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32 Cards in this Set
- Front
- Back
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What is the most common type of R-L shunt?
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Tetralogy of fallot
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What causes TOF?
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-Anterior-superior displacement of the infundibular septum with misalignment with the muscular septum
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What are the 4 features of TOF?
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1. VSD (large and nonrestrictive usually perimembranous)
2. Pulmonary stenosis 3. Aorta overrides the VSD 4. RVH |
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What are really the only 2 requirements of TOF?
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-Large non restrictive VSD
-Pulmonary stenosis |
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What valve is often bicuspid or unicuspid in TOF?
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The pulmonic valve
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What often complicates repair plans in TOF?
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Anomalous coronary artery branching
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What do the clinical consequences of TOF depend on?
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The severity of pulmonary stenosis and obstruction to right ventricular outflow
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What is it called when pulmonary stenosis is mild and it's like an isolated VSD?
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Pink tetralogy
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What happens as the resistance to RV outflow approaches systemic vasc resistance levels?
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Right-to left shunting through the VSD becomes more severe.
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So pink tetralogy differs from classic tetralogy how?
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Pink - mild, no cyanosis
Classic - R-L shunting because of severe pulm hypertension; cyanotic |
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What happens with progressively worse TOF?
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-Pulmonic vessels shrink
-Aorta gets bigger |
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What is the bright side of there being pulmonic stenosis in TOF?
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It prevents volume overload in the RV, and the pressure is decompressed as blood flows into the LV; RV failure is rare.
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What weird thing is seen in 20% of patients with TOF?
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Right sided aortic arch
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What is the most severe type of TOF?
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Pulmonary ATRESIA with the VSD
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When the pulmonic vessel is atretic, how does blood get to the lungs for oxygenation?
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Must be thru the ductus, or systemic arteries anastomosing with the bronchial arteries.
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What R-L shunt CHD is strongly associated with males?
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TGA - transposition of the great vessels
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What is seen in TGA?
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-Aorta coming off the RV
-Pulmonic coming off the LV |
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What is required to allow for mixing and some oxygenation of systemic blood in TGA?
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A shunt
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What are 2 types of shunts seen in TGA? Which is stable? Which is more commonly seen?
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-VSD which is stable; only 35%
-Patent ductus - unstable; 65% |
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How is TGA repaired?
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By surgically switching the vessels back to their correct connections.
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What did older repairs do?
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Switched them sortve, leaving the RV with the systemic pressure work
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What do repairs do now?
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Switch them back the right way
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What is a Truncus arteriosus?
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Failure of the embryologic truncus to separate into the aorta and pulmonary artery.
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What is the result of a truncus arteriosus?
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One single great artery that recieves blood from both ventricles.
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What is Truncus arteriosus always associated with?
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A large VSD
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Over what is the truncus vessel situated in 2/3 or cases? 1/3?
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2/3 - overrides the septum
1/3 - entirely off the RV |
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What is the hallmark of Tricuspid atresia?
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Hypoplastic Right ventricle
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Why is there R-L shunting in Tricuspid atresia?
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Because blood can't get out of the RA; it has to shunt thru a patent foramen or ASD
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What is seen clinically in Tricuspid atresia cases?
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Marked cyanosis and high mortality early in life.
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What is Total anomalous pulmonary venous connection associated with?
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Heterotaxy syndromes and defects in body situs
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What happens in TAPVC?
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The pulm veins don't return to the left atrium, but elsewhere.
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What is the resulting pathologic change in the heart in TAPVC?
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Hypoplastic left atrium
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