Total Anomalous Pulmonary Venous Connection

Total repair is carried out soon after establishing the diagnosis and medical stabilization of the patient. The procedure can be emergent shortly after birth when there is obstruction of the common pulmonary venous channel (as with a subdiaphragmatic connection), or in the early days of life when there is obstruction at the atrial septal level (supracardiac or intracardiac connection). When there is no obstruction to pulmonary venous return, surgery is required in the early weeks of life because the large left-to-right shunt causes congestive heart failure or failure to thrive with or without pulmonary artery hypertension.

The operation is performed with cardiopulmonary bypass and moderate hypothermia with aortic clamping and cardioplegia. Periods of reduced flow may augment exposure in small infants, but total circulatory arrest with regional cerebral perfusion is almost never needed.

12-1-1. Supracardiac Connection

Figure 12-1. The right lieartirt border is dissected to expose thetie posterior wall of the left atrium.n. The common pulmonary vein in that connects right and left vein^ns is seen adjacent to the back ofof the left atrium.

superior vena cava right pulmonary artery common transverse pulmonary vein ceph

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main pulmonary artery ascending pulmonary venous channel pericardium

Figure 12-2. The ascending pulmonary venous channel is dissected along the left upper heart border. It can be approached by working outside the pericardium or through a short incision in the pericardium that is adjacent to the main pulmonary artery and anterior to the left phrenic nerve. This vein connects the transverse pulmonary venous channel to the innominate vein.

main pulmonary artery ascending pulmonary venous channel pericardium

Figure 12-2. The ascending pulmonary venous channel is dissected along the left upper heart border. It can be approached by working outside the pericardium or through a short incision in the pericardium that is adjacent to the main pulmonary artery and anterior to the left phrenic nerve. This vein connects the transverse pulmonary venous channel to the innominate vein.

Figure 12-3. The enlarged innominate vein is seen. Its large size is caused by cxcess Row in this vessel, because all pulmonary venous drainage passes though it. This vein should liol be compressed during the dissection in preparation for cardiopulmonary bypass because this might occlude pulmonary venous return and compromise cardiac output. A transesophageal ECHO probe is not passed for the same reason.

Figure 12-3. The enlarged innominate vein is seen. Its large size is caused by cxcess Row in this vessel, because all pulmonary venous drainage passes though it. This vein should liol be compressed during the dissection in preparation for cardiopulmonary bypass because this might occlude pulmonary venous return and compromise cardiac output. A transesophageal ECHO probe is not passed for the same reason.

innominate vein ceph

Ci ceph

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atrial septal defect

Figure 12-4. After the cardiopulmonary bypass is established, a transverse mid right atri-otomy is made, directing this incision posteriorly to the mid part of the atrial septal defect (ASD).

atrial septal defect

Figure 12-4. After the cardiopulmonary bypass is established, a transverse mid right atri-otomy is made, directing this incision posteriorly to the mid part of the atrial septal defect (ASD).

posterior left atrial wall orifice of left atrial appendage atrial septum

Figure 12-5. The transverse atriotomy is extended along the posterior mid left atrial wall to the base of the left atrial appendage. An alternative technique is a limited longitudinal right atriotomy with a separate posterior transverse left atriotomy; the anastomosis is performed by retracting the right atrium toward the infant's left or by working intraatrially. The ASD is then closed working though the right atriotomy.

posterior left atrial wall orifice of left atrial appendage

Figure 12-5. The transverse atriotomy is extended along the posterior mid left atrial wall to the base of the left atrial appendage. An alternative technique is a limited longitudinal right atriotomy with a separate posterior transverse left atriotomy; the anastomosis is performed by retracting the right atrium toward the infant's left or by working intraatrially. The ASD is then closed working though the right atriotomy.

atrial septum ceph caud

right upper pulmonary vein right lower pulmonary transverse pulmonary left lower pulmonary

Figure 12-6. The posterior left atriotomy is positioned adjacent to the transverse common pulmonary vein.

right upper pulmonary vein right lower pulmonary transverse pulmonary left lower pulmonary

Figure 12-6. The posterior left atriotomy is positioned adjacent to the transverse common pulmonary vein.

left atrial wall transverse pulmonary

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Figure 12-7. A generous transverse incision is made in the common pulmonary vein. The cephalad rim of the left atrium to pulmonary vein anastomosis is constructed with a continuous suture.

opening into transverse pulmonary vein

Figure 12-8. The caudad rim of the anastomosis is also constructed with a continuous suture. The right end of this anastomosis is at the level of the atrial septum, and it is completed with interrupted sutures to allow for growth of the anastomosis.

opening into transverse pulmonary vein

Figure 12-8. The caudad rim of the anastomosis is also constructed with a continuous suture. The right end of this anastomosis is at the level of the atrial septum, and it is completed with interrupted sutures to allow for growth of the anastomosis.

transverse pulmonary vein to left atrium anastomosis atrial septum transverse pulmonary vein to left atrium anastomosis atrial septum ceph

Figure 12-9. The posterior anastomosis in the left atrial chamber is seen through the ASD.

