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Ann Thorac Surg 1999;67:751-755
© 1999 The Society of Thoracic Surgeons

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Original Articles

Aortic atresia with a ventricular septal defect: modified single-stage neonatal biventricular repair

^a Division of Cardiovascular Surgery, The Hospital for Sick Children and The University of Toronto, Toronto, Ontario, Canada
^b Division of Cardiology, The Hospital for Sick Children and The University of Toronto, Toronto, Ontario, Canada

Accepted for publication July 30, 1998.

Address reprint requests to Dr Black, Division of Cardiovascular Surgery, Lucile Packard Children’s Hospital, Stanford University School of Medicine, Stanford, CA 94305-5407
e-mail: michael.black{at}stanford.edu

	Abstract

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
Background. The spectrum of hypoplastic left heart disease is diverse but the surgical repair is strictly dichotomous, culminating in either a univentricular or a biventricular surgical repair. Although aortic atresia with a ventricular septal defect historically has been managed by conversion to a univentricular physiology, a biventricular repair occasionally has been attempted in stages or in conjunction with the implantation of multiple allografts or prosthetic conduits. Our repair strategy recently has evolved to the use of a modified single-stage biventricular repair using only autologous tissues without conduits.

Methods. Retrospective analysis (1989 to 1997) of neonates with aortic atresia with a ventricular septal defect.

Results. Five neonates underwent repair of aortic atresia with a ventricular septal defect. One died in the hospital. The mean age and weight of the neonates who underwent repair were 7.8 days (range, 2 to 17 days) and 3.2 kg (range, 3 to 3.6 kg), respectively. Three neonates had a univentricular repair and 2 had a modified biventricular repair. The latter two procedures were successful and the patients were discharged from the hospital.

Conclusions. Long-term results are lacking to attest to this surgical modification’s superiority over either the standard multistage univentricular operation or the single-stage biventricular repair using multiple conduits. However, we are optimistic that routine use of this modification will enable a greater percentage of neonates to undergo a biventricular repair without the need for serial conduit revisions or future aortoplasty.

	Introduction

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The spectrum of hypoplastic left heart disease is varied and occasionally includes aortic atresia with a large ventricular septal defect (VSD) and two well-developed ventricles. This condition historically has been managed by conversion to a univentricular physiology [1]. A single-stage biventricular repair has been described previously; however, the surgical procedure has involved the implantation of either one or two allografts or prosthetic conduits [2]. Maintenance of retrograde coronary perfusion has been accomplished through a lengthy and minute ascending aorta. We developed a modified single-stage neonatal repair that may enable survival without the need for early conduit revision. In addition, we established a refined physiologic method of coronary blood delivery ("anterograde" flow).

	Material and methods

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Patients
Two neonates were transferred to The Hospital for Sick Children in Toronto, Ontario, Canada. One of the neonates had a provisional diagnosis of a common arterial trunk (ie, truncus arteriosus communis). Transthoracic echocardiography demonstrated situs solitus, atrioventricular concordance, and ventriculoarterial concordance. Aortic atresia was identified, with a large-outlet VSD and two well-developed and functioning ventricles. The ascending aorta measured 0.20 to 0.26 cm, with the transverse arch approaching 0.43 to 0.48 cm. Retrograde coronary blood flow was identified originating from the large patent ductus arteriosus. The branch pulmonary arteries were enlarged (0.59 to 0.80 cm). Prostaglandins were administered and the neonates were maintained spontaneously ventilated on room air and prepared for surgical intervention. The neonates were managed with a single ventricular physiology (Fig 1A).

View larger version (125K): [in this window] [in a new window]   Fig 1. (A) Repair of aortic atresia with a ventricular septal defect. Note the diminutive size of the ascending aorta as a result of its supply of only retrograde coronary blood flow. (B) The ascending aorta is transected and beveled, then reimplanted end-to-side in the neoaortic root. A Lecompte maneuver is performed in addition to reconstruction of the transverse aortic arch. A rotational flap of descending aorta to transverse arch is believed to lessen the occurrence of recoarctation of the aorta. (C) The right ventricular outflow tract is completed by attachment of the posterior wall of the pulmonary arterial confluence to the most superior aspect of the right ventriculotomy. A hood of either homograft or autologous pericardium treated in glutaraldehyde completes the repair. (Asc. AO = ascending aorta; Neo AO = neoaorta; REV = reparation a l’etage ventriculaire.)

