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
Which LV wall is usually spared in HOCM?
Basal inferolateral
Advantages and disadvantages of Sano shunt
Advantage:
- less decrease in Ao diastolic pressure (better CPP)
Disadvantage:
- RV ventriculotomy (can become an RV aneurysm with time)
Advantages and disadvantages of BTS
Advantage:
- No RV ventriculotomy
Disadvantage:
- Lower Ao diastolic pressures (worse CPP)
What is the inheritance pattern for HOCM?
Autosomal dominant with variable penetrance and variable expressivity
What are patients with William’s syndrome at risk for under anesthesia?
Sudden death with anesthesia
What chromosomal anomaly is associated with a BAV?
Turner’s syndrome
What is LV noncompaction? What ratio is diagnostic?
Hypertrabeculated, deeply recessed, disorganized, non-functional myocardium
Noncompacted-to- compacted myocardium >2:1 end systole (NC/C >2:1)
Noncompaction seen in mid and apical inferior and lateral walls
What is the wall thickness cutoff for HCM in a non-dilated LV?
> 15mm
