Right Ventricular Outflow Tract After Non-Conduit Repair of Tetralogy of Fallot With Coronary Anomaly Sanjeev Kalra, MCh, Rajesh Sharma, MCh, Shiv Kumar Choudhary, MCh, Balram Airan, MCh, Anil Bhan, MCh, Anita Saxena, DM, Shyam Sunder Kothari, DM, and Panangipalli Venugopal, MCh Department of Cardiothoracic and Vascular Surgery, and Department of Cardiology, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India
Background. A total of 25 patients with tetralogy of Fallot and an important coronary artery crossing the right ventricular outflow tract underwent complete repair without use of an extracardiac conduit between January 1990 and December 1994. Repair was exclusively done by the transatrial or transatrial-transpulmonary approach. Age of these patients ranged from 1 to 12 years (mean 3.6 years). Three of the patients had already received a systemic to pulmonary artery shunt. Methods. All patients reporting for follow-up (n ⴝ 18) were subjected to transthoracic echocardiography and, if required, cardiac catheterization and angiography. Right ventricle to pulmonary artery gradients were noted preoperatively, at discharge following repair and at follow-up study. Results. Mean follow-up was 40.6 months (24 to 62
months). Mean early postoperative gradient was 23.5 ⴞ 13.4 mm Hg and 4 patients had significant (> 30 mm Hg) gradients. Mean late postoperative gradient was 20.6 ⴞ 12.4 mmHg and 2 patients had gradients greater than 30 mmHg. All the patients were in New York Heart Association functional class I at the time of last follow-up. Conclusions. Acceptable gradients across the right ventricular outflow tract are achievable following repair of tetralogy of Fallot in the presence of anomalous coronary artery across the right ventricular outflow tract using the transatrial or transatrial-transpulmonary approach. Most gradients were found not to vary significantly on subsequent follow-up. (Ann Thorac Surg 2000;70:723– 6) © 2000 by The Society of Thoracic Surgeons
A
normal coronaries is known to achieve durable results utilizing both transatrial and transventricular approaches [5–15], late results of a nonconduit approach to tetralogy of Fallot with anomalous coronary artery are still not widely known [2]. We decided to study the long-term fate of the right ventricular outflow tract in the patients of tetralogy of Fallot with an abnormal coronary artery who had undergone repair without use of an extracardiac conduit.
bout 2% to 9% of patients [1–3] with tetralogy of Fallot have an anomalous coronary artery crossing the right ventricular outflow tract that influences the surgical approach to the lesion. Repair may be accomplished with or without use of an extracardiac conduit. In either method, immediate and long-term outlook depends upon adequate and sustained relief of right ventricular outflow obstruction without injury to a coronary artery supplying a significant portion of myocardium. Conduit repairs performed in childhood are doomed to certain reoperation for conduit replacement [3] when the child outgrows the same or when conduit degeneration causes appearance of fresh gradients. Conduit compressing the coronary artery may lead to fatal myocardial ischemia [4]. The alternative methods for repair of tetralogy of Fallot, ie, repair utilizing a transatrial-transpulmonary artery approach or repair utilizing a ventriculotomy parallel to the anomalous coronary artery [3], are well known to provide adequate early relief from right ventricular obstruction. Whereas tetralogy of Fallot with
Accepted for publication Mar 31, 2000. Address reprint requests to Dr Sharma, Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029, India; e-mail:
[email protected].