Once the nadir temperature was achieved, a dose of anterograde sanguineous cardioplegia was administered through the pulmonary artery (the descending aorta and brachiocephalic vessels were clamped). The pump was stopped and the neonates were exsanguinated. The main pulmonary artery was transected just proximal to the level of the branch pulmonary arteries. The ductus arteriosus was ligated and divided. A Lecompte maneuver was performed. Remnants of ductal tissue inserting into the descending aorta were trimmed and the transverse arch was incised on its undersurface.

Using a modification of techniques described by Bu’Lock and colleagues [3] and by Fraser and Mee [4], the main pulmonary artery was anastomosed to the undersurface of the transverse arch and to the descending aorta (following a rotation flap of descending aorta to transverse aortic arch). In this way, both the arch hypoplasia and the coarctation were repaired with solely autologous tissue (Fig 1B). Marking sutures were placed strategically along the commissures. The aorta was deaired carefully and the patients were recannulated through the previous pursestring, which was now the proximal neoaorta. The snares on the brachiocephalic vessels were removed.

The neonates were rewarmed to approximately 25°C. The ascending aorta was transected at its insertion site on the transverse arch. The distal end was ligated. A second selective dose of cardioplegia was administered anterograde down the proximal ascending aorta with a no. 16 Angiocath (Jeko, Johnson and Johnson, Arlington, TX). The ascending aorta was incised and spatulated so that it could be anastomosed to the proximal neoaorta (extended end-to-side). A coronary punch was used to create a defect in the neoaorta (approximately 3 mm in diameter) and the anastomosis was completed using a partial occluding clamp and 8-0 polypropylene sutures (Prolene; Ethicon, Inc, Somerville, NJ). The commissural marking sutures were used to avoid iatrogenic neoaortic valve injury. A patent foramen ovale was reconstituted from an ostium secundum atrial defect through a right atriotomy.

The subarterial VSD was identified through a transverse right ventriculotomy. Closure and baffling of the left ventricle to the neoaorta was accomplished with a Dacron (C. R. Bard, Haverhill, PA) synthetic patch and a running pledgeted monofilament suture. A strip of pericardium reinforced the epicardial surface of the right ventriculotomy (most cephalad) and provided security for the direct pulmonary arterial-to-right ventricular anastomosis (reparation a l’etage ventriculaire).

The aortic cross-clamp was removed after careful deairing of the heart. The neonates were fully rewarmed. Electrical and mechanical activity resumed. A hood of pericardium completed the pulmonary arterial-to-right ventricular connection once the reparation a l’etage ventriculaire had been performed (Fig 1C).

The patients were weaned from cardiopulmonary bypass with a moderate amount of inotropic support. Transesophageal echocardiography demonstrated good surgical repair (ie, no VSD leaks, good biventricular function, no left ventricular outflow tract obstruction, and a minimal gradient across the branch pulmonary arteries). In one neonate, because of free pulmonary insufficiency associated with flow disturbance across the ostia of the branch pulmonary arteries, a planned diagnostic cardiac catheterization was performed before the patient was discharged from the hospital to ascertain anatomic sites of possible branch pulmonary arterial stenosis.

	Results

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
Five neonates underwent correction of aortic atresia with a VSD at our institution; one died in the hospital. The mean age and weight of the neonates who underwent surgical repair were 7.8 days (range, 2 to 17 days) and 3.2 kg (range, 3 to 3.6 kg), respectively. Three neonates underwent conversion to a univentricular physiology (1 died in the hospital). The remaining 2 neonates underwent the modified single-stage biventricular repair. Their procedures were successful and they were discharged from the hospital with an in-series circulation (Table 1).

View larger version (176K): [in this window] [in a new window]   Fig 2. Angiogram demonstrating a patent reparation a l’etage ventriculaire connection. Although there is no discrete stenosis, "stretching" of the branch pulmonary arteries is demonstrated.

