Palliation and Repair of Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Infants born with this rare syndrome may develop aneurysmal dilatation of the main pulmonary artery and/or its branches; this may cause tracheal and bronchial obstruction resulting in severe respiratory distress. In the rare case when totally corrective surgery cannot be undertaken, airway obstruction may be relieved by translocating the offending right or left pulmonary artery from the posterior to the anterior mediastinum. This operation is largely of historic interest but emphasizes the effectiveness of debulking the mediastinum in successfully treating this anomaly. In symptomatic infants, the ideal operation is total repair including placement of a pulmonary valve with tailoring of the aneurysmal pulmonary arteries regardless of the size or age of the patient.

Figure 9-75. In this small infant with right bronchial obstruction, palliation is performed by working through a right thoracotomy. After the pericardium is opened, the enlarged right pulmonary artery is seen.

Figure 9-75. In this small infant with right bronchial obstruction, palliation is performed by working through a right thoracotomy. After the pericardium is opened, the enlarged right pulmonary artery is seen.

Right Pulmonary Artery

ascending aorta aneurysmal right pulmonary artery ascending aorta aneurysmal right pulmonary artery

Mouse Ascending Aorta

ascending aorta right pulmonary artery superior vena cava

Figure 9-76. The right pulmonary artery is dissected from its origin and silk sutures for snaring are passed around the branches.

ascending aorta right pulmonary artery superior vena cava

Figure 9-76. The right pulmonary artery is dissected from its origin and silk sutures for snaring are passed around the branches.

Pulmonary Arteries Superior Vena Cava

ascending aorta proximal right pulmonary artery superior vena cava distal right pulmonary artery

Figure 9-77. The proximal right pulmonary artery near the main pulmonary artery is divided and closed. The distal right pulmonary artery is shifted lateral to the superior vena cava.

ascending aorta proximal right pulmonary artery superior vena cava

Figure 9-77. The proximal right pulmonary artery near the main pulmonary artery is divided and closed. The distal right pulmonary artery is shifted lateral to the superior vena cava.

distal right pulmonary artery

Superior Vena Cava Syndrome
ascending aorta
Superior Vena Cava Syndrome

right ventricle ascending aorta main pulmonary artery

Figure 9-79. In another neonate, totally corrective surgery is undertaken. The enlarged main pulmonary artery and dilated right ventricle are seen.

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»- caud superior vena cava distal right pulmonary artery

Figure 9-78. Using a side-biting clamp on the anterior main pulmonary artery, a synthetic tubular graft is anastomosed to this vessel. Right pulmonary arterial vascular continuity is reconstructed by attaching this graft to the distal pulmonary artery. The graft passes in front of the ascending aorta and superior vena cava, removing the bulk of the aneurysmal right pulmonary artery from the posterior mediastinum; this removes the extrinsic pressure on the right bronchus and relieves the bronchial obstruction.

ascending aorta main pulmonary artery

Figure 9-79. In another neonate, totally corrective surgery is undertaken. The enlarged main pulmonary artery and dilated right ventricle are seen.

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Absent Pulmonary Valve Repair Procedure

ascending aorta main pulmonary artery right pulmonary artery

Figure 9-80. By retracting the ascending aorta the huge right pulmonary artery is seen. Its diameter is at least twice that of the ascending aorta.

ascending aorta main pulmonary artery right pulmonary artery

Figure 9-80. By retracting the ascending aorta the huge right pulmonary artery is seen. Its diameter is at least twice that of the ascending aorta.

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Absent Pulmonary Valve Syndrome

pulmonary valve annulus

Figure 9-81. The child is placed on cardiopulmonary bypass, and the proximal main pulmonary artery opened. The pulmonary valve annulus is seen; however, only rudimentary valve leaflets are present.

pulmonary valve annulus

Figure 9-81. The child is placed on cardiopulmonary bypass, and the proximal main pulmonary artery opened. The pulmonary valve annulus is seen; however, only rudimentary valve leaflets are present.

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Absent Pulmonary Valve

removed anterior segment of right and left pulmonary arteries ascending aorta

Figure 9-82. A large segment of the anterior wall of the right and left pulmonary arteries is excised.

removed anterior segment of right and left pulmonary arteries ascending aorta caud

Figure 9-82. A large segment of the anterior wall of the right and left pulmonary arteries is excised.

Aorta Proximal Pulm Artery

area of anterior resection of left pulmonary artery ascending aorta area of anterior resection of right pulmonary artery

Figure 9-83. After right and left pulmonary arteries are tailored, there is less bulk in the mediastinum; this relieves tracheal and bronchial obstruction.

area of anterior resection of left pulmonary artery ascending aorta caud area of anterior resection of right pulmonary artery

Figure 9-83. After right and left pulmonary arteries are tailored, there is less bulk in the mediastinum; this relieves tracheal and bronchial obstruction.

