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Review
Pulmonary venous connection anomalies
| Question | Answer |
|---|---|
| name the 4 pulmonary venous anomalies | 1. abnormal number of pulm veins; 2 normal connections but anomalous drainage; 3 stenotic connections; 4 anomalous connections |
| An abnormal number of pulmonary veins is commonly encountered; what's the most common? | 25% of population have single right or single left pulm vein (more common) |
| name the 2 syndromes with higher PAPVC | PAPVC: Turner and Noonan |
| name the 3 syndromes with higher TAPVC | TAPVC: Holt-Oram, cat-eye, heterotaxy asplenia type (a common pulm vein accepts veins from both sides then drains into LA) |
| what do lungs and tracheobronchial tree derive from? | foregut |
| at what gestation age (in days) pulm veins get connected to common pulm vein and incorporated into posterior aspect of left atrium? | 32-33 days of gestation |
| how does normal pulm venous connections lead to anomalous drainage | leftward septum primum common in polysplenia (+no secundum); common atrium and ipsilateral drainage; sinus venosus |
| how does sinus venosus defect result in PAPVC? | sinus venosus = absence of sinus venosus btw RPVs and SVC or RA = 1-abnormal drainage |
| What are the stenotic pulm venous connection abnormalities | 1-cor triatriatum sinister; 2- individual PV stenosis can occur in normal heart or with ASD, VSD, less often with complex CHD like heterotaxy; 3- common PV stenosis often with infracardiac/infradiaphragmatic type of TAPVC Worse form is atresia Common PV |
| what is Cor triatriatum sinister? | incomplete incorporation of the CPV into the posterior aspect of the LA |
| what s a classic form of cor triatriatum? | membrane dividing LA into 2 compartments with a hole in it and atrial septum is intact. partial or subtotal cor triatriatum = involve veins from one side of the lungs vs. complete cor |
| PAPVC definition | One or more but not all pulm vein involved, different from abnormal drainage |
| what is most common PAPVC? | left PVs connecting to left cardinal vein derivatives (left innominate vein via vertical vein and coronary sinus AND right pvs connecting to right cardinal vein derivatives (IVC in Scimitar syndrome and SVC) – cross drainage possible |
| Scimitar syndrome (only with PAPVC) | Right PVs drain into IVC below diaphragm + Hypoplasia of right lung and RPA, secondary dextrocardia, bronchial abnormality, anomalous arterial connection from aorta to right lung, pulmonary sequestration |
| 4 types of TAPVCs | 1- supracardiac, 2-cardiac, 3-infracardiac, and 4-mixed. All need ASD or PFO to live. |
| Most common type of TAPVC | Supracardiac 47% of all TAPVC and 36% have left PV connecting to LIV via vertical vein that passes anterior to the LPA and mainstem bronchus, although occasionally this will pass between these structures results in clinically significant obstruction |
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