© 2000 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Patients and Methods Thirty two, out of a total of 776, patients with tetralogy of Fallot who underwent repair at the All India Institute of Medical Sciences, New Delhi from January 1990 to December 1994 had an important coronary artery crossing the right ventricular outflow tract. Seven of these patients received extracardiac conduits and were excluded from the study. The case records of the 25 patients who had been repaired without resorting to an extracardiac conduit were retrieved. All 25 patients were advised to report for evaluation. Age of these patients ranged from 1 year to 12 years (mean 3.6 years). Sixteen of them were boys. Three of the patients had already received a systemic to pulmonary 0003-4975/00/$20.00 PII S0003-4975(00)01512-5
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Table 1. Classification of the Coronary Artery Crossing the Right Ventricular Outflow Tract 1. Single left anterior descending artery from right coronary artery 2. Accessory left anterior descending artery from right coronary artery 3. Right coronary artery from left anterior descending artery 4. Large conal artery from right coronary artery
13 9 2 1
artery shunt. In 2 patients, right modified BlalockTaussig shunt was performed at the age of 2 and 4 months for severe hypercyanotic spells. Another patient who presented at the age of 5 months with diagnosed coronary anomaly, also received a shunt and underwent complete repair at the age of 3 years. All patients underwent preoperative cardiac catheterization and angiography, as it is routinely done for all patients of tetralogy of Fallot at our center. Only 21 patients had angiographic localization of the anomalous coronary artery. The anomalous coronary artery anatomy was classified as in Table 1.
Surgical Technique Preexisting systemic to pulmonary artery shunts were interrupted in the accepted manner. All patients underwent repair on cardiopulmonary bypass established with ascending aortic and bicaval cannulation, at moderate hypothermia, and with sanguinous cardioplegia given through the ascending aorta. In all patients, after venting the left heart through the right superior pulmonary vein, the right atrium was opened and the tricuspid valve retracted to expose the ventricular septal defect. Circumferential resection of the parietal and septal bands and the hypertrophied anterior wall of the right ventricular outflow tract was then done up to the level of the pulmonary annulus, taking care to leave a margin for ventricular septal defect closure. Following ventricular septal defect closure, the pulmonary valve leaflets were inspected and the annulus sized. A pulmonary annulus 2 mm larger than the mean normal diameter [16], was ensured. If there was any doubt about the adequacy of the pulmonary annulus, the main pulmonary artery was opened longitudinally. An annulus that was smaller than acceptable was incised and the pulmonary arteriotomy was extended across the annulus into the right ventricle to a distance of about 3 to 4 mm from, and parallel to, the anomalous vessel. The combination of a small pulmonary annulus and a coronary artery so close as to prevent enlargement of the restrictive annulus was indication for an extracardiac conduit. In patients with immobile, thick pulmonary valve cusps, the pulmonary valve leaflets were also excised. Finally, the right ventricle outflow tract was reconstructed using a patch of pericardium treated with 0.6% glutaraldehyde for 20 minutes. No additional method was utilized to deal with any resulting pulmonary valve incompetence.
Evaluation and Follow-up Before discharge from the hospital, all patients had assessment of right ventricle to pulmonary artery gradi-
ents by transthoracic echocardiography. A gradient greater than 30 mm Hg across the right ventricular outflow tract was considered significant. All patients reporting for follow-up were subjected to transthoracic echocardiography and, if thought necessary, cardiac catheterization and angiography. On followup, cardiac catheterization and angiography was done in all patients where gradients were more than 20 mm Hg on echocardiography. Early and late postoperative gradients were compared using paired Student’s t test, and a p value of less than 0.05 was considered significant.
