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Ann Thorac Surg 1999;67:1137-1141
© 1999 The Society of Thoracic Surgeons

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Original Articles

Direct neonatal ventriculo-arterial connections (REV): early results and future implications

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

Accepted for publication September 30, 1998.

Address reprint requests to Dr Black, Cardiac Surgery, Lucile Packard Children’s Hospital, Stanford University School of Medicine, Stanford, CA 94305-5407

	Abstract

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Background. Is the time-honored supposition correct that insertion of a competent prosthetic conduit or homograft is necessary to achieve right ventricular (RV) to pulmonary artery continuity in neonates? A direct ventriculo-arterial connection, or réparation à l’étage ventriculaire (REV), has been successfully used to achieve RV to pulmonary artery continuity in neonates without mortality or major morbidity. Acute RV function is preserved even in the face of free pulmonary insufficiency, but scrupulous preservation of pulmonary artery patency (unobstructed pathway) must be achieved.

Methods. We retrospectively reviewed the cases of 6 neonates who underwent direct ventriculo-arterial connection to achieve RV to pulmonary artery continuity during open heart procedures in the last 3 years.

Results. The 6 neonates had a mean age of 12.3 days (range, 2 days to 6 weeks) and a mean weight of 3.2 kg (range, 2.7 to 3.6 kg) at operation. Two of them had a diagnosis of aortic atresia + ventricular septal defect and successfully achieved an in-series circulation. Two had pulmonary atresia + ventricular septal defect and 2, double-outlet right ventricle + transposition of the great arteries + ventricular septal defect + pulmonary atresia. Follow-up is a mean of 16 months (range, 6 to 22 months). Surgical reintervention was required in 3 neonates and resulted in excellent hemodynamics in 2 of them. In the other patient, who had bilateral long-segment branch pulmonary artery hypoplasia, stents were placed without hemodynamic benefit. All children are currently alive with preserved RV function even in the presence of free pulmonary insufficiency.

Conclusions. Although the creation of a direct ventriculo-arterial connection has routinely been used for children older than 1 year with satisfactory initial results, its application in neonate is very limited. Why have neonates been denied this viable alternative? Perhaps the answer involves legitimate concerns about the consequent free pulmonary insufficiency and the presumed acute RV diastolic dysfunction.

	Introduction

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Is the time-honored supposition correct that insertion of a competent prosthetic conduit, a homograft, or both is necessary to achieve right ventricular (RV) to pulmonary artery (PA) continuity in neonates? Reconstruction of the RV outflow tract is commonly performed with a valved allograft, a prosthetic conduit, or both to prevent early RV failure, especially in the presence of abnormal PA arborization, major tricuspid regurgitation, and persistent pulmonary hypertension. However, failure of both the valve and the conduit can be expected, even within a short time after the original surgical procedure. The creation of a direct ventriculo-arterial connection between the right ventricle and the PA (réparation à l’étage ventriculaire [REV]), with or without a Lecompte maneuver, in neonates may prevent several subsequent reoperations for conduit failure. Late insertion of a pulmonary valve is anticipated to preserve RV function. However, the avoidance of multiple early conduit replacements should be beneficial to the neonate or child, especially as the current supply of appropriately sized grafts remains limited.

	Material and methods

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We used the REV to correct complex congenital heart defects in 6 neonates at The Hospital for Sick Children in Toronto.

Surgical technique with Lecompte maneuver
This technique has been used in patients with aortic atresia + ventricular septal defect (VSD) (Fig 1A). Once the nadir temperature is achieved, blood cardioplegia is administered antegrade through the PA (the descending aorta and brachiocephalic vessels are snared). The pump is stopped and the neonate, exsanguinated. The main PA is transected just proximal to the confluence of the branch PAs. The ductus arteriosus is ligated and divided. Remnants of ductal tissue in the descending aorta are extracted, and the transverse arch is incised on its undersurface.

