Aortic Atresia and Hypoplastic Left Heart Syndrome

The Norwood procedure or Stage I palliation or plastic reconstruction of the aortic root is performed in neonates as an urgent operation after medical stabilization, including maintenance of ductal patency to provide adequate systemic perfusion. pH and blood gases are corrected while pulmonary blood flow may be controlled with manipulation of pH and pC02. Stage II or the hemi-Fontan procedure or bidirectional cavopulmonary shunt is usually carried out at 2 to 4 months of age, while Stage III or the completion Fontan operation is generally performed around age 2 years. The clinical condition of the child, including the hemodynamic state, peripheral oxygenation, and other factors, may alter this timing. With appropriate surgery at an experienced center, survival today should be equivalent to all other major congenital heart operations.

Stage I palliation is performed with cardiopulmonary bypass, deep hypothermia (20°C), and cardioplegic arrest. Low-flow cerebral perfusion is used during arch reconstruction and total circulatory arrest is only necessary for the few minutes during which atrial septectomy is carried out.

18-8-1. Norwood Procedure with a Blalock Shunt

Gore Bypass

Gore-Tex graft attached to innominate artery purse strings for subsequent cannulation

Figure 18-66. The severely hypoplastic ascending aorta and large main pulmonary artery are seen. The larger arch and head vessels are dissected. Silks for snaring are passed around the head vessels for later use.

Figure 18-67. A 3.5-mm tubular Gore-Tex® graft is anastomosed to the innominate artery. The arterial perfusion cannula will be placed in the graft and utilized for cardiopulmonary bypass, as well as low-flow cerebral perfusion. A single right atrial appendage venous cannula is used for venous drainage. Purse strings are placed in the proximal main pulmonary artery for later arterial cannulation.

Gore-Tex graft attached to innominate artery purse strings for subsequent cannulation

Transverse Aorta Arch Graft

caud left carotid artery innominate artery transverse arch hypoplastic ascending aorta main pulmonary artery caud

Confluence Innominate Vein
Figure 18-68. Two patches of pulmonary artery homograft wall material are cut. The smaller will be used to close the distal main pulmonary artery or pulmonary arterial confluence, while the larger one is used to construct the neoaorta.

pulmonary artery branch confluence patch ceph

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Pulm Artery Branches

pulmonary artery branch confluence patch

Figure 18-69. The child has been placed on bypass with arterial inflow through the Gore-Tex® graft and venous return through a single right atrial cannula. Much of the dissection is performed during the cooling phase. Silks for snaring are passed around the pulmonary artery branches and head vessels. The ductus arteriosus, aortic isthmus, and upper descending thoracic aorta are dissected. The first dose of cardioplegia can be delivered with a catheter in the proximal main pulmonary artery after snaring all head vessels and the descending thoracic aorta below the ductus. A snare is in place around the proximal innominate artery below the Gore-Tex® graft which will be used later for low-flow cerebral perfusion. The main pulmonary artery is divided proximal to the branches. A drainage catheter is passed through the pulmonary valve to collect excess blood. The ductus arteriosus has been closed with two hemoclips. The smaller homograft pulmonary artery wall patch is being stitched to the open confluence of the pulmonary artery branches that have been snared.

pulmonary artery branch snares proximal main pulmonary artery appendage cannula

Figure 18-69. The child has been placed on bypass with arterial inflow through the Gore-Tex® graft and venous return through a single right atrial cannula. Much of the dissection is performed during the cooling phase. Silks for snaring are passed around the pulmonary artery branches and head vessels. The ductus arteriosus, aortic isthmus, and upper descending thoracic aorta are dissected. The first dose of cardioplegia can be delivered with a catheter in the proximal main pulmonary artery after snaring all head vessels and the descending thoracic aorta below the ductus. A snare is in place around the proximal innominate artery below the Gore-Tex® graft which will be used later for low-flow cerebral perfusion. The main pulmonary artery is divided proximal to the branches. A drainage catheter is passed through the pulmonary valve to collect excess blood. The ductus arteriosus has been closed with two hemoclips. The smaller homograft pulmonary artery wall patch is being stitched to the open confluence of the pulmonary artery branches that have been snared.

