TAPVR

• Accounts for 1% of CHD
• pulmonary veins bypass LA and drain to ectopic location
• there is always an ASD or PFO because it is the only way blood gets to the LA
• obstructive lesions may be usually infracardiac
• widely split s2 (ASD), s3 or s4 (lots of flow through RA, RV, since blood from both lungs and body drain to RA/RV.

Presentation: mild cyanosis from birth; tachypnea and CHF in infancy
PE: systolic ejection murmur of relative PS; diastolic rumble of relativeTS; widely split and fixed S2
EKG: RVH
CXR: cardiomegaly, increased pulmonary vascular markings

Pulmonary veins drain to:

1. Supracardiac 50%:  SVC > RA  > RV/lungs and ASD  > LA
2. Cardiac 20%:  RA > RV/lungs and ASD > LA > LV
3. Cardiac: coronary sinus > RA > ASD > LA
4. Infracardiac 20%: portal veins, hepatic veins or IVC

TAPVR (5 letters) Total anomalous pulmonary venous return
Tetralogy of Fallot (tetra=4)
Transposition of the Great Arteries (2 arteries)
Tricuspid Atresia (tri=3)
Truncus Arteriosus (1 trunk)

CHLA Board Review 2005
  Park MK. Pediatric Cardiology for Practictioners, 3rded. St. Louis,
    Mosby, 1996.