Congenital Heart Disease Flashcards
What is the Pentalogy of Cantrell?
5 midline defects:
- Omphalocele
- Anterior diaphragmatic hernia
- Sternal cleft
- Ectopic cordis (heart outside the chest)
- Intracardiac defects (VSD, diverticulum of the LV)
William’s syndrome CHD association
Supravalvular aortic stenosis
Aortic arch abnormality associated with ToF
Right-sided aortic arch (about 25% of cases)
Morphologic characteristics of the RA
- Pectinate muscles extend outside the RAA
- RAA is broad based, triangular, anterior
- Septum secundum (limbus of fossa ovalis) rests on the right side of the interatrial septum
- IVC, SVC, and CS (usually)
- Crista terminalis, tinea sagitallis
Morphologic characteristics of the LA
- Pectinate muscles confined to the LAA (smoother walled)
- LAA is long, narrow (finger-like), located more posteriorly
- Septum primum rests on the left side of the interatrial septum
- Pulmonary veins (usually)
Which chamber does the atrioventricular valve define?
The ventricle (TV=RV, MV=LV)
Morphologic characteristics of the LV
- Mitral valve
- Smoother walls (less trabeculations)
- Two well-defined papillary muscles
- No muscular outflow tract
- Fibrous continuity between the atrioventricular and semilunar valve (can see in the ME LAX view)
Morphologic characteristics of the RV
- Tricuspid valve
- Trabeculated walls
- Moderator band
- Muscular conus
What is L-TGA?
“Living”
- Double discordance (atrioventricular discordance + ventriculoarterial discordance)
- Ventricular inversion
- Congenitally corrected
- Aortic and pulmonic valves in the same plane
- High incidence other cardiac abnormalities
- Very unstable conduction system! Put pacer pads on (high risk of heart block)
What is D-TGA?
“Deadly”
- Atrio-ventricular concordance
- Ventriculoarterial DISCORDANCE (Aorta comes off RV, pulmonary artery comes off LV)
- Two parallel circulations
- Reverse differential cyanosis (sats higher in the feet than the upper extremity due to flow through PDA)
- Rarely associated with other noncardiac abnormalities
- Ductal dependent
- 50% of patients have a VSD
What are the 5 cardiac lesions associated with L-TGA?
- VSD
- Tricuspid valve abnormalities
- LVOT obstruction (subpulmonic)
- Mitral valve abnormalities
- Complete heart block
What is the most common cyanotic CHD?
Tetrology of Fallot
What is the threshold gradient for intervention in HOCM and drug-refractory symptoms?
50mmHg
What gradient is the threshold for increased risk of SCD in HOCM?
30mmHg
What post-bypass LVOT peak instantaneous gradient should prompt a return to bypass?
> 3m/sec
