract was 15 mm H g (standard deviation, 24 mm Hg). Actuarial freedom from reoperation for any reason has been 95% (95% confidence limits, 92% to 97%) at 5-year and 10-year follow-up. These early and medium-term results encourage us to continue with transatrial, transpulmonary repair of tetralogy of Fallot. We believe that this approach has an operative risk similar to or lower than transventricular repair, and that it will result in better preservation of right ventricular function in the long term. (Ann Thoruc Surg 1992;54:903-7)
N
Patients a n d Methods
early 40 years have passed since Lillehei and associates’ [l] first successful repair of tetralogy of Fallot (TOF) using cross-circulation. Since then, thousands of patients have undergone correction, and currently many pediatric cardiac centers are reporting excellent early results for repair of most TOF variants [2-61. A major unresolved issue is the timing of operation relative to early and late survival, freedom from reoperation, and late functional status. Ideally, a repair should be designed and timed to offer low operative and late mortality, minimal attrition rate for children awaiting operation, preservation of anatomy and ventricular function, good early and late hemodynamic profile, and freedom from reoperation. Hudspeth and associates [7] first reported the transatrial repair of TOF in 1963. Subsequently, it has been shown that TOF can be reliably repaired through a transatrial, transpulmonary approach [2, 5, 6, 8, 91. Documentation of right ventricular (RV) dysfunction, pulmonary insufficiency, and ventricular arrhythmia after transventricular repair of TOF [2, 10-161 led us to adopt the transatrial, transpulmonary approach in 1980, and the results are reported herein. Accepted for publication March 2, 1992. Dr Sano’s current address is Okayama University Medical Center, Okayama, Japan. Address reprint requests to Dr Karl, Victorian Paediatric Cardiac Surgical Unit, Royal Children‘s Hospital, Flemington Rd, Parkville, Victoria, Australia 3052.
0 1992 by The Society of Thoracic Surgeons
In the interval spanning December 1980 to December 1991, 366 patients underwent transatrial, transpulmonary repair of TOF in our unit. Patients with pulmonary atresia and ventricular septal defect and patients with absent pulmonary valve syndrome were excluded from this series, as we consider them different from both an anatomic and physiologic point of view. Included were all TOF patients with pulmonary artery (PA) anomalies (hypoplasia, stenosis, discontinuity), atrioventricular septal defect, anomalous origin or course of a coronary artery, and all degrees of aortic override (double-outlet right ventricle with subaortic ventricular septal defect and right ventricular outflow tract obstruction [RVOTO]). Patients with double-outlet right ventricle and aortomitral discontinuity were excluded. Four patients operated on early in this period underwent a transventricular repair and are not included in this study. Since March 1981, all patients having operation for TOF in our unit were repaired using the transatrial, transpulmonary approach (n = 366). The median age at the time of operation was 15.3 months. The age distribution is displayed graphically in Figure 1.Mean weight was 12.3kg (standard deviation, 10 kg). Associated cardiac anomalies were present in 72 of 366 patients (20%): Left superior vena cava Persistent ductus Secundum atrial septal defect
26 12 10 0003-4975/92/$5.00
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Age (months)
Fig 1 . Age distribution of 366 patients undergoing transatrial, transpulmonay repair of tetralogy of Fallot. Transannular patch use is indicated for each age stratum.
