Volume 12, Issue 2 , Pages 110-140, Summer 2007
Staged Repair of Tetralogy of Fallot with Pulmonary Atresia and Major Aortopulmonary Collateral Arteries
Article Outline
Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) is a rare but important anatomical configuration. Unifocalization is one surgical procedure that creates an adequate pulmonary circulation in the setting of MAPCAs. In this text, the variable anatomy of MAPCAs and the correspondingly variable unifocalization procedures are described in seven case presentations.
Case 1 is a 5-year-old boy with tetralogy of Fallot, pulmonary atresia, MAPCAs, and a right aortic arch with aberrant left subclavian artery. Two MAPCAs originate from the right descending aorta (Fig 1A). MAPCA L1 connects to the left pulmonary artery, which has dual supply. MAPCA L2 does not have a connection with the pulmonary artery, which is called an “arborization anomaly.” The chest is opened through a left posterolateral thoracotomy (Fig 1B).
Figure 1.
A: MAPCA L1 has dual supply, but L2 has an arborization anomaly. B: The aberrant left subclavian artery rounds the left bronchus anteriorly. MAPCA L1 and L2 round the left bronchus posteriorly. PLSVC = persistent left superior vena cava. C: MAPCA L1 is ligated. A modified Blalock–Taussig shunt is established between the aberrant left subclavian artery and the left pulmonary artery with a 5-mm Gore-Tex graft. D: MAPCA L2 is anastomosed to the left pulmonary artery. E: Left unifocalization is completed. LPA = left pulmonary artery.
MAPCA L1 is ligated (Fig 1C), and a left modified Blalock–Taussig shunt is established between the aberrant left subclavian artery and the left pulmonary artery. MAPCA L2 is unifocalized by anastomosis to the left pulmonary artery (Fig 1D). Polytetrafluoroethylene (PTFE) tape is wrapped around the modified Blalock–Taussig shunt (Fig 1E). A Gore-Tex tape (Gore-Tex, Flagstaff, Arizona) is passed beneath the persistent left superior vena cava and phrenic nerve and anchored to the posterior wall of the sternum.
Case 2 is 13-year-old boy with tetralogy of Fallot, pulmonary atresia, and MAPCAs. He has undergone a right modified Blalock-Taussig shunt (Fig 2A). Two MAPCAs originate from the left descending aorta. Both have arborization anomaly. The central pulmonary arteries do not have a main pulmonary trunk (Fig 2A). The chest is opened through the left fourth intercostal space (Fig 2B). The left pulmonary artery and two MAPCAs are well mobilized into the intrapulmonary space behind the hilus. MAPCA L1 courses behind the vagus nerve; the inferior branch of MAPCA L1 courses between the bifurcation of the branches of the vagus nerve. Then MAPCA L1 is divided from the aorta, and the inferior branch of MAPCA L1 is divided from the main trunk of MAPCA L1 to free the vagus nerve (Fig 2C). The inferior branch of the MAPCA L1 is divided and passed beneath the vagus nerve. Then it is again anastomosed to the main trunk of MAPCA L1 (Fig 2C). MAPCA L2 is anasotomosed to the inferior branch of L1 (Fig 2D). The base of L1 is anastomosed to the left pulmonary artery (Fig 2E). A left modified Blalock–Taussig shunt is established by a 6-mm Gore-Tex graft between the left subclavian artery and left pulmonary artery. Left unifocalization is thus accomplished.
Figure 2.
A: After right modified Blalock–Taussig shunt. A main pulmonary trunk is absent. Two MAPCAs originate from the left descending aorta. B: Two MAPCAs originate from the descending aorta. MAPCA L1 courses beneath the vagus nerve. C: Anastomosis of the inferior branch of MAPCA L1. D: Anastomosis of MAPCA L2 to L1. E: Left unifocalization is accomplished. LPA = left pulmonary artery.
