- Have a high index of suspicion for PAPVR if unexplained RA and RV enlargement without evidence of an ASD
- As with ASDs, having pulmonary HTN is uncommon with PAPVR
- Can easily be missed on TTE and cath
- Normally, the four pulmonary vv. are return oxygenated blood into the LA. In the case of PAPVR, you have one or more pulmonary veins dumping oxygenated blood into the systemic venous system (usually RA, SVC, or IVC).
- Most commonly, PAPVRs involve the:
- LUPV (left upper pulmonary vein) → ascending vertical vein → innominate vein or
- RUPV → SVC
- This type is often associated with a concurrent sinus venosus defect connecting the RA and LA
- Scimitar syndrome is a subtype of PAPVR in which part or all of the blood from the right lung is returned into the IVC.