Discontinuous left pulmonary artery Interrupted inferior vena cava Aortopulmonary window Common atrium Mitral stenosidleft ventricular hypoplasia Straddling mitral valve Partially anomalous pulmonary venous drainage Totally anomalous pulmonary venous drainage Complete atrioventricular septal defect Anomalous coronary artery
4 3 1 1 1 1 1 1 9 11
One hundred thirty-five of 366 patients (37%) had undergone some form of previous palliation. Most of the palliative procedures (other than modified right BlalockTaussig shunt) were performed in other units, the patients having been referred to us for intracardiac repair. The operative technique was uniform throughout this 10-year period. Repairs were performed using hypothermic cardiopulmonary bypass with bicaval cannulation and cardioplegic arrest. Early in the series, circulatory arrest was used for parts of the repair in some patients. Systemic to PA shunts, if present, were divided after commencement of cardiopulmonary bypass. Working through the right atrium and tricuspid valve, we excised the parietal and septal extensions of the infundibular septum and carried dissection upward to the level of the pulmonary annulus. Other obstructing fibrous tissue and muscle bands were excised as well. Hegar dilators were passed through the tricuspid valve into the RV outflow tract to estimate its size. If the annular diameter at this point was less than the mean normal predicted by Rowlatt and colleagues [17] (as applied by Kirklin and associates [MI), then the main PA was incised. Valvotomy was performed through existing commissures whenever possible, and if necessary the incision was extended via the anterior commissure for 5 to 10 mm onto the RV free wall. The PA sizes were measured, and autologous pericardial patch enlargement was performed if required. Main PA and RV incisions were likewise repaired with a pericardial patch calculated to establish a diameter 2 to 3 mm larger than a normal main PA. Transannular patch use as a
Ann Thorac Surg 1992;54:90%7
function of age at operation is displayed in Figure 1. Ventricular septal defects were closed transatrially, using interrupted pledgeted sutures and a Dacron patch. Other surgical procedures were performed as indicated for the coexisting cardiac defects. Significant coronary anomalies were encountered in 11 patients, including anomalous origin of the left anterior descending artery from the right coronary artery (n = 7), right coronary artery from left coronary artery (n = 3), and circumflex from right coronary artery (n = 1). The presence of the anomalous coronary artery did not influence the surgical approach. Six of these 11 patients required a transannular patch for relief of RVOTO. Follow-up data were collected by ourselves or our referring cardiologists. Analysis of data was performed using Egret software (Statistics and Epidemiology Research Corp, Seattle, WA). Survival analysis employed the Kaplan-Meier technique, using 95% confidence limits (CL). Any patients lost to follow-up were censored from the analysis at the time of last follow-up. Proportion means are expressed with 70% CL with continuity correction for upper and lower limit.
Results There were two hospital deaths, for an overall operative The first occurred mortality of 0.5% (70%CL, 0.2% to 1%). in a 15-month-old child with TOF plus anomalous origin of the left PA from the descending aorta, mitral stenosis, and small left ventricle. This child died of low cardiac output 24 hours after operation. The second occurred in a 4-month-old patient with TOF plus aortopulmonary window. He died suddenly of unknown causes the day after operation. The 364 survivors have been followed up for a total of 1,129 patient-years (mean follow-up time, 37 months). Follow-up is complete for 92% of the patients. During this time there have been four late deaths documented. A 4-year-old patient with Down's syndrome and doubleoutlet right ventricle anatomy died of progressive pulmonary hypertension and pneumonia, probably on the basis of chronic upper airway obstruction. Postmortem examination showed grade 4 pulmonary vascular obstructive disease. A 6-month-old patient with Down's syndrome and complete atrioventricular septal defect plus TOF died suddenly at home 2 months postoperatively, 3 hours after administration of diphtheria/pertussis/tetanus vaccine. The third patient was a 21-month-old child with TOF plus bronchomalacia and spastic quadriparesis. He died 2 weeks after a reoperation for relief of left PA stenosis and pulmonary arteriopexy. His death is presumed to have been due to arrhythmia, and this was the only death after reoperation. The fourth patient was a 6-month-old baby who had been born prematurely and in whom bronchopulmonary dysplasia developed during a long period of ventilator dependence after birth. She had been on chronic oxygen therapy before and after TOF repair, which was done for relief of severe cyanotic spells. She died at home of respiratory failure related to infection, 6 months after repair.
