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Pulmonary venous drainage development starts about the ____ week of gestation
Third
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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.
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A ______ refers to the exact anatomic attachment of the pulmonary vein to a chamber or other vein.
Connection
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______ is venous flow that is diverted from the chamber it actually connected to. Usually a result of an ____.
- Drainage
- ASD or malpositioned IAS
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Abnormalities in pulmonary venous connections are (common/rare)
Rare
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What are the 2 major subgroups of pulmonary venous anomalies?
Total anomalous pulmonary venous connections (TAPVCs)
Partial anamalous pulmonary venous connections (PAPVCs)
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TAPVC:
Total Anamalous PV Connections. All PVs, from both lungs, don't connect to the LA. Pt must have ASD or PFO to survive.
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PAPVC:
Abnl connection of PV to the LA (at least one connected). Many variations, may have ASD.
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What are "common" diseases besides TAPVC and PAPVC?
- Abnl # of PV
- Nl PV connection w/ anomalous drainage
- Stenotic connections
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Which view is best to determine the # of PV in infants and young children?
Subcostal or SSN crab view
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What are the different goals of the echocardiographic exam?
- Determine # of PVs
- PV Connections
- PV drainage
- Position/status of IAS
- Systemic venous connection
- Hemodynamics
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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
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How are TAPVCs classified?
By position of anomalous connection relative to the heart.
Supracardiac, cardiac, infracardiac, mixed
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Supracardiac TAPVC:
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.
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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
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Cardiac TAPVC:
All PVs connect to a vein that directly enters the r. side of the heart, usually CS w/o obstruction.
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Infracardiac TAPVC:
All PVs connect to a vertical vein the descends below the diaphragm.
Associated with severs PHTN and obstruction of that vertical vein @ the diaphragm.
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TAPVC usually require _____ _____ after birth.
emergency surgery
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There's a single right or single left PV in ___% of the population.
25%
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TAPVCs occur more commonly in (females/males)
Males
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PAPVCs can involve R or L sided PVs, but (rarely/commonly) from both lungs in same pt.
Rarely
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What is the most commonly seen PAPVC?
r. sided PV connection to the SVC or RA
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Pulmonary venous obstruction causes _______ in the affected lobe(s).
PHTN
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What determines the effect of a TAPVC?
- Presence of venous obstruction
- Ability of ASD to allow flow into systemic circulation
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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
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A (big/small) interatrial connection causes overload of pulmonary circulation, worsening PHTN and systemic output.
Small
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Obstructed TAPVCs occur more frequently in (males/females)
Males
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Infants w/ obstructed TAPVC usually are _____ in their first month.
Symptomatic. Dyspnea w/ rapidly progressing cardiorespiratory failure.
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PAPVC pathophysiology is similar to an ____.
ASD
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What's the most common cause of anamalous drainage w/ nl connections?
Leftward malposition of the septum primum (thin part of fossa ovalis)
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SVD:
Sinus venosus defect: absence of sinous venosus tissue between r. PVs and SVC
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Cor triatriatum sinister:
Result of incomplete incorporation of the common PV into the posterior LA during development.
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Atresia of the common PV is (rare/common)
Rare
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PAPVC is associated with _______ syndromes
Turner and Noonan
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TAPVCs are associated with _____, ______, and _______ syndromes.
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