View larger version (123K): [in this window] [in a new window]   Fig 1. (A) Anatomy in patient with aortic atresia + ventricular septal defect (VSD) prior to surgical intervention. (B) The ascending aorta (Asc. Ao) is transected, beveled, and anastomosed in end-to-side fashion to the neo-aortic root (neo-Ao). A Lecompte maneuver is performed in addition to reconstruction of the transverse aortic arch. A rotational flap of descending aorta to the transverse arch is believed to lessen the occurrence of recoarctation of the aorta. (C) Repair of aortic atresia + VSD. Through a right ventriculotomy made to assist with VSD closure, additional muscle bundles are excised or transected. The ventriculotomy is extended as cephalad as possible to assist in creation of the direct ventriculo-arterial connection (réparation à l’étage ventriculaire [REV]). (D) The right ventricular outflow tract is completed by attaching the posterior wall of the pulmonary artery confluence to the most superior aspect of the right ventriculotomy. A hood of homograft or autologous pericardium treated in glutaraldehyde completes the repair. (Reprinted from Black MD, Smallhorn JF, Freedom RM. Aortic atresia with ventricular septal defect: modified single-stage neonatal biventricular repair. Ann Thorac Surg 1999;67:751–5.)

The distal severed end (ascending aorta) is doubly ligated. A second dose of cardioplegia is administered antegrade down the proximal ascending aorta with a No. 16 angiocatheter. The ascending aorta is incised and spatulated so that it can be anastomosed end-to-side to the proximal neo-aorta (Fig 1B). A coronary punch is used to create the defect in the neo-aorta (approximately 3 mm in diameter), and then the anastomosis is completed using a partial occluding clamp and 8-0 polypropylene suture (Prolene; Ethicon, Inc Somerville, NJ). The commissural marking sutures previously placed are now used to avoid iatrogenic injury to the neo–aortic valve. A patent foramen ovale is reconstituted from an ostium secundum atrial defect through a right atriotomy.

The subarterial VSD is identified through a transverse right ventriculotomy. Closure and baffling of the left ventricle to the neo-aorta are accomplished with a Dacron sauvage patch and interrupted pledgeted sutures. A strip of pericardium reinforces the epicardial surface of the right ventriculotomy (most cephalad) in addition to providing security to the RV–PA anastomosis (REV) (Fig 1C). The aortic cross-clamp is removed after air is removed from the heart. The neonate is fully rewarmed. A hood of pericardium completes the RV–PA connection (Fig 1D).

We wean patients from cardiopulmonary bypass with a standard amount of inotropic support (5 µg · kg^-1 · min^-1 of dopamine hydrochloride and 2 µg · kg^-1 · min^-1 of sodium nitroprusside). Intraoperative transesophageal echocardiography and monitoring of RV and PA are performed.

Surgical technique without Lecompte maneuver
This technique has been used in patients with pulmonary atresia + VSD or double-outlet right ventricle + pulmonary atresia + transposition of the great arteries + VSD. Once the intracardiac defects have been surgically addressed, a main pulmonary arteriotomy is fashioned and extended cephalad to one or both branch PAs (commonly present, left PA stenosis secondary to a ductal ring). Extension of the main pulmonary arteriotomy caudad toward the right ventriculotomy allows removal of concomitant obstructing muscle bundles. The main PA is separated from the heart. The RV outflow tract is not simply reconstructed with an outflow tract patch because of concerns about iatrogenic stenosis at the level of the primitive pulmonary valve annulus (plate). Frequently there is a gap or posterior malalignment of the PA relative to the RV cavity. To rectify this potential iatrogenic outflow tract stenosis and "kink," an absorbable continuous monofilament suture is used to secure the main PA to the superior end of the ventriculotomy. The main PA is carefully spread across the ventriculotomy to maintain a larger surface area and thus provide greater future growth potential. The ventriculotomy remains positioned as cephalad as possible to create a laminar pathway for the egress of blood from the right ventricle. A hood of pericardium completes the repair.

	Results

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Six neonates underwent correction of complex congenital heart defects with an REV at our institution with no mortality (Table 1). The mean age was 12.3 days (range, 2 days to 6 weeks [uncorrected age of 1,800-g premature infant]) and the mean weight, 3.2 kg (range, 2.7 to 3.6 kg). All neonates had biventricular corrections and remain well during the early follow-up. Intraoperatively all repairs were verified as being satisfactory by direct needle measurement of RV pressures and transesophageal echocardiograms.

View larger version (163K): [in this window] [in a new window]   Fig 2. (Patients.) Angiogram demonstrating patent direct ventriculo-arterial connection (réparation à l’étage ventriculaire [REV]). Although there is no discrete stenosis, stretching of the branch pulmonary arteries (PA) is demonstrated. Distal peripheral arborization abnormalities (peripheral pulmonary artery branch stenosis) are indicated (x). (RV = right ventricle.)