The actuarial survival for all patients, including operative mortality, was 97.5% (95% CL, 95% to 99%) at 42 months with no deaths documented after that follow-up time (Fig 2). The great majority of survivors were in New York Heart Association class I at the time of most recent follow-up. Postoperative cardiac catheterization studies have been performed in 61 patients at a mean interval of 23 months. This population is possibly skewed toward patients with suspected residual problems detected noninvasively, who were more likely to undergo catheter study earlier in the series. Systolic RV/left ventricular pressure ratio range was 0.17 to 1.2 (mean, 0.46; standard deviation, 0.28), with a systolic RV/PA pressure gradient of 0 to 90 mm Hg (mean, 15 mm Hg; standard deviation, 24 mm Hg). There was no difference in RV/left ventricular pressure ratio between patients with and without a transannular patch ( p > 0.05). Although pulmonary insufficiency was detectable in a majority of patients, this was only rarely of important severity. Doppler echocardiographic studies were generally satisfactory in prediction of catheter findings, and are currently our main follow-up evaluation modality. Reoperations have been required in 15 patients over this follow-up period. The indications for reoperation were as follows: ventricular septal defect, 3 patients; RVOTO, 6; PA branch stenosis, 2; ventricular septal defect and PA branch stenosis, 1; ventricular septal defect and RVOTO, 1; and RV outflow tract aneurysm, 2. Reoperations for residual RVOTO were performed through a ventriculotomy in 2 patients, but otherwise repeat transatrial approach was satisfactory. There was one reoperation-associated death, as mentioned in the discussion of late mortality. Actuarial freedom from reoperation for any reason was 95% (95% CL, 92% to 97%)at 5 years and 95% (95% CL, 87% to 98%)at 10 years (Fig 3).
Comment The surgical risk for TOF repair is currently less than 5% in many centers [2, 5, 61. Late results have also been good,
0.90
1 Transatrial-Transpulmonary Repair
0.80 0.75 -
0.70
II
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.--CL
KM Probability I
I
I
I I
I
I
I
I
Probabilitv I
----------I 0.85
Transatrial-Transpulmonary Repair
0.80
0.75 0.70
- KM I
--..CL
Probability I
I
I
I
I
1
I
I
with 20-year survivial often exceeding 90% [13, 19, 201. These results have been marred, however, by a poor late hemodynamic status in some long-term survivors [2, 10, 11, 21-24]. Furthermore, sudden death from ventricular arrhythmia has been noted in 0.5% to 5% of patients within 10 years of operation [12-141. Both late RV dilatation/dysfunction and an increased risk of ventricular ectopic activity are related, at least in part, to the presence of a large RV incision [2, 10, 11, 15, 19, 21-23]. In an attempt to minimize or avoid these problems, we adopted our current approach in 1980, encouraged by the results obtained in other units. Transatrial tetralogy repair differs from the classic approach in that the RV incision, if any, is just long enough to relieve the RVOTO. Ventriculotomy is therefore considerably shorter than would be required for ventricular septal defect closure, which can always be done transatrially in TOF. Kawashima and associates [2] have demonstrated that the transatrial, transpulmonary approach results in lower RV end-diastolic index and higher RV ejection fraction during isoproterenol infusion, as well as a lower ventricular arrhythmia incidence relative to the transventricular approach. Other studies have also suggested that arrhythmia problems could be decreased if the ventriculotomy were limited in size, although there are conflicting data on this point [15, 19, 24, 251. Theoretically, with adequate relief of RVOTO, a competent or nearly competent pulmonary valve, and an intact right ventricle, patients without transannular patch (41%in our series) should have the maximum potential for good RV function. Even patients with a limited transannular patch should not develop severe pulmonary insufficiency, and in most cases we would predict a good probability of preserved RV function in the long term. As the transatrial, transpulmonary repair is suitable for all TOF variants (including cases with anomalous coronary arteries), we have used it exclusively since 1981. A major question in the care of babies with TOF is when to operate. The detrimental effects of TOF (and cyanotic heart disease in general) on the myocardium, brain, lungs, and other organ systems are at least in part