The second neonate required reoperation approximately 10 months after the first procedure because of recurrent right ventricular outflow tract obstruction isolated within the right ventricle. Muscle bundles were excised successfully through the previously placed pericardial hood. The branch pulmonary arteries were of good caliber and required no surgical intervention. Approximately 19 months after the initial surgical intervention, the child has a biventricular repair and free pulmonary insufficiency with right ventricular systemic pressures of less than 50%.

	Comment

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
Aortic atresia is not invariably synonymous with hypoplastic left heart syndrome. In the spectrum of hypoplastic disease, aortic atresia with two well-developed ventricles occurs in 4% to 6% of cases, almost always associated with a VSD. Fusion of the infundibular septum to the left ventriculoinfundibular fold (concordant atrioventricular and ventriculoatrial connections) obliterates the subaortic area. A defect thus remains in the ventricular septum (infundibular-malalignment type). Normal inflow to the ventricle occurs independent of these embryologic malformations [5].

Until recently, the standard surgical approach to the treatment of children with aortic atresia was a multistage single ventricular repair using the morphologically right ventricle as the systemic ventricle [1, 6, 7]. The work of Freedom and colleagues [9] made a biventricular repair a theoretically possible surgical alternative. A two-stage approach thus evolved, with palliation early in infancy and repair at a later date. The use of prosthetic material and homograft conduits was the rule [9, 10–13]. Long-term results were less than satisfactory [2, 14, 15].

The first description of a single-stage repair of aortic atresia with a normal left ventricle in a neonate was published by Austin and associates [2]. Unfortunately, the incorporation of several homografts was required and recognized by the authors as a major pitfall of their newly described technique. Because of the high fatality rate associated with the single-stage neonatal operation, several institutions continue to use a Norwood operation as a multistage alternative that provides palliation to a "single" ventricular physiology. Could two well-formed ventricles provide an extra element of safety in patients who undergo a Norwood procedure? Although specific data are not yet available, recent publications provide support for this hypothesis. Ventricular systole (ie, atrial relaxation, atrioventricular valve descent) has a great influence on the transpulmonary blood flow in the functional single ventricle [6]. Jacobs and colleagues [7] suggest the possibility of late conversion to a biventricular repair, noting that "applying the Norwood operation in the neonatal period does not by any means obviate the possibility of a biventricular repair later on." One theoretic disadvantage of performing this delayed conversion at 3 to 6 months of age (with an early LeCompte maneuver combined with a systemic-to-pulmonary artery shunt) is the early reliance on the pulmonary vascular resistance as a variable in postoperative recovery.

Although it first was described in 1982, réparation à létage ventriculaire first was used as an adjuvant to septal resection for the repair of classic transposition of the great arteries in association with a VSD and pulmonary outflow tract obstruction [16, 17]. Réparation à l’étage ventriculaire has been used for the modification described herein and for other anomalies of ventriculoarterial connections. It has allowed the achievement of right ventricular-to-pulmonary arterial continuity in neonates, supplanting the need for inevitable early conduit changes [18].

Our modified surgical strategy was chosen more on a temporal basis than on any anatomic or structural considerations. Surgical implementation of this modified technique was based on our desire to maintain a biventricular repair in our patients (especially because both ventricles were well formed), establish little reliance on the pulmonary vascular resistance for postoperative management, avoid volume loading and induced ventricular hypertrophy through a palliative systemic-to-pulmonary shunt, and avoid the need for conduit changes with somatic growth. The complete avoidance of prosthetic or homograft conduits is appealing given the predictable failure of the small conduits initially implanted at our institution [19]. Extrapolation of this technique to other neonatal congenital cardiac abnormalities is theoretically appealing (ie, pulmonary atresia with VSD or double-outlet right ventricle with transposition of the great arteries and pulmonary stenosis) because postoperative systemic pulmonary pressures are less than 50% [17]. Postoperative pharmacologic management has been enhanced with the use of amrinone and phenoxybenzamine, with excellent hemodynamic (right ventricular) effects. In addition, a prophylactic peritoneal drain has been useful in the postoperative period.