Case 3 is a 4-year-old boy with tetralogy of Fallot, pulmonary atresia, and MAPCAs. The left chest is opened through the fourth intercostal space (Fig 3A). Two MAPCAs originate from the descending aorta. They are well mobilized around the hilus. MAPCA L1 has dual supply from the descending aorta and the left pulmonary artery. The attenuated segment between MAPCA L1 and the left pulmonary artery is widely opened. MAPCA L2 is divided and opened along its bifurcation (Fig 3B). The opened stump of MAPCA L2 is anasotomosed to the long segment of MAPCA L1 (Fig 3C). A 6-mm PTFE graft originating on the left subclavian artery is then anastomosed between the suture line of MAPCA L2 and the left pulmonary artery (Fig 3D). A stump of MAPCA L1 is adopted for patch enlargement of the left pulmonary artery (Fig 3E). Gore-Tex tape surrounding the shunt is anchored to the posterior wall of the sternum. Left unifocalization is thus accomplished.
Figure 3.
A: A narrow segment connects MAPCA L1 and the left pulmonary artery. B: The narrow segment and MAPCA L2 are widely opened. C: MAPCA L2 is anastomosed to the narrow segment. D: The left modified Blalock–Taussig shunt is established. E: A piece of MAPCA L1 is adopted for enlargement of the left pulmonary artery. LPA = left pulmonary artery; LSCA = left subclavian artery.
Case 4 is a 7-year-old girl with tetralogy of Fallot, pulmonary atresia, MAPCAs, and right aortic arch. The chest is opened through the right third intercostal space (Fig 4A). MAPCA R1 has a long connecting segment to the right pulmonary artery. This long segment courses between the right pulmonary artery and the right bronchus. MAPCA R1 is then anastomosed to the right pulmonary artery (Fig 4B). MAPCA R2 is anastomosed to the long connecting segment of MAPCA R1 in an end-to-end fashion. Azygos vein is removed and adopted for patch enlargement of the right pulmonary artery. A 6-mm Gore-Tex graft is anastomosed to the azygos patch as a modified Blalock–Taussig shunt (Fig 4B).
Figure 4.
A: The long segment connecting MAPCA R1 to the right pulmonary artery courses between the right pulmonary artery and the right bronchus. B: MAPCA R2 is anastomosed to the long connecting segment in end-to-end fashion. A modified Blalock–Taussig shunt is anastomosed to the azygos vein patch. Asc. Ao. = ascending aorta; RPA = right pulmonary artery.
Case 5 is a 1-year-old girl with tetralogy of Fallot, pulmonary atresia, and many MAPCAs. MAPCA R1 originates from the left side of descending aorta and connects to the central pulmonary arteries as a patent ductus arteriosus. Two right-sided MAPCAs (R2 and R3) and three left-sided MAPCAs (L1, L2, L3) have arborization anomaly and hence need unifocalization (Fig 5A). The right chest is opened through the right fifth intercostal space (Fig 5B). The azygos vein is removed. MAPCA R1 is divided and enlarged by an azygos vein patch (Fig 5C). This enlarged R1 is anastomosed to MAPCA R2. MAPCA R3 is also anastomosed to R2 (Fig 5C). A modified Blalock–Taussig shunt is then established between the right subclavian artery and MAPCA R2. Gore-Tex tape surrounding the shunt is anchored behind the sternum (Fig 5D).
Figure 5.
