congenital heart disease Flashcards
Types of ASDs
- secundum (most common, associated with MVP)
- primum (associated with cleft