	Comment

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Described in 1982, the REV was first 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 [4]. Currently the REV is used for other anomalies of ventriculo-arterial connections, and in our series, it permitted the achievement of RV to PA continuity in neonates, thereby obviating the inevitable early conduit replacements [5–10].

One of the recent accomplishments in neonatal cardiac surgery is the correction of defects in association with pulmonary outflow tract obstruction. In 1969, Rastelli conceived a novel technique to reconstruct the pulmonary outflow tract for the surgical correction of transposition of the great arteries with VSD and pulmonary stenosis, which has provided good results both early and late [11, 12]. However, when unfavorable intracardiac anatomy is present, the surgical correction has been delayed for 5 to 10 years.

Predictable conduit obstruction, valvular incompetence, and inevitable reoperations have provided the impetus to search for a longer-lasting palliative neonatal procedure [12–17]. The ideal valve or arterial conduit has not yet been developed. Children have demonstrated an accelerated propensity for obstruction of right-sided extracardiac valved conduits [16, 17]. Reoperation or reintervention for recurrent RV outflow tract obstruction will be required eventually in all patients with a nonautologous correction, especially if it was done when they were neonates.

Long-term complications are not infrequent when prosthetic conduits are used [16, 17]. Lack of somatic growth remains a cardinal cause of reoperation, as do the development of malignant calcification and the development of an intimal peel [18]. Reconstruction of the pulmonary outflow tract without a prosthetic conduit remains highly desirable.

In this era of early complete repair, we have not denied the neonate the establishment of a normal in-series circulation because of age alone. Recognition of the long-term complications with valves and conduits in children spurred the search for viable alternatives to achieve RV to PA continuity. The REV has been successful in addressing a number of our concerns. In combination with extensive PA mobilization, anterior translocation of the pulmonary bifurcation in some children (ie, aortic atresia + VSD), and direct reimplantation of the main PA trunk into the right ventriculotomy, RV to PA continuity is restored. The completed autologous pathway has the potential for growth. The anterior wall is usually finished with autologous pericardium treated in glutaraldehyde. Although many groups have deferred the REV until the child is 1 year of age, we have taken an aggressive approach and applied the procedure to neonates, with initial success.

We used the REV in 6 neonates with a variety of morphologic abnormalities without mortality or major morbidity. Early reintervention has been required in 3 patients. Right ventricular pressures have remained less than 50% of systemic pressures except in 1 of them. Obstruction caused by the stretching of the proximal branch PAs, previously identified as a potential problem, became evident only in this patient. Concomitant peripheral arterial pathology remains a nidus for persistent RV hypertension, albeit at approximately 65% of systemic pressure.

The other 2 patients required surgical revision of the RV outflow tract. One had excision of RV muscle bundles and the other, revision of the outflow tract patch concomitant with resection of a moderate degree of fibrosis. Both operations were retrospectively regarded as technically simple but had the risks of repeat sternotomy. Replacement with a conduit such as a homograft would likely have been a substantially more technically difficult procedure with a protracted cardiopulmonary bypass time. Right ventricular pressures after reoperation remain less than 50% of systemic levels with well-preserved ventricular function.

When the REV is completed without a Lecompte maneuver (ie, pulmonary atresia + VSD or double-outlet right ventricle + pulmonary atresia + transposition of the great arteries + VSD), the resultant hemodynamics have been excellent (RV pressures less than 35% of systemic pressures). Postoperative pharmacologic management includes the use of amrinone lactate, phenoxybenzamine hydrochloride, or both, with excellent effect. Extrapolation of the REV to other neonatal congenital cardiac abnormalities remains theoretically tantalizing (eg, truncus arteriosus because postoperative pulmonary pressures remain lower than 50% of systemic pressures). There are no long-term results to attest to the superiority of this surgical modification over the standard repair with early repeat conduit replacements. However, with a dwindling supply of appropriately sized homografts, neonatal REV can be considered an acceptable alternative.

	Acknowledgments

Top Abstract Introduction Material and methods Results Comment Acknowledgments References

 
We thank Mr Phil Dakin for his artistic contributions.

	References

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