Figure 12-9. The posterior anastomosis in the left atrial chamber is seen through the ASD.

atrial septal defect patch left atrial line

Figure 12-10. A continuous suture is used to close the ASD with a Dacron® patch. A left atrial line passes beneath the patch and will be exteriorized through the right atri-otomy. If the left atrium were small, it would be enlarged by shifting the atrial septal patch more anteriorly, attaching it to the lateral atrial wall.

atrial septal defect patch

Figure 12-10. A continuous suture is used to close the ASD with a Dacron® patch. A left atrial line passes beneath the patch and will be exteriorized through the right atri-otomy. If the left atrium were small, it would be enlarged by shifting the atrial septal patch more anteriorly, attaching it to the lateral atrial wall.

caud left atrial line

repaired transverse right atrictorny left atrial line

Figure 12-11. The transverse right atriotomy is closed with a continuous suture, and the left atrial line is exteriorized. Alternatively, the left atrial line can be placed in the left atrial appendage, but it should not be placed in the right upper pulmonary vein that is near the anastomosis.

repaired transverse right atrictorny left atrial line

Figure 12-11. The transverse right atriotomy is closed with a continuous suture, and the left atrial line is exteriorized. Alternatively, the left atrial line can be placed in the left atrial appendage, but it should not be placed in the right upper pulmonary vein that is near the anastomosis.

12-1-2. Supracardiac Connection at the Superior Vena Cava to Right Atrial Junction

superior vena cava right pulmonary vein main pulmonary artery ascending aorta

Figure 12-12. The right pulmonary vein can be seen entering the posterior low superior vena cava. A tape is passed around the cava above this area in anticipation of direct superior caval cannulation for cardiopulmonary bypass.

caud superior vena cava right pulmonary vein main pulmonary artery ascending aorta

Figure 12-12. The right pulmonary vein can be seen entering the posterior low superior vena cava. A tape is passed around the cava above this area in anticipation of direct superior caval cannulation for cardiopulmonary bypass.

superior vena cava left pulmonary superior vena cava left pulmonary ceph

Figure 12-13. The left pulmonary vein is seen; it is adjacent to the right vein. The low superior vena cava at the atrial junction is enlarged due to additional flow from the pulmonary veins.

Figure 12-13. The left pulmonary vein is seen; it is adjacent to the right vein. The low superior vena cava at the atrial junction is enlarged due to additional flow from the pulmonary veins.

superior vena cava orifice of anomalous pulmonary veins atrial septal defect

Figure 12-14. After cardiopulmonary bypass is established, a high lateral longitudinal right atriotomy is made. The atriotomy is anterior to the sinoatrial (SA) node to avoid damage to that structure.

The orifice of both pulmonary veins is seen within the lower cava, and they are separated from the ASD by the septum formed by the upper wall of the left atrium and the adjacent wall of the common pulmonary venous channel.

superior vena cava orifice of anomalous pulmonary veins atrial septal defect

Figure 12-14. After cardiopulmonary bypass is established, a high lateral longitudinal right atriotomy is made. The atriotomy is anterior to the sinoatrial (SA) node to avoid damage to that structure.

The orifice of both pulmonary veins is seen within the lower cava, and they are separated from the ASD by the septum formed by the upper wall of the left atrium and the adjacent wall of the common pulmonary venous channel.

pulmonary veins posterior left atrial wall

Figure 12-15. The tissue partition between the pulmonary veins and left atrium is excised to establish a direct pathway for pulmonary venous return.

pulmonary veins posterior left atrial wall

Figure 12-15. The tissue partition between the pulmonary veins and left atrium is excised to establish a direct pathway for pulmonary venous return.

superior vena cava repaired endocardium superior vena cava repaired endocardium caud

Figure 12-16. The endocardium along the posterior wall of the heart is approximated with interrupted sutures; this obliterates the raw surface to guard against clot formation. It also ensures hemostasis in the event a full-thickness defect is inadvertently made at the time of the septal excision. Interrupted stitches are used for this closure to avoid narrowing of the new pathway, which might occur because of the purse string effect of a continuous suture.

