No direct communication between the pulmonary veins and the LA. Instead, they
drain anomalously into the systemic venous tributaries or into the RA .
1)Supracardiac: 50% of TAPVR patients. The common pulmonary venous sinus
(posterior chamber) drains into the right SVC through the left vertical vein and the left
2)Cardiac: 20% of TAPVR patients. The pulmonary veins enter the RA separately
through four openings or the common pulmonary venous sinus drains into the
3)Infracardiac: 20% of TAPVR patients. The common pulmonary venous sinus drains
to the portal vein, hepatic vein, or IVC. The common pulmonary vein penetrates the
diaphragm through the esophageal hiatus.
4)Mixed type: combination of the other types, 10% of TAPVR patients.
Marked cyanosis and respiratory distress develop in the neonatal period
with failure to thrive.
Cyanosis worsens with feeding, especially in infants with the infracardiac
type, resulting from compression of the common pulmonary vein by the
food filled esophagus.
Features Common to All Types:
A large RV with a compressed LV (i.e., relative hypoplasia of the LV) A
RA and a small LA, dilated Pas.
An interatrial communication is usually present. PFO occurs in 70% of
patients, and secundum ASD occurs in 30%. RESTRICTIVE OR NOT BY
ECHO ,,, CT >> Size otherwise Rashkind procedure.
A large common chamber .
Without surgical repair, two thirds of the infants without obstruction die
before reaching 1 year of age. They usually die from superimposed
Patients with the infracardiac type rarely survive for longer than a few
weeks without surgery. Most die before 2 months of age.
Confirm the diagnosis ,,,,, Anatomy .
Exclude obstruction of the vertical /descending vein.
Size of ASD is it adequate for mixing or not .
Describe the course of the vertical vein ,,,, (not intra-parenchymal).
Supracardiac type,,, large, side-to-side anastomosis is made between the
common pulmonary venous sinus and the LA. The vertical vein is ligated. The ASD
TAPVR to the RA. The atrial septum is excised, and a patch is sewn in such
a way that the pulmonary venous return is diverted to the LA .
TAPVR to the coronary sinus.
An incision is made in the anterior wall of the coronary sinus (“unroofing”) to
make a communication between the coronary sinus and the LA.
Then ASD & the ostium of the coronary sinus are closed by patch.
Infracardiac type. A large vertical anastomosis is made between the
common pulmonary venous sinus and the LA. The common pulmonary
vein, which descends vertically to the abdominal cavity, is ligated above
One or more (but not all) pulmonary veins drain into the RA or its venous
tributaries such as the SVC, IVC, coronary sinus, and left innominate vein.
The right pulmonary veins are involved twice as often as the left pulmonary
The right pulmonary veins may drain into the SVC, often associated with a
sinus venosus defect , or drain into the IVC in association with an intact
atrial septum and bronchopulmonary sequestration (scimitar syndrome ).
The left pulmonary veins either drain into the left innominate vein or into
the coronary sinus . ASD is common.
Hemodynamic alterations are similar to those in ASD. Pulmonary blood
The magnitude of the pulmonary recirculation is determined by the
number of anomalous pulmonary veins, the presence and size of the ASD,
and the PVR.
Confirm the diagnosis.
Full assessment of the pulmonary veins (number, drainage of each
segment , exclude ostial stenosis)
If u noted four pulmonary veins draining into LA, this doesn't mean normal
venous drainage always ,,,,anomalous segmental drainage may be present .
Distance of each draining vein to SVC/RA junction.( what above the level
of the RPA /LPA not suitable for repair )
In cases of significant left-to-right shunt with a Qp/Qs ratio of greater than
Scimitar syndrome with severe hypoplasia of the right lung even with a
Qp/Qs ratio less than 2:1.
Surgery is carried out between the age of 2 and 5 years.