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time-related [26-291. Consequently, neonatal repair (for symptomatic and nonsymptomatic patients) has been advocated [30-321. Success with neonatal repair of other lesions (for which delaying the operation is not an option) has provided some of the impetus [31]. In the long term, however, one must take into account attrition rate while awaiting operation, mortality after palliation, operative mortality, and late hemodynamic result. Although neonatal repair is attractive relative to the first two points, it remains to be seen whether the possibly higher operative mortality in this group will neutralize some of the (theoretical) benefits. Moreover, most neonatal repairs have been done using the transventricular approach, and therefore cannot solve the RV incision-related problems. The transatrial, transpulmonary repair can be performed in any size child, but is technically more satisfactory in those weighing more than 6 to 8 kg. We have attempted to strike a balance between time-related complications of the disease and the likelihood of achieving a good technical repair without ventriculotomy. The mean age at operation has been lower in recent years, and our current practice is repair at around 10 to 15 months of age. Anatomically good candidates for transatrial, transpulmonary repair might have correction earlier if symptomatic. We also prefer to delay complete repair for patients with hypoplastic branch pulmonary arteries. Infants in this latter group have usually come forward for palliation before our usual elective correction age. The right modified Blalock-Taussig shunt remains our palliative procedure of choice for smaller or younger patients who are symptomatic but are not ideal candidates for transatrial, transpulmonary repair. These palliated children will also have complete repair at about 12 to 18 months of age. Our nonneonatal one- or two-stage transatrial, transpulmonary repair has been satisfactory from the point of view of early and late mortality. Risk of palliation for TOF in our own unit (modified Blalock-Taussig shunt) currently cpproaches 0% (1 death in 10 years). The attrition rate of patients awaiting operation is difficult to know, as a substantial portion of our children are referred to us from other units (in and out of Australia) at the time of intracardiac repair. As the denominator in this group is unknown, the overall morbidity of waiting remains speculative. Certainly among patients followed up in our own cardiac unit from birth, the mortality is extremely low and mainly related to associated noncardiac problems. Although at this point it is impossible to prove statistically, our belief is that early and midterm resullts of our current program compare favorably with those of neonatal tetralogy repair in other units. As our follow-up is currently only approaching 10 years, we are unable to comment on the long-term outlook of transatrial repair relative to transventricular repair. We are aware that late hemodynamic deterioration (beyond 10 years' follow-up) that has been seen after transventricular repair is multifactorial and might occur with our approach as well. As a final point, the transatrial, transpulmonary approach allows TOF repair in the presence of anomalous coronary arteries, which may be present in 2% to 9% of patients [33, 341. Right ventricle to PA conduits were not
required in any of our 11 patients, and the coronary arteries were preserved in all cases. Among these 11 patients, there have been no operative deaths and thus far only one reoperation for residual RVOTO. In conclusion, we believe that this treatment program satisfies most of the criteria outlined in the introduction and will compare favorably with routine transventricular neonatal repair, in which a higher rate of RV dysfunction and late pulmonary insufficiency is projected, in addition to a (possibly) higher early mortality. We are aware that our follow-up time is limited and that the late hemodynamic deterioration based on other TOF-related factors may occur with our approach as well. Also, the overall mortality and morbidity for uncorrected patients in the first year of life remains somewhat speculative, although it has not been a major factor among patients followed up in our own unit. We, and many others, would therefore recommend nonneonatal transatrial, transpulmonary repair for children with TOF, as either a one-stage or, if necessary, a two-stage approach. Some of the TOF operations in the earlier part of this series were performed by Mr William Brawn, currently Consultant Cardiac Surgeon in Birmingham, UK. Also, we acknowledge the role of our cardiologists, anesthetists, intensivists, nurses, and perfusionists in the management and care of these children.