A: Many MAPCAs on both sides. MAPCA R1 supplies the central pulmonary arteries. B: The right chest is opened. The azygos vein is removed. C: MAPCA R1 is enlarged by an azygos vein patch and anastomosed to R2. R3 is anastomosed to R2 as well. D: Right-sided unifocalization is accomplished. E: Two MAPCAs, L1 and L2, are near to the left bronchus, but L3 is beneath the left pulmonary vein. F: Through the seventh intercostal space, MAPCA L3 is divided and anastomosed to MAPCA L1. G: L2 is widely spatulated. Inset, the upward arrow on the superior branch of the LPA is the incision for unifocalization; the downward arrow on the LPA is the incision for the subsequent Rastelli operation. H: L2 is sutured along the inferior margin of the incision on the superior branch of the left pulmonary artery. I: The spatulated flap of L2 is sutured along the superior margin of the incision. J: MAPCA L1 is sutured to the anastomosis of L2 and the superior branch of the LPA. K: Left-sided unifocalization is accomplished. Gore-Tex tape around the modified Blalock–Taussig shunt is anchored over the phrenic nerve. L: Through a median sternotomy incision, a longitudinal incision is placed across the right ventricular outflow tract, blind pulmonary trunk, and left pulmonary artery. M: The incision is extended into the main basal branch of the left pulmonary artery. N: The proximal end of the pulmonary trunk is sutured to the right ventricular outflow tract. O: The ventricular septal defect is closed by interrupted sutures and a patch. Inferiorly, two sutures are placed in the membranous flap. P: The left pulmonary artery is enlarged by an autologous pericardial patch. Q: Total correction is accomplished after bilateral unifocalizations.
The left chest is opened through the fourth and seventh intercostal spaces. There are three MAPCAs, L1, L2, and L3 (Fig 5E). Through the seventh intercostal space, MAPCA L3 is divided and anastomosed to MAPCA L1 in an end-to-side fashion (Fig 5F). Through the fourth intercostal space, a longitudinal incision is placed on the superior branch of the left pulmonary artery beyond its bifurcation (Fig 5G, inset). MAPCA L2 is widely spatulated (Fig 5G). MAPCA L2 is anastomosed to the superior branch of the left pulmonary artery (Fig 5H). The long flap of MAPCA L2 is turned and sutured along the counter side to enlarge the anastomosis (Fig 5I). Then MAPCA L1 is anastomosed to the distal end of the suture of L2 and the superior branch of the LPA (Fig 5J). Finally, a 5-mm Gore-Tex graft is anastomosed to the newly created orifice as a left modified Blalock–Taussig shunt. Left-side unifocalization is thus accomplished (Fig 5K).
Through a median sternotomy incision, the Gore-Tex tapes encircling both the right and the left modified Blalock–Taussig shunts are exposed (Fig 5L). Under cardiopulmonary bypass, both Blalock–Taussig shunts are ligated. The blind small pulmonary trunk is incised. The incision is extended proximally into the right ventricular outflow tract and distally along the main basal branch of the left pulmonary artery (Fig 5L and M). The proximal end of the pulmonary trunk is anastomosed to the distal end of the right ventricular outflow tract (Fig 5N). The ventricular septal defect is patched, utilizing the membranous flap along the inferior margin of the defect (Fig 5O). The left pulmonary artery is enlarged using autologous pericardium (Fig 5P). A Gore-Tex bicuspid patch is adopted for the right ventricular outflow reconstruction (Fig 5Q).
Case 6 is a 23 year-old man with tetralogy of Fallot, pulmonary atresia, nonconfluent pulmonary arteries, and MAPCAs. Left-sided unifocalization was already performed by a xenopericardial conduit. The right chest is opened through the fourth intercostal space (Fig 6A). MAPCA R1 courses behind the right bronchus. Although a small connection exists between the right pulmonary artery and MAPCA R1, unifocalization is required for MAPCA R1. R1 is anastomosed to the right pulmonary artery and R2 is anastomosed to the distal part of R1 (Fig 6B). Then the azygos vein is interposed between the right pulmonary artery and the anastomotic site of R1 and R2 (Fig 6B). A large xenopericardial conduit is anastomosed to the widely created orifice at the site of anastomosis between the right pulmonary artery and the azygos vein (Fig 6C). A modified Blalock–Taussig shunt is eventually established by a 6-mm Gore-Tex graft between the right subclavian artery and the proximal end of the xenopericardial conduit (Fig 6C).