Results Twenty-three patients required a transannular patch to enlarge a restrictive pulmonary annulus. Two patients were operated by a pure transatrial approach because of an adequate pulmonary annulus and normal pulmonary valve leaflets. There was no operative mortality. Early postoperative gradient in these 25 patients was 24.0 ⫾ 13.2 mm Hg (range 8 to 45 mm Hg). So far, none of the patients has needed reoperation. Although all 25 patients were alive and in New York Heart Association functional class I and off all medications at last follow-up, only 18 patients reported for evaluation at a mean follow-up period of 3.2 years (range 2 to 5 years). The preoperative, early, and late postoperative gradients are shown in Table 2. Mean gradient at this time was found to be 20.6 ⫾ 12.4 mm Hg (range 10 to 55 mm Hg) which was statistically not different from the early postoperative gradient of 23.5 ⫾ 13.4 mm Hg (t ⫽ 0.67, p ⬎ 0.10). Immediate postoperative gradients were divided into three categories ie, those with gradients less than 20 mm Hg, between 20 and 40 mm Hg, and those in excess of 40 mm Hg (Fig 1). Late measurement of gradients across the right ventricular outflow tract revealed the following results. Eight patients had gradients less than 20 mm Hg immediately after repair. Six of these stayed below 20 mm Hg at late evaluation. Two however, registered an increase of gradient, ie, from 16 mm Hg to 26 mm Hg and from 10 mm Hg to 30 mm Hg. None of these elevations were considered significant. Seven patients had right-sided gradients between 20 and 40 mm Hg following repair. In this group of patients, late gradients stayed constant in 5, had dropped to 10 mm Hg from 22 mm Hg in 1 patient, and had increased to 55 mm Hg from 36 mm Hg in another patient. Three patients with gradients in excess of 40 mm Hg following repair showed persistence of the same magnitude of gradient in 1, reduction to 30 mm Hg in 1, and 10 mm Hg in another.
Comment Nonconduit methods of dealing with tetralogy of Fallot with abnormal coronary artery anatomy include a transventricular approach with the ventriculotomy placed parallel to the abnormal coronary artery [3]. Other approaches include construction of a double barrel outlet in
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Table 2. Preoperative, Early, and Late Postoperative Gradientsa
No.
Preop RV-PA Gradient (mm Hg)
1. 80 2. 90 3. 82 4. 90 5. 80 6. 74 7. 75 8. 80 9. 92 10. 100 11. 94 12. 80 13. 94 14. 80 15. 90 16. 94 17. 70 18. 90 Mean 84.7 mm Hg SD ⫾ 7.9
Previous Surgery
Nature of RVOT Reconstruction
... ... ... ... BTS ... ... ... ... ... ... BTS ... ... ... ... BTS ...
TAP TAP TAP TAP TAP TAP TAP TAP TAP TAP TAP NPb TAP NPb TAP TAP TAP TAP
Postop RV-PA Gradient (mm Hg) at Discharge
Late Postop RV-PA Gradient (mm Hg)
Time Since Operation (Mo)
13 36 10 30 24 10 16 8 10 36 45 30 48 8 10 43 22 24 23.5 mm Hg ⫾ 13.37
10 20 10 22 20 10 26 13 30 55 30 25 10 10 10 40 10 20 20.6 mm Hg ⫾ 12.44
24 48 25 49 50 52 50 48 62 36 28 26 29 53 26 52 24 50 40.6 mo ⫾ 12.9
a Gradients mentioned are differences between respective peak systolic pressures estimated by echocardiography or cardiac catheterization. transatrial approach without pulmonary arteriotomy or right ventriculotomy.
BTS ⫽ Blalock-Taussig shunt; NP ⫽ no patch; RVOT ⫽ right ventricular outflow tract; SD ⫽ standard deviation; TAP ⫽ transannular patch.
patients with a restrictive pulmonary annulus where the anterior wall of the main pulmonary artery is reflected down and sutured to the superior edge of the oblique ventriculotomy and the anterior defect patched with pericardium [17]. Finally, mobilization of the abnormal coronary artery with the right ventricular patch placed underneath it has also been described [18]. An internal thoracic artery to left anterior descending anastomosis
Fig 1. Distribution of early and late postoperative gradients. (RV ⫽ right ventricle; PA ⫽ pulmonary artery.)