Left Lung Artery

left subclavian artery ductus arteriosus

Figure 18-70. Homograft patch closure of the pulmonary artery confluence is complete. Hemoclips on the large ductus arteriosus are seen.

left subclavian artery ductus arteriosus pulmonary artery branch confluence homograft patch

Figure 18-70. Homograft patch closure of the pulmonary artery confluence is complete. Hemoclips on the large ductus arteriosus are seen.

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Pictures Plastic Pulimonary Arteries

head vessel snares arch stump aortotomy descending aorta

Figure 18-71. The distal aortic arch beyond the left subclavian artery is doubly clipped with hemoclips and divided. The aortic isthmus, including any coarctation along with the distal divided ductal stump, are excised. A clamp is placed on the descending thoracic aorta that is moved into the field. The transverse aortic arch is incised along its inferior margin and the aortotomy is extended down the medial aspect of the hypoplastic ascending aorta. Low flow cereleral perfusion is carried out through the Gore-Tex® graft.

head vessel snares arch stump aortotomy descending aorta ceph

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Figure 18-71. The distal aortic arch beyond the left subclavian artery is doubly clipped with hemoclips and divided. The aortic isthmus, including any coarctation along with the distal divided ductal stump, are excised. A clamp is placed on the descending thoracic aorta that is moved into the field. The transverse aortic arch is incised along its inferior margin and the aortotomy is extended down the medial aspect of the hypoplastic ascending aorta. Low flow cereleral perfusion is carried out through the Gore-Tex® graft.

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Coarctation Isthmus Part

Figure 18-72. The descending thoracic aorta is anastomosed to the distal arch and this forms part of the back wall of the neoaorta.

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Coarctation Isthmus Part

ascending aorta suture tine

Figure 18-73. A short incision is made in the medial aspect of the proximal main pulmonary artery. The hypoplastic ascending aorta is incised down to this point. These two vessels are anastomosed with 7.0 Prolene. A 1-mm probe is passed proximally through the ascending aorta to confirm patency down to the coronary orifices.

left subclavian artery arch stump descending aorta arch aortotomy caud

Figure 18-72. The descending thoracic aorta is anastomosed to the distal arch and this forms part of the back wall of the neoaorta.

ascending aorta suture tine

Figure 18-73. A short incision is made in the medial aspect of the proximal main pulmonary artery. The hypoplastic ascending aorta is incised down to this point. These two vessels are anastomosed with 7.0 Prolene. A 1-mm probe is passed proximally through the ascending aorta to confirm patency down to the coronary orifices.

caud proximal main pulmonary artery pulmonary artery branch confluence caud

Pictures Plastic Aorta

homograft patch posterior suture line proximal main pulmonary artery

Figure 18-74. The larger pulmonary artery homograft wall patch is stitched posteriorly to the transverse arch and ascending aorta to complete the back wall of the neoaorta.

homograft patch posterior suture line caud

Figure 18-74. The larger pulmonary artery homograft wall patch is stitched posteriorly to the transverse arch and ascending aorta to complete the back wall of the neoaorta.

proximal main pulmonary artery

Ductal Arch Heart

ductal stump

Figure 18-75. The homograft patch is tailored and then stitched anteriorly to the transverse arch and ascending aorta. It is then connected to the proximal main pulmonary artery, completing construction of the neoaorta. A new arterial perfusion catheter is placed in the proximal neoaorta for arterial inflow. The catheter in the Gore-Tex® graft is removed and this graft will be moved caudad for anastomosis to the pulmonary arteries. The pulmonary artery branch confluence has been moved to beneath the ascending aorta for construction of the modified Blalock shunt.

head vessel and subclavian artery snares native arch homograft patch in neoaorta aortic cannula