Figure 12-16. The endocardium along the posterior wall of the heart is approximated with interrupted sutures; this obliterates the raw surface to guard against clot formation. It also ensures hemostasis in the event a full-thickness defect is inadvertently made at the time of the septal excision. Interrupted stitches are used for this closure to avoid narrowing of the new pathway, which might occur because of the purse string effect of a continuous suture.

ceph

Figure 12-17. A Dacron® patch is stitched over the orifice of the pulmonary veins, the new pathway, and the ASD. The area behind the patch is inspected to be sure the new pathway is unobstructed.

Figure 12-17. A Dacron® patch is stitched over the orifice of the pulmonary veins, the new pathway, and the ASD. The area behind the patch is inspected to be sure the new pathway is unobstructed.

ceph caud

Dacron patch

Figure 12-18. Pulmonary venous return is now diverted by the patch to the left atrium.

Dacron patch

Figure 12-18. Pulmonary venous return is now diverted by the patch to the left atrium.

12-1-3. Intracardiac Connection to the Right Atrium ceph

right pulmonary veins

Figure 12-19. After the cardiopulmonary bypass is established, with aortic clamping, cardioplegia, and profound local cardiac cooling, a lateral longitudinal right atriotomy is made. Right upper and right lower pulmonary veins enter the right atrium separately. The left pulmonary veins form a common channel, which drains to the right atrium posterior to the ASD.

atrial septal defect left pulmonary veins right pulmonary veins

Figure 12-19. After the cardiopulmonary bypass is established, with aortic clamping, cardioplegia, and profound local cardiac cooling, a lateral longitudinal right atriotomy is made. Right upper and right lower pulmonary veins enter the right atrium separately. The left pulmonary veins form a common channel, which drains to the right atrium posterior to the ASD.

Figure 12-20. The coronary sinus is a separate orifice and is located between the left pulmonary vein orifice and the tricuspid valve.
tricuspid valve

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Figure 12-21. Tissue along the lower margin of the ASD and between the left pulmonary veins and left atrium is incised to establish an unobstructed pathway for pulmonary venous flow after the repair. The endocardium is closed with interrupted sutures.

incised septum between left pulmonary veins and left atrium

Figure 12-21. Tissue along the lower margin of the ASD and between the left pulmonary veins and left atrium is incised to establish an unobstructed pathway for pulmonary venous flow after the repair. The endocardium is closed with interrupted sutures.

Figure 12-22. A large nonobstructing Dacron® patch is stitched over the ASD and all pulmonary veins. The atrio-ventricular (AV) node is caudad to the coronary sinus, which is remote from the repair.

12-1-4. Intracardiac Connection to the Coronary Sinus

coronary sinus left pulmonary veins right pulmonary veins

Figure 12-23. After cardiopulmonary bypass is established, with aortic clamping, cardioplegia, and profound local cardiac cooling, a longitudinal right atriotomy is made. The coronary sinus is large, and the orifices of left and right pulmonary veins, respectively, are seen within the sinus.

coronary sinus left pulmonary veins right pulmonary veins

Figure 12-23. After cardiopulmonary bypass is established, with aortic clamping, cardioplegia, and profound local cardiac cooling, a longitudinal right atriotomy is made. The coronary sinus is large, and the orifices of left and right pulmonary veins, respectively, are seen within the sinus.

coronary sinus

ceph caud right pulmonary veins

Figure 12-24. The restrictive ASD is cephalad to the coronary sinus.

right pulmonary veins

Figure 12-24. The restrictive ASD is cephalad to the coronary sinus.

right angle clamp tip in septum

Figure 12-25. A right-angle clamp is passed through the ASD to expose the septum that separates the roof of the coronary sinus and the left atrium.

Figure 12-25. A right-angle clamp is passed through the ASD to expose the septum that separates the roof of the coronary sinus and the left atrium.

right angle clamp tip in septum

base of excised septum caud

Figure 12-26. The septum that separates the coronary sinus from the left atrium is excised, resulting in a large communication between the coronary sinus and the left atrium.

base of excised septum caud

Figure 12-26. The septum that separates the coronary sinus from the left atrium is excised, resulting in a large communication between the coronary sinus and the left atrium.

Dacrori patch

Figure 12-27. A Dacron® patch is stitched over the coronary sinus and adjacent ASD. Along the caudad margin of the coronary sinus, stitches are placed deep in the sinus, remote from the AV node and His' bundle. These sutures are interrupted and will be tied after removal of the aortic cross-clamp when sinus rhythm is observed. If sutures are encroaching on conductive tissue, AV dissociation will occur as the offending stitch is tied. In that event it is replaced. A continuous suture is used along the posterior and cephalad margins of the patch.