b
Purely
RV-PA ⫽ right ventricular to pulmonary artery;
for an inadvertently injured coronary artery has been used on several occasions [19, 20]. We selectively used a transatrial and, if required, transpulmonary approach to manage this subgroup of patients. That transatrial-transpulmonary repair of tetralogy of Fallot gives excellent results over the short- and long-term is well accepted [3, 6, 8, 9, 12, 13, 21–24]. The technique applied to hearts with an anomalous coronary artery crossing the right ventricular anterior wall is similar except that injury to the vessel in question needs to be assiduously avoided. This may lead to underresection of the hypertrophied anterior wall of the right ventricle for fear of “button holing” of the anterior wall of the right ventricle thereby injuring the anomalous coronary artery and, thus, could lead to higher gradients than in a tetralogy of Fallot heart with coronary arteries in the normal distribution. Of the 7 patients discharged from the hospital with right ventricular outflow gradients of at least 30 mm Hg, repeat study revealed persisting gradients greater than, or equal to, the discharge levels in 2 patients (numbers 10 and 16; Table 2), while in 5 patients the gradients had actually diminished significantly. In one of these patients (number 10), the gradient had actually risen from 36 mm Hg at discharge to 55 mm Hg at follow-up. Other than these 2 patients, all other patients had a right ventricular outflow tract gradient less than or equal to 30 mm Hg at late evaluation.
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In this study, all patients with gradients less than 30 mm Hg continued to have satisfactory relief of right ventricle outflow tract gradients on follow-up. However, gradients greater than 30 mm Hg at discharge behaved inconsistently on late evaluation. Dynamic on-table postrepair gradients contributed by hypercontractile musculature secondary to inotrope usage may be expected to subside with time, whereas gradients because of residual anatomic obstruction may be expected to persist or even worsen with lapse of time [13]. Differentiating between the two etiologies may provide a guide as to need for further resection in the patient with a significant residual gradient. However, persistence of gradient at the time of discharge excludes the possibility of inotropic causation. Possibly the outflow tract may remodel itself over a period of time in some patients. However, because of the retrospective nature of this study, we are unable to point out any differences in these patient categories who have been discharged with a significant gradient and have behaved differently from each other on follow-up. Possibly an intraoperative transesophageal echocardiogram done following repair would help in identifying patients with an anatomic characteristic that predicts progression of postoperative right ventricular outflow tract gradient. Four of our patients (numbers 10, 11, 13, 16; Table 2) had significant gradients across the right ventricular outflow tract at discharge. Though the gradient decreased over the period in 2 of them, it either remained stationery or rather increased in 2 other patients. Thus, clearly, this approach may not be uniformly successful and some patients may need reoperation. In conclusion, the transatrial transpulmonary approach to tetralogy of Fallot, is a suitable option for that subgroup of tetralogy of Fallot with abnormal coronary artery anatomy where repair can be accomplished without an extracardiac conduit. Although most residual gradients improve or do not increase significantly, there is no uniformity. Therefore, continued surveillance is advised to look for aggravation of existing gradients.
References 1. Hurwitz RA, Smith W, King H, Girod DA, Caldwell RL. Total correction with abnormal coronary artery 1967–1977. J Thorac Cardiovas Surg 1980;80:129–34. 2. Berry BE, McGoon DC. Total correction for tetralogy of Fallot with anomalous coronary artery. Surg 1973;74:894– 8. 3. Humes RA, Driscoll JD, Danielson GK, Puga FJ. Tetralogy of Fallot with anomalous origin of left anterior decending artery: surgical options. J Thorac Cardiovasc Surg 1987;94: 784–7. 4. Daskalopoulos DA, Edward WD, Driscoll DJ, Danielson GK, Puga FJ. Coronary artery compression with fatal myocardial ischemia. J Thorac Cardiovasc Surg 1983;85:546–51. 5. Nollert G, Fischlein T, Bouterwek S, Bohmer C, Klinner W, Reichart B. Long term results of total repair of tetralogy of Fallot in adulthood: 35 years followup in 104 patients corrected at the age of 18 years or older. Am J Cardiol 1997;30: 1374– 83.