Figure 18-75. The homograft patch is tailored and then stitched anteriorly to the transverse arch and ascending aorta. It is then connected to the proximal main pulmonary artery, completing construction of the neoaorta. A new arterial perfusion catheter is placed in the proximal neoaorta for arterial inflow. The catheter in the Gore-Tex® graft is removed and this graft will be moved caudad for anastomosis to the pulmonary arteries. The pulmonary artery branch confluence has been moved to beneath the ascending aorta for construction of the modified Blalock shunt.

ductal stump ceph

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Atrial Septectomy

atrial septal defect

Figure 18-76. A brief period of circulatory arrest is used for atrial septectomy. The venous cannula can be removed from the appendage and exposure of the atrial septum accomplished through this opening.

atrial septal defect

Figure 18-76. A brief period of circulatory arrest is used for atrial septectomy. The venous cannula can be removed from the appendage and exposure of the atrial septum accomplished through this opening.

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modified

Blalock shunt

MV02 catheter right atrial catheters

Figure 18-77. During construction of the Gore-Tex® graft to pulmonary artery anastomosis, rewarming on full bypass is carried out. Here, a metal clip is placed on the Blalock shunt to control flow through same prior to removal from bypass. An MV02 catheter is placed in the superior vena cava and passed into the upper right atrium while two additional catheters are placed directly in the right atrium for fluid and medication infusion.

modified

Blalock shunt

MV02 catheter right atrial catheters

Figure 18-77. During construction of the Gore-Tex® graft to pulmonary artery anastomosis, rewarming on full bypass is carried out. Here, a metal clip is placed on the Blalock shunt to control flow through same prior to removal from bypass. An MV02 catheter is placed in the superior vena cava and passed into the upper right atrium while two additional catheters are placed directly in the right atrium for fluid and medication infusion.

18-8-2. Modified Norwood Procedure with Right Ventricle to Pulmonary Artery Shunt (Sano Procedure)

Innominate Vessel The Heart

innominate artery bulldog damp

Figure 18-78. The innominate artery is isolated with a distal snare and a proximal bulldog clamp. An incision is made in this vessel for the graft anastomosis.

innominate artery bulldog damp

Figure 18-78. The innominate artery is isolated with a distal snare and a proximal bulldog clamp. An incision is made in this vessel for the graft anastomosis.

Innominate Vessel The Heart

Gore-Tex graft

Figure 18-79. A beveled 3.5-mm tubular Gore-Tex® graft will be used.

Gore-Tex graft

Figure 18-79. A beveled 3.5-mm tubular Gore-Tex® graft will be used.

Venous Patch Grafts

Figure 18-80. The Gore-Tex® graft is anastomosed to the innominate artery.

innominate artery main pulmonary artery ascending aorta caud

Innominate Vessel The Heart

Figure 18-81. The arterial perfusion cannula is placed in the Gore-Tex® graft for arterial inflow.

caud arterial cannula

Figure 18-81. The arterial perfusion cannula is placed in the Gore-Tex® graft for arterial inflow.

Pulmonary Artery Cannula

Figure 18-82. A single right atrial venous cannula is utilized.

right atrial cannula

Figure 18-82. A single right atrial venous cannula is utilized.

Pulmonary Artery Cannula

Figure 18-83. A circular homograft pulmonary artery wall patch is used in the construction of a composite Gore-Tex® graft.

homograft patch

Gore-Tex conduit

Figure 18-83. A circular homograft pulmonary artery wall patch is used in the construction of a composite Gore-Tex® graft.

ho mo g raft patch

Figure 18-84. An incision is made in the middle of the homograft patch and the GoreTex® graft is anastomosed to same.

ho mo g raft patch

Figure 18-84. An incision is made in the middle of the homograft patch and the GoreTex® graft is anastomosed to same.