Long-term results are lacking to attest to this surgical modification’s superiority over either the standard multistage univentricular operation or the single-stage biventricular repair using the conduits described earlier. However, both of our patients are currently doing well, with right and left ventricular pressures of less than 50% and no evidence of recurrent left ventricular outflow tract obstruction. These cases demonstrate that an in-series neonatal circulation (biventricular repair) can be achieved even in patients with hypoplastic left heart disease and diminutive aortic valves, as long as the left atrioventricular valve is nearly "normal" [20]. [8]

	Acknowledgments

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
We acknowledge Mr Phil Dakin for his artistic contributions.

	References

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 

1. Rychik J., Murdison K.A., Chin A.J., Norwood W.I. Surgical management of severe aortic outflow obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis. J Am Coll Cardiol 1991;18:809-816.[Abstract]
2. Austin E.H., Jonas R.A., Mayer J.E., Jr, Castaneda A.R. Aortic atresia and normal left ventricle. Single-stage repair in the neonate. J Thorac Cardiovasc Surg 1989;97:392-395.[Abstract]
3. Bu’Lock F.A., Stumper O., Jagtap R., et al. Surgery for infants with a hypoplastic systemic ventricle and severe outflow obstruction: early results with a modified Norwood procedure. Br Heart J 1995;73:456-461.[Abstract/Free Full Text]
4. Fraser C.D., Jr, Mee R.B.B. Modified Norwood procedure for hypoplastic left heart syndrome. Ann Thorac Surg 1995;60:S546-S549.
5. Freedom R.M., Williams W.G., Dische M.R., Rowe R.D. Anatomical variants in aortic atresia. Potential candidates for ventriculoaortic reconstitution. Br Heart J 1976;38:821-826.[Abstract/Free Full Text]
6. Rychik J., Fogel M.A., Donofrio M.T., et al. Comparison of patterns of pulmonary venous blood flow in the functional single ventricle heart after operative aortopulmonary shunt versus superior cavopulmonary shunt. Am J Cardiol 1997;80:922-926.[Medline]
7. Jacobs M.L., Rychik J., Murphy J.D., Nicolson S.C., Steven J.M., Norwood W.I. Results of Norwood’s operation for lesions other than hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 1995;110:1555-1562.[Abstract/Free Full Text]
8. Kanter K.R., Miller B.E., Cuadrado A.G., Vincent R.N. Successful application of the Norwood procedure for infants without hypoplastic left heart syndrome. Ann Thorac Surg 1995;59:301-304.[Abstract/Free Full Text]
9. Freedom R.M., Culham J.A.G., Rowe R.D. Aortic atresia is not synonymous with the hypoplastic left heart syndrome: an analysis of the variable expression of hearts with aortic atresia and a ‘normal’ left ventricle. Pediatr Cardiol 1988;9:196.
10. Yasui H., Kado H., Nakano E., et al. Primary repair of interrupted aortic arch and severe aortic stenosis in neonates. J Thorac Cardiovasc Surg 1987;93:539-545.[Abstract]
11. Thiene G., Gallucci V., Macartney F.J., Del Torso S., Pelegrino P.A., Anderson R.H. Anatomy of aortic atresia. Cases presenting with a ventricular septal defect. Circulation 1979;59:173-178.[Abstract/Free Full Text]
12. Bogers A.J.J.C., Sreeram N., Hess J., Sutherland G.R., Quaegebeur J.M. Aortic atresia with normal left ventricle: one-stage repair in early infancy. Ann Thorac Surg 1991;51:312-314.[Abstract]
13. Barbero-Marcial M., Baucia J.A., Jatene A. Valved conduits of bovine pericardium for right ventricle to pulmonary artery connections. Semin Thorac Cardiovasc Surg 1995;7:148-153.[Medline]
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17. Sakata R., Lecompte Y., Batisse A., Borromee L., Durandy Y. Anatomic repair of anomalies of ventriculoarterial connection associated with ventricular septal defect. I. Criteria of surgical decision. J Thorac Cardiovasc Surg 1988;95:90-95.[Abstract]
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