Double outlet ventricle is present when all of one great vessel and more than 50% of the second great vessel arise from one ventricle. The presence of pul- monary stenosis protects the lungs from overperfusion, and in its absence the lungs are flooded and there is congestive heart failure. Total correction is under- taken at any age when symptoms dictate. Surgery is performed with cardiopul- monary bypass, moderate hypothermia, aortic cross-clamping, and cardioplegia with local cardiac cooling.
10-1. Double Outlet Right Ventricle
FIGURE 10-1. In this infant, the great vessels and semilunar valves are oriented side by side and at the same level.
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ventricular septal defect
FIGURE 10-2. After placing the child on bypass with aortic clamping and cardioplegia, a high longitudinal right ventriculotomy is made and extended cephalad into the proximal main pulmonary artery. There is a common annulus between the semilunar valves and both arise from the right ventricle. The ventricular septal defect is subaortic.
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ventricular septal defect
ventricular septum
FIGURE 10-3. The ventricular septal defect (VSD) is restrictive and will be enlarged ante- riorly or toward the infant's left.
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ventricular septal incision
FIGURE 10-4. The ventricular septum is incised in a more cephalad area and just below the pulmonary valve annulus, keeping the incision remote from the His bundle.
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pulmonary valve
aortic valve
ventricular septal defect
FIGURE 10-5. Interrupted felted mattress sutures are placed along the rim of the VSD. In the posterior inferior corner of the VSD, caution is used to avoid damage to the His bundle, similar to repair of tetralogy of Fallot. Working cephalad from this point, stitches are placed to the right of the aortic valve annulus and then along the cephalad rim of that annulus. In contradistinction to repair of tetralogy, there is a greater distance from the posterior inferior corner of the VSD to the upper right aspect of the aortic valve annulus.
A greater number of sutures will be required in this area.
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FIGURE 10-6. The Dacron® patch is cut in a somewhat rectangular shape that is needed to construct the tunnel from the VSD to the aortic valve. The longer part of the patch is positioned from left to right. A redundant patch is placed to avoid left ventricle to aortic obstruction. The aortic valve is now beneath the patch, while the pulmonary valve leaflets are on the right ventricular side of the patch.
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intra ventricular baffle
FIGURE 10-7. Stitches are tied as the patch is lowered into position. The upper margin of the patch will be attached directly to the common annulus between the semilunar valves or in the base of the pulmonary valve leaflets, being careful to avoid injury to the aortic valve leaflets.
outflow tract patch
FIGURE 10-8. A Gore-Tex® patch is used to reconstruct the outflow tract in order to avoid right ventricular outlet obstruction caused by the bulging intraventricular baffle.
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outflow tract patch
FIGURE 10-9. The repair is complete with placement of the outflow tract patch.
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main left coronary artery right coronary artery
FIGURE 10-10. In this child, double outlet left ventricle is associated with an anomalous right coronary artery that arises from the main left coronary artery and courses on the surface of the upper right ventricle.
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aortic valve
pulmonary valve
ventricular septum
ventricular septal defect
FIGURE 10-11. After cardiopulmonary bypass is established, with aortic clamping, cardio- plegia, and profound local cardiac cooling, a high right ventriculotomy is made trans- versely in anticipation of placement of an extracardiac conduit. The aortic valve is posterior and rightward, while the pulmonary valve is to the child's left. Both valves are seen through the VSD and are on the left ventricular side of the septum.
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common annulus
aortic valve
fibrous tissue remnant of membranous septum
anterior leaf of tricuspid valve
FIGURE 10-12. The ventricular septum is retracted anteriorly and the common annulus between the pulmonary and aortic valves is seen. A fibrous tissue remnant of underde- veloped membranous ventricular septum is seen at the posterior inferior corner of the VSD adjacent to the tricuspid valve leaflet. Repair stitches can be safely placed in this fibrous tissue and in the base of the anterior tricuspid valve leaflet to avoid damage to the His' bundle. Multiple interrupted felted mattress sutures will be used for the repair, start- ing at the posterior inferior rim of the ventricular septal defect. Stitches will be placed around the rim of the VSD to the left of the common annulus and between the pulmonary and aortic valve annuli toward the patient's right.
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R^- - • L
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pulmonary valve
patch
FIGURE 10-13. Stitches are placed in the rectangular-shaped knitted Dacron® patch and tied. The length of the rectangular patch is placed from side to side to cover the VSD and the aortic valve. The pulmonary valve remains on the right ventricular side of the patch.
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porcine valved Dacron conduit
FIGURE 10-14. To avoid right ventricular outflow tract obstruction caused by the large intraventricular baffle, a porcine-valved Dacron® conduit is placed from the ventriculo- tomy to the main pulmonary artery. The integrity of the right coronary artery is maintained.
main pulmonary artery
aorta
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FIGURE 10-15. In another patient, the great vessels are normally related, but the proximal main pulmonary artery is in a more posterior location.
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ventricular septum
ventricular septal defect
FIGURE 10-16. After cardiopulmonary bypass is established and with aortic clamping, car- dioplegia, and profound local cardiac cooling, a high longitudinal right ventriculotomy is made. The VSD is seen, but neither semilunar valve is related to the right ventricle. Only ventricular septum is seen in the cephalad region of the right ventricle because the pul- monary valve is not found here.
upper infundibulum
area of pulmonic and aortic valve
FIGURE 10-17. The ventricular septum is retracted, and the blind end of the right ventric- ular infundibulum is seen. The pulmonic and aortic valves are on the left ventricular side of the septum.
right ventriculotomy
FIGURE 10-18. A longitudinal main pulmonary arteriotomy is made and the pulmonic valve is seen. An instrument placed through the valve passes into the left ventricle to confirm the presence of the double outlet left ventricle.
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caud
area of common annulus of aortic and pulmonic valves
baffle patch
FIGURE 10-19. The ventriculotomy is extended to the pulmonary arteriotomy. Multiple interrupted felted mattress sutures are placed around the rim of the VSD. In the cepha- lad area, these stitches are placed in the common annulus of the semilunar valves, while, to the patient's right, stitches are placed in front of the aortic valve annulus. A rectangu- lar-shaped Dacron® patch is then positioned to construct the intraventricular baffle. The right ventricular outflow tract will be reconstructed with a patch over the ventriculotomy and the proximal main pulmonary artery.