Figure 18-85. The child has been placed on cardiopulmonary bypass and cooled, during which the extensive dissection is performed. Cardioplegic arrest is induced and the ductus arteriosus closed. The main pulmonary artery is divided proximal to the branches. The composite Gore-Tex® conduit is brought into the field for anastomosis to the pulmonary artery branches.

pulmonary artery branch snares pulmonary artery branch confluence composite homograft-Gore-tex conduit

Figure 18-85. The child has been placed on cardiopulmonary bypass and cooled, during which the extensive dissection is performed. Cardioplegic arrest is induced and the ductus arteriosus closed. The main pulmonary artery is divided proximal to the branches. The composite Gore-Tex® conduit is brought into the field for anastomosis to the pulmonary artery branches.

composite

Gore-Tex graft proximal main pulmonary artery

Figure 18-86. The posterior row of the anastomosis between the homograft patch cuff and the posterior pulmonary artery branch confluence is complete.

composite

Gore-Tex graft

Figure 18-86. The posterior row of the anastomosis between the homograft patch cuff and the posterior pulmonary artery branch confluence is complete.

proximal main pulmonary artery ceph

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Figure 18-87. The anterior part of this anastomosis is complete.

composite conduit to pulmonary artery anastomosis ceph

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Figure 18-87. The anterior part of this anastomosis is complete.

Pictures Plastic Pulimonary Arteries

ascending aorta proximal main pulmonary artery

Figure 18-88. A clamp has been placed on the proximal main pulmonary artery for hemo-stasis and to prevent air from entering the venous catheter during cardiopulmonary bypass. At this point low-flow cerebral perfusion is induced and the arch and ascending aorta opened.

ascending aorta proximal main pulmonary artery

Figure 18-88. A clamp has been placed on the proximal main pulmonary artery for hemo-stasis and to prevent air from entering the venous catheter during cardiopulmonary bypass. At this point low-flow cerebral perfusion is induced and the arch and ascending aorta opened.

Pictures Plastic Aorta

Figure 18-89. The ductus has been divided, the distal arch beyond the left subclavian artery has been divided, the isthmus has been excised, and the descending aorta anastomosed to the posterior distal arch. A homograft patch has been stitched to the posterior ascending aorta and will now be folded over to form the anterior wall of the neoaorta.

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Figure 18-89. The ductus has been divided, the distal arch beyond the left subclavian artery has been divided, the isthmus has been excised, and the descending aorta anastomosed to the posterior distal arch. A homograft patch has been stitched to the posterior ascending aorta and will now be folded over to form the anterior wall of the neoaorta.

descending aorta homograft patch ascending aorta proximal main pulmonary artery composite

Gore-Tex graft

Venous Patch Grafts

Figure 18-90. The homograft patch has been anastomosed anteriorly. Only the homograft to proximal main pulmonary artery anastomosis is necessary to complete the neoaorta. After this, a brief period of circulatory arrest will be used during which the atrial septectomy is performed. Next, full cardiopulmonary bypass will be used for rewarming with arterial inflow through the innominate artery Gore-Tex® graft and venous return through the right atrial appendage cannula.

ho m og raft patch

Figure 18-90. The homograft patch has been anastomosed anteriorly. Only the homograft to proximal main pulmonary artery anastomosis is necessary to complete the neoaorta. After this, a brief period of circulatory arrest will be used during which the atrial septectomy is performed. Next, full cardiopulmonary bypass will be used for rewarming with arterial inflow through the innominate artery Gore-Tex® graft and venous return through the right atrial appendage cannula.

Neo Aorta Hypoplastic Right Heart

Figure 18-91. A small high circular ventriculotomy is made immediately below the neo-aortic valve using care to avoid injury to the leaflets. The proximal composite Gore-Tex® graft is stitched to the ventriculotomy. Here, the graft passes to the right of the neoaorta. Alternatively, it may also be placed to the left of the neoaorta.

neoaorta pulmonary artery branch confluence

Figure 18-91. A small high circular ventriculotomy is made immediately below the neo-aortic valve using care to avoid injury to the leaflets. The proximal composite Gore-Tex® graft is stitched to the ventriculotomy. Here, the graft passes to the right of the neoaorta. Alternatively, it may also be placed to the left of the neoaorta.

right ventricle to composite Gore-Tex graft anastomosis

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