Figure 6.
A: The right chest is opened. MAPCA R1 has a small connection with the right pulmonary artery. B: Unifocalization utilizing the azygos vein. C: Right-sided unifocalization is accomplished by a xenopericardial conduit as a central pulmonary artery. D: The ventricular septal defect is closed by interrupted sutures and a patch. E: A Rastelli operation is accomplished after bilateral unifocalizations for nonconfluent pulmonary arteries.
Two months later, a Rastelli procedure is performed through a median sternotomy incision. Under cardiopulmonary bypass, the ventricular septal defect is closed by interrupted sutures and a patch (Fig 6D). The bilateral xenopericardial conduits are connected by a third xenopericardial conduit, resulting in confluent pulmonary arteries (Fig 6E). A fourth xenopericardial conduit with a Gore-Tex tricusp (homograft is preferable) is adopted for use as an extracardiac conduit from the right ventricle to the reconstructed central pulmonary arteries (Fig 6E).
Case 7 is a 4-month-old girl with tetralogy of Fallot, pulmonary atresia, nonconfluent pulmonary arteries, MAPCAs, and a right aortic arch. The right chest is opened through the fourth intercostal space. Three MAPCAs, R1, R2, and L1, originate from the descending aorta (Fig 7A). MAPCAs R1 and R2 go into the right lung, and L1 goes into the left lung. R1 and R2 are divided and sutured together over half of their circumferences (Fig 7B). A xenopericardial conduit is anastomosed to the junction of R1 and R2 (Fig 7C). A 5-mm Gore-Tex graft is interposed between the right subclavian artery and the medial end of the xenopericardial conduit.
Figure 7.
A: View is through a right thoracotomy. Three MAPCAs originate from the right descending aorta. B: Half circumferences of R1 and R2 are sutured together. C: A xenopericardial conduit is anastomosed to the R1-R2 junction. D: View is through a left thoracotomy. A single MAPCA, L1, comes from the right between the bronchus and esophagus. E: L1 is divided and spatulated. F: Left-sided unifocalization is accomplished. G: Large holes are created in the bilateral xenopericardial conduits. H: The third xenopericardial conduit is interposed between the bilateral xenopericardial conduits. I: A Gore-Tex tricusp xenopericardial conduit is anastomosed to the newly created central pulmonary arteries. J: The ventricular septal defect is closed by interrupted sutures. K: A Rastelli procedure is accomplished after bilateral unifocalizations and creation of confluent central pulmonary arteries.
The left chest is opened through the third intercostal space. MAPCA L1 originates from the right descending aorta and passes into the left lung between the left bronchus and esophagus (Fig 7D). MAPCA L1 is divided and spatulated (Fig 7E). A xenopericardial conduit is anastomosed to the spatulated MAPCA L1 (Fig 7F). A 4-mm Gore-Tex graft is interposed between the left subclavian artery and the xenopericardial conduit. Gore-Tex tape is anchored to the anterior chest wall (Fig 7F).
Through a median sternotomy incision and under cardiopulmonary bypass, a large hole is created on the tops of the xenopericardial conduits used for the bilateral unifocalizations (Fig 7G). A third xenopericardial conduit is interposed between the bilateral xenopericardial conduits, creating confluent central pulmonary arteries (Fig 7H). A fourth xenopericardial conduit with a Gore-Tex tricusp is anastomosed to the confluent central pulmonary arteries (Fig 7I). The ventricular septal defect is closed by interrupted sutures (Fig 7J). A Rastelli procedure is thus accomplished after bilateral unifocalizations (Fig 7L).
PII: S1522-2942(07)00059-1
doi:10.1053/j.optechstcvs.2007.05.001
© 2007 Elsevier Inc. All rights reserved.
Volume 12, Issue 2 , Pages 110-140, Summer 2007
