12 Pulmonary Venous Anomalies
12-1. Total Anomalous Pulmonary Venous Connection
Total repair is carried out soon after establishing the diagnosis and medical sta- bilization of the patient. The procedure can be emergent shortly after birth when there is obstruction of the common pulmonary venous channel (as with a subdi- aphragmatic 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 cere- bral perfusion is almost never needed.
12-1-1. Supracardiac Connection
FIGURE 12-1. The right heart border is dissected to expose the posterior wall of the left atrium.
The common pulmonary vein that connects right and left veins is seen adjacent to the back of the left atrium.
214
ceph R « - - • L
caud
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 excess flow in this vessel, because all pul- monary venous drainage passes though it. This vein should not be compressed during the dis- section in preparation for car- diopulmonary bypass because this might occlude pulmonary venous return and compromise cardiac output. A transeso- phageal E C H O probe is not passed for the same reason.
atrial septal defect
caud
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 per- formed by retracting the right atrium toward the infant's left or by working intraatrially.
The ASD is then closed working though the right atriotomy.
ceph
R « - - • L
caud
pulmonary vein right lower pulmonary vein
transverse pulmonary vein
left lower pulmonary vein
FIGURE 12-6. The posterior left atriotomy is positioned adjacent to the transverse common pulmonary vein.
R ^
left atrial wall
transverse pulmonary vein
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 con- tinuous suture.
ceph
R<- - • L caud
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 com- pleted with interrupted sutures to allow for growth of the anastomosis.
ceph
R««- - • L
caud
transverse pulmonary vein to left atrium anastomosis
atrial septum
FIGURE 12-9. The posterior anastomosis in the left atrial chamber is seen through the ASD.
ceph R < - - • L
caud
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.
ceph R < - - • L
caud
repaired transverse right atriotomy
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
ceph
R < - - • L
caud
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 supe- rior caval cannulation for cardiopulmonary bypass.
ceph
R < - - • L
caud
left pulmonary
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 pul- monary veins.
ceph R « - - • L
caud
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 sepa- rated 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.
ceph R < - - • L
caud
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
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 nar- rowing of the new pathway, which might occur because of the purse string effect of a con- tinuous suture.
ceph R < - - • L
caud
Dacron patch
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 R < - - • L
caud
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 R««- - • L
caud
atrial septal defect left pulmonary veins
right pulmonary veins
FIGURE
12-19. After the cardiopulmonary bypass is established, with aortic clamping, car-
dioplegia, 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.
ceph R « - - > L
caud
left pulmonary
FIGURE
12-20. The coronary sinus is a separate orifice and is located between the left pul- monary vein orifice and the tricuspid valve.
ceph R < - - • L
caud
tricuspid valve
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.
ceph R < - - • L
caud
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
ceph R « - - • L
caud
coronary sinus
left pulmonary
right pulmonary veins
FIGURE
12-23. After cardiopulmonary bypass is established, with aortic clamping, cardio-
plegia, 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.
ceph R < - - • L
caud
atrial septal defect
coronary sinus
right pulmonary
FIGURE 12-24. The restrictive ASD is cephalad to the coronary sinus.
ceph R < - - • L
caud
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.
ceph R < - - • L
caud
base of excised septum
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.
Dacron 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 R < - - > L
t caud
patent ductus arteriosus left pulmonary artery main pulmonary artery
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 visu- alizing 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.
R<-
right upper pulmonary vein
left pulmonary vein
right lower pulmonary vein common pulmonary
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.
ceph R < - - • L
caud
left atrlotomy
opening in common pulmonary vein
FIGURE
12-30. Repair is carried out with cardiopulmonary bypass and moderate hypother- mia with intermittent low flow when needed. An opening is made in the common pul- monary vein. An adjacent left atriotomy is made.
ceph R<r- - • L
caud
left atrium
common pulmonary
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.
ceph R < - - • L
caud
right atriotomy
anastomosis
FIGURE
12-32. The more anterior margin of the anastomosis is constructed with a con- tinuous suture, in part, and with interrupted sutures that may allow for growth of the anas- tomosis. An adjacent right atriotomy is made through which to visualize the ASD.
ceph R < - - * L
caud
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 pul-
monary vein below the anastomosis is ligated, and a left atrial line is placed through the
left atrial appendage remote from the anastomosis.
This rare anomaly occurs at the entrance of pulmonary veins to the left atrium.
Because of the severe consequences of pulmonary venous hypertension, includ- ing congestive heart failure and/or pulmonary vascular obstructive disease, surgery to repair this lesion is indicated immediately after discovery, regardless of the age of the patient. Direct repair of the pulmonary vein is performed in the presence of mild-to-moderate stenosis. An alternative repair is sutureless with stitches placed in adjacent pericardium and in the pulmonary vein wall remote from the venotomy.
FIGURE 12-34. After cardiopulmo-
nary 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
atrial septal defect
> L
FIGURE 12-35. The stenotic orifice of the left pulmonary veins is seen through the ASD.
stenotic left pulmonary vein orifice
ceph R < - - • L
caud
left upper pulmonary
base of left atrial appendage
FIGURE 12-36. The obstruction is near the confluence of the left upper and lower pul- monary veins. A Y-shaped incision is made from the base of the left atrial appendage onto the upper and lower pulmonary veins, respectively.
left lower pulmonary
FIGURE 12-37. The incision over the area of stenosis is extended onto the left lower pulmonary vein.
R ^
anastomosis
FIGURE 12-38. Repair is performed by closing the incision as a V. The corner of the inci- sions over the pulmonary veins is moved to the child's right and stitched to the end of the incision at the base of the left atrial appendage. The closure is longitudinal, while the inci- sion was originally made in a transverse plane from the atrium into the veins.
ceph
R < - - > L
caud
closure of atrial septum
FIGURE 12-39. The ASD is closed primarily.
ceph R « - - • L
caud
atrial septum
stenotic orifice of right pulmonary veins
FIGURE 12-40. In another patient and after the cardiopulmonary bypass is established, with aortic clamping, cardioplegic arrest, and profound local cardiac cooling, a right atri- otomy is made. Stenosis of the right pulmonary veins is seen through the ASD.
right pulmonary vein orifice
caud
FIGURE 12-41. The area of vein stenosis admits a 1-mm probe.
R <
interrupted stitches in anastomosis
FIGURE
12-42. By working outside the heart, a transverse incision is made from the atrial wall into the right pulmonary vein and across the area of stenosis. This incision is closed longitudinally with interrupted sutures.
R ^
right pulmonary veins
caud
FIGURE
12-43. After the repair is completed, a 5-mm probe passes easily through the
anastomosis.
ceph R < - - • L
caud
patch over atrial septal defect
FIGURE 12-44. To avoid distortion of the vein repair, the laterally positioned ASD is closed with a Dacron® patch.
