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Pulmonary venous drainage development starts about the ____ week of gestation
Describe the embryology of the PVs.
A common atrium is formed beneath the splanchic plexus which is connected with the umbilicovitelline vein and cardinal veins.
Early in the second month, those two veins lose their connection and a common pulmonary vein drains into the LA.
This common PV is eventually absorbed be the LA and 4 smaller PVs are left.
A ______ refers to the exact anatomic attachment of the pulmonary vein to a chamber or other vein.
______ is venous flow that is diverted from the chamber it actually connected to. Usually a result of an ____.
- ASD or malpositioned IAS
Abnormalities in pulmonary venous connections are (common/rare)
What are the 2 major subgroups of pulmonary venous anomalies?
Total anomalous pulmonary venous connections (TAPVCs)
Partial anamalous pulmonary venous connections (PAPVCs)
Total Anamalous PV Connections. All PVs, from both lungs, don't connect to the LA. Pt must have ASD or PFO to survive.
Abnl connection of PV to the LA (at least one connected). Many variations, may have ASD.
What are "common" diseases besides TAPVC and PAPVC?
- Abnl # of PV
- Nl PV connection w/ anomalous drainage
- Stenotic connections
Which view is best to determine the # of PV in infants and young children?
Subcostal or SSN crab view
What are the different goals of the echocardiographic exam?
- Determine # of PVs
- PV Connections
- PV drainage
- Position/status of IAS
- Systemic venous connection
What is included in the hemodynamic assessment of the PVs?
- Flow direction
- Presence of PV obstruction
- Presence of atrial septal restriction
- Loading conditions
- Eval of TI jet
- RV dysfunction
How are TAPVCs classified?
By position of anomalous connection relative to the heart.
Supracardiac, cardiac, infracardiac, mixed
PVs come together in a common channel that enters a "vertical vein" which drains into the innominate vein on the l. side of the chest.
What is the most common TAPVC? When is it detected?
Supracardiac. Detected when kid starts gaining weight, is tachypnic, r. heart enlargement on x-ray
All PVs connect to a vein that directly enters the r. side of the heart, usually CS w/o obstruction.
All PVs connect to a vertical vein the descends below the diaphragm.
Associated with severs PHTN and obstruction of that vertical vein @ the diaphragm.
TAPVC usually require _____ _____ after birth.
There's a single right or single left PV in ___% of the population.
TAPVCs occur more commonly in (females/males)
PAPVCs can involve R or L sided PVs, but (rarely/commonly) from both lungs in same pt.
What is the most commonly seen PAPVC?
r. sided PV connection to the SVC or RA
Pulmonary venous obstruction causes _______ in the affected lobe(s).
What determines the effect of a TAPVC?
- Presence of venous obstruction
- Ability of ASD to allow flow into systemic circulation
What happens when there's no obstruction with a TAPVC?
Complete mixing of systemic venous and pulmonary venous flow in RA. RV dilation/hypertrophy. PHTN
A (big/small) interatrial connection causes overload of pulmonary circulation, worsening PHTN and systemic output.
Obstructed TAPVCs occur more frequently in (males/females)
Infants w/ obstructed TAPVC usually are _____ in their first month.
Symptomatic. Dyspnea w/ rapidly progressing cardiorespiratory failure.
PAPVC pathophysiology is similar to an ____.
What's the most common cause of anamalous drainage w/ nl connections?
Leftward malposition of the septum primum (thin part of fossa ovalis)
Sinus venosus defect: absence of sinous venosus tissue between r. PVs and SVC
Cor triatriatum sinister:
Result of incomplete incorporation of the common PV into the posterior LA during development.
Atresia of the common PV is (rare/common)
PAPVC is associated with _______ syndromes
Turner and Noonan
TAPVCs are associated with _____, ______, and _______ syndromes.