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12. Foster V, McGoon DC, Kennedy MA, et al. Long term evaluation (12-22 years) of open heart surgery for tetralogy of Fallot. Am J Cardiol 1980;46:625-82. 13. Katz NM, Blackstone EH, Kirklin JW, et al. Late survival and symptoms after repair of tetralogy of Fallot. Circulation 1982;65:40>10. 14. Rosing DR, Borer JS, Kent KM, et al. Long term hemodynamic and electrocardiographic assessment following operative repair repair of tetralogy of Fallot. Circulation 1978;58: (Suppl 1):209-17. 15. Basagoitia AM, Iturralde P, Galvan 0, et al. Disorders of the rhythm and conduction in patients operated on for a total correction of tetralogy of Fallot. Arch Inst Cardiol Mex 1991;61:27-32. 16. Zimmermann M, Friedli B, Adamec R, Oberhansli I. Ventricular late potentials and induced ventricular tachycardia after surgical repair of tetralogy of Fallot. Am J Cardiol 1991;67: 873-8. 17. Rowlatt JF, Rimoldi HJA, Lev M. The quantitative anatomy of the normal child’s heart. Pediatr Clin North Am 1963;10:499. 18. Kirklin JW, Barratt-Boyes BG. Ventricular septa1 defect and pulmonary stenosis or atresia. In: Kirklin JW, Barratt-Boyes BG. Cardiac surgery. New York Wiley, 1986:701-99. 19. Kawashima Y, Kobayashi J, Matsuda A. Long term evaluation after correction of tetralogy of Fallot. Kyobu Geka 1990; 43:66&5. 20. Miyamura H, Eguchi S. Long term follow-up (20-25 years) of tetralogy of Fallot after correction. Kyobu Geka 1990;43: 6404. 21. Korns ME, Schwarz CJ, Lillehei CW, Edwards JE. Sequelae and complications of ventriculotomy. A pathologic study. Circulation 1969;39(Suppl 3):124-7. 22. March HW, Ross JK, Weirich WL, Gerbode F. The influence of the ventriculotomy site on the contraction and function of the right ventricle. Circulation 1961;24572-7. 23. Stirling GR, Stanley PH, Lillehei CW. The effects of cardiac bypass and ventriculotomy upon right ventricular function. Surg Forum 1957;8:43>8.
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24. Kato H, Nakano S, Matsuda H, et al. The influence of pulmonary regurgitation on left ventricular function after repair of tetralogy of Fallot. Nippon Kyobu Geka Gakkai Zasshi 1990;38:2257-63. 25. Abe T, Komatsu S. Long term followup studies in 211 patients with tetralogy of Fallot after corrective surgery over ten years. Kyobu Geka 1990;43:598-604. 26. Yamaki S. Pulmonary vascular disease in shunted and nonshunted patients with tetralogy of Fallot. Tohoku J Exp Med 1990;162:109-19. 27. Hausdorf G, Hinrichs C, Nienaber CA, Keck EW. Left ventricular contractile state after surgical correction of tetralogy of Fallot: risk factors for late left ventricular dysfunction. Pediatr Cardiol 1990;11:61-8. 28. Chandar JS, Wolff GS, Garson A Jr, et al. Ventricular arrhythmias in postoperative tetralogy of Fallot. Am J Cardiol 1990;65:55-61. 29. Li RK, Mickle DA, Weisel RD, et al. Effect of oxygen tension on the antioxidant enzyme activities of tetralogy of Fallot ventricular myocytes. J Mol Cell Cardiol 1989;21:567-75. 30. Guirgis NH, Losay J, Serraf A, et al. Complete repair of tetralogy of Fallot in infants under the age of six months. Arch Ma1 Coeur 1991;84:679-83. 31. Di Donato RM, Jonas RA, Lang P, et al. Neonatal repair of tetralogy of Fallot with and without pulmonary atresia. J Thorac Cardiovasc Surg 1991;101:12&37. 32. Gay F, Guarnera S, Tamisier D, et al. Results of the surgical treatment of tetralogy of Fallot before six months of age. A consecutive series of 62 cases with 49 complete repairs. Arch Ma1 Coeur 1990;83:511-6. 33. Fellows ICE, Freed MD, Keane JF, Van Praagh R, Bernhard WF, Castaneda AL. Results of routine preoperative coronary angiography in tetralogy of Fallot. Circulation 1975;51:561-6. 34. Hurvitz RA, Smith W, King H, Girod DA, Caldwell RL. Tetralogy of Fallot with abnormal coronary artery. J Thorac Cardiovasc Surg 1980;8:12%34.