Dacrori patch

Figure 12-27. A Dacron® patch is stitched over the coronary sinus and adjacent ASD. Along the caudad margin of the coronary sinus, stitches are placed deep in the sinus, remote from the AV node and His' bundle. These sutures are interrupted and will be tied after removal of the aortic cross-clamp when sinus rhythm is observed. If sutures are encroaching on conductive tissue, AV dissociation will occur as the offending stitch is tied. In that event it is replaced. A continuous suture is used along the posterior and cephalad margins of the patch.

12-1-5. Subdiaphragmatic Connection ceph

caud

Figure 12-28. The ductus arteriosus is dissected at the distal main pulmonary artery. The anatomy may be unclear because of the large size of the ductus, and it is identified by visualizing the proximal right and left pulmonary arteries, respectively. The ductal structure is located between those two vessels. The ductus is closed with a ligature or a metal clip. In the presence of preoperative pulmonary artery hypertension, an ECHO study may not show ductal flow when it is patent. This structure is, therefore, surgically closed in all cases of total anomalous pulmonary venous connection.

ascending aorta

Figure 12-28. The ductus arteriosus is dissected at the distal main pulmonary artery. The anatomy may be unclear because of the large size of the ductus, and it is identified by visualizing the proximal right and left pulmonary arteries, respectively. The ductal structure is located between those two vessels. The ductus is closed with a ligature or a metal clip. In the presence of preoperative pulmonary artery hypertension, an ECHO study may not show ductal flow when it is patent. This structure is, therefore, surgically closed in all cases of total anomalous pulmonary venous connection.

patent ductus arteriosus left pulmonary artery main pulmonary artery ceph

right upper pulmonary vein left pulmonary right lower pulmonary common pulmonary vein

Figure 12-29. The left atrium along the right heart border is dissected. The right and left pulmonary veins form a confluence at the common pulmonary vein, which then passes caudad to below the diaphragm.

right upper pulmonary vein left pulmonary right lower pulmonary common pulmonary vein

Figure 12-29. The left atrium along the right heart border is dissected. The right and left pulmonary veins form a confluence at the common pulmonary vein, which then passes caudad to below the diaphragm.

left atriotomy opening in common pulmonary vein

Figure 12-30. Repair is carried out with cardiopulmonary bypass and moderate hypothermia with intermittent low flow when needed. An opening is made in the common pulmonary vein. An adjacent left atriotomy is made.

left atriotomy opening in common pulmonary vein

Figure 12-30. Repair is carried out with cardiopulmonary bypass and moderate hypothermia with intermittent low flow when needed. An opening is made in the common pulmonary vein. An adjacent left atriotomy is made.

left atrium common pulmonary vein

Figure 12-31. A side-to-side anastomosis between the pulmonary vein and left atrium is constructed, using a continuous suture along the posterior rim of the anastomosis.

left atrium common pulmonary vein

Figure 12-31. A side-to-side anastomosis between the pulmonary vein and left atrium is constructed, using a continuous suture along the posterior rim of the anastomosis.

right atriotomy anastomosis

Figure 12-32. The more anterior margin of the anastomosis is constructed with a continuous suture, in part, and with interrupted sutures that may allow for growth of the anastomosis. An adjacent right atriotomy is made through which to visualize the ASD.

right atriotomy anastomosis caud

Figure 12-32. The more anterior margin of the anastomosis is constructed with a continuous suture, in part, and with interrupted sutures that may allow for growth of the anastomosis. An adjacent right atriotomy is made through which to visualize the ASD.

suture closure of atrial septal defect

Figure 12-33. Here, the ASD is closed primarily. If the left atrium were restrictive, the atrial septum would be moved to a more anterior position to enlarge the left atrium; this can be carried out by using a patch to close the septal defect. The lower common pulmonary vein below the anastomosis is ligated, and a left atrial line is placed through the left atrial appendage remote from the anastomosis.

suture closure of atrial septal defect

Figure 12-33. Here, the ASD is closed primarily. If the left atrium were restrictive, the atrial septum would be moved to a more anterior position to enlarge the left atrium; this can be carried out by using a patch to close the septal defect. The lower common pulmonary vein below the anastomosis is ligated, and a left atrial line is placed through the left atrial appendage remote from the anastomosis.

Figure 12-34. After cardiopulmonary bypass is established, with aortic clamping, cardioplegic arrest, and profound local cardiac cooling, a right atriotomy is made. Normal right pulmonary veins are seen through the ASD.

right pulmonary veins atrial septal detect

Figure 12-34. After cardiopulmonary bypass is established, with aortic clamping, cardioplegic arrest, and profound local cardiac cooling, a right atriotomy is made. Normal right pulmonary veins are seen through the ASD.

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