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6. Atallah-Yunes NH, Kavey RE, Bove EL, Smith FC, Kveselis DA, Byrum CJ, Gaum WE. Postoperative assessment of modified surgical approach to repair tetralogy of Fallot. Long term follow up. Circulation 1996;94(Suppl 9):1122. 7. Horneffer PJ, Zahka KG, Rowe SA, Manolio TA, Gott VA, Reitz BA, Gandner TJ. Long term results of total repair of tetralogy of Fallot in childhood. Ann Thorac Surg 1990;50: 179– 85. 8. Coles JG, Kirklin JW, Pacifico AD, Kirklin JK, Blackstone EH. The relief of pulmonary stenosis by a transatrial versus a transventricular approach to the repair of tetralogy of Fallot. Ann Thorac Surg 1988;45:7–10. 9. Edmunds LH, Saxena NC, Friedman S, Rashkind WJ, Dodd PF. Transatrial approach repair of tetralogy of Fallot. Surg 1976;80:681– 8. 10. Lillehei CW, Warden HE, DeWall RA, et al. The first open heart corrections of tetralogy of Fallots. A 26 –31 year follow up of 106 patients. Ann Surg 1986;204:490 –502. 11. Pacifico AD, Sand ME, Bargeron LM Jr, Colvin EC. Transatrial transpulmonary repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 1987;93:919–24. 12. Kawashima Y, Matsuda H, Hirose H, et al. Ninety consecutive corrective operations for tetralogy of Fallot with or without minimal right ventriculotomy. J Thorac Cardiovasc Surg 1985;90:856– 63. 13. Kaushal SK, Iyer KS, Sharma R, et al. Surgical experience with total correction of tetralogy of Fallot in infancy. Int J Cardiol 1996;56:35– 40. 14. Hudspeth AS, Cordell AR, Johnston FR. Transatrial approach to total correction of tetralogy of Fallot. Circulation 1963;27:796 – 801. 15. Dietl CA, Torres AR, Cazzaniga ME, Favaloro RG. Right atrial approach for surgical correction of tetralogy of Fallot. Ann Thorac Surg 1989;47:546–51. 16. Rowlatt UF, Rimoldi HJ, Lev M. The quantitative anatomy of the normal child’s heart. Pediatr Clin North Am 1963;10:499 – 558. 17. van Son JA. Repair of tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. J Thorac Cardiovasc Surg 1995;110:561–2. 18. Bonchek LI. A method of outflow tract reconstruction tetralogy of Fallot with anomalous anterior descending coronary artery. Ann Thorac Surg 1976;21:451–3. 19. Bhutani AK, Koppala MM, Abraham KA, Balakrishnan KR, Desai RN. Inadvertent transection of anomalously arising left anterior descending artery during tetralogy of Fallot repair: bypass grafting with left internal mammary artery [letter]. J Thorac Cardiovasc Surg 1994;108:589–90. 20. Cooley DA, Duncan JM, Gillette PC, McNamara DG. Reconstruction of coronary artery anomaly in an infant using the internal mammary artery: 10-year follow-up. Pediatr Cardiol 1987;8:257–9. 21. Karl TR, Sano S, Pornviliwan S, Mee RB. Tetralogy of Fallot: favorable outcome of nonneonatal transatrial, transpulmonary repair. Ann Thorac Surg 1992;54:903–7. 22. Binet JP. Correction of tetralogy of Fallot with combined transatrial and pulmonary approach experience with 184 consecutive cases. J Card Surg 1987;93:919–24. 23. Wu Q. Indications and techniques of total correction of tetralogy of Fallot in 228 patients. Ann Thorac Surg 1996;61: 1769–74. 24. Kaushal SK, Sharma R, Iyer KS, Kothari SS, Venugopal PV. Randomised trial of transatrial-transpulmonary and transventricular approaches to repair of tetralogy of Fallot. Cardiol Young 1997;3:323–31.