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Absent Pulmonary Artery Unilateral

anterior resection of left pulmonary artery anterior resection of main pulmonary artery

Figure 9-84. Part of the anterior main pulmonary artery is resected, as well as the anterior left pulmonary artery.

anterior resection of left pulmonary artery anterior resection of main pulmonary artery

Figure 9-84. Part of the anterior main pulmonary artery is resected, as well as the anterior left pulmonary artery.

Absent Left Pulmonary Artery Cmr

right pulmonary artery

Figure 9-85. The anterior pulmonary arteries are closed with a continuous suture.

right pulmonary artery

Figure 9-85. The anterior pulmonary arteries are closed with a continuous suture.

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Pulmonary Posterior Ascending Artery

ascending aorta left pulmonary artery right pulmonary artery posterior suture

Figure 9-86. In another child, the pulmonary arteries are tailored posteriorly. The posterior aspect of the aneurysmal vessels is resected and then closed primarily within the pulmonary arteries. This may be advantageous because aneurysmal tissue that is in direct contact with the trachea and bronchi is removed and this may result in improved relief of tracheal bronchial obstruction.

ascending aorta left pulmonary artery right pulmonary artery posterior suture

Figure 9-86. In another child, the pulmonary arteries are tailored posteriorly. The posterior aspect of the aneurysmal vessels is resected and then closed primarily within the pulmonary arteries. This may be advantageous because aneurysmal tissue that is in direct contact with the trachea and bronchi is removed and this may result in improved relief of tracheal bronchial obstruction.

Tricuspid Aneurysmal Tissue

pulmonary valve annulus ventricular septal defect tricuspid valve leaflet

Figure 9-87. The subcristal ventricular septal defect is seen, and multiple interrupted sutures are placed around its rim.

pulmonary valve annulus ventricular septal defect tricuspid valve leaflet

Figure 9-87. The subcristal ventricular septal defect is seen, and multiple interrupted sutures are placed around its rim.

Pulmonary Artery ReplacementMitral Valve Leaflets

anterior mitral valve leaflet of homograft aortic homograft

Figure 9-89. An aortic valve homograft with attached ascending aorta is used for pulmonary valve replacement. The homograft anterior mitral valve leaflet will be used in the repair. An aortic homograft is used when residual pulmonary artery pressure elevation is anticipated.

anterior mitral valve leaflet of homograft aortic homograft

Figure 9-89. An aortic valve homograft with attached ascending aorta is used for pulmonary valve replacement. The homograft anterior mitral valve leaflet will be used in the repair. An aortic homograft is used when residual pulmonary artery pressure elevation is anticipated.

Homograft Vascular Graft

native pulmonary artery homograft anterior mitral valve leaflet of homograft

Figure 9-90. The homograft is tailored, and the posterior graft annulus is stitched to the native pulmonary valve annulus. The upper end of the homograft is stitched to the inner wall of the main pulmonary artery posteriorly (proximal to the native branch pulmonary artery orifices) and the rim of the native pulmonary artery anteriorly.

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Figure 9-90. The homograft is tailored, and the posterior graft annulus is stitched to the native pulmonary valve annulus. The upper end of the homograft is stitched to the inner wall of the main pulmonary artery posteriorly (proximal to the native branch pulmonary artery orifices) and the rim of the native pulmonary artery anteriorly.

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anterior mitral valve leaflet of homograft

Figure 9-91. The homograft mitral valve leaflet is stitched to the right ventriculotomy and forms a hood inferior to the homograft valve; this allows the valve to remain in an anatomic position and avoids valve regurgitation caused by annular distortion.

Pulmonary Valve Regurgitation

pulmonary valve homograft

Figure 9-92. In another child, a homograft pulmonary valve with an attached main pulmonary artery is used for pulmonary valve replacement.

pulmonary valve homograft

Figure 9-92. In another child, a homograft pulmonary valve with an attached main pulmonary artery is used for pulmonary valve replacement.

Palliation

posterior muscle ledge native pulmonary valve annulus

Figure 9-93. The repair is begun by attaching the posterior subannular homograft muscle bar to the region of the native valve annulus with a continuous suture.

posterior muscle ledge native pulmonary valve annulus

Figure 9-93. The repair is begun by attaching the posterior subannular homograft muscle bar to the region of the native valve annulus with a continuous suture.

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Figure 9-94. Insertion of the homograft is similar to insertion of an aortic valve homo-graft. The anterior muscle ledge of the homograft is stitched to the ventriculotomy, which forms the hood over the lower end of the conduit.

Blood Pressure Health

Blood Pressure Health

Your heart pumps blood throughout your body using a network of tubing called arteries and capillaries which return the blood back to your heart via your veins. Blood pressure is the force of the blood pushing against the walls of your arteries as your heart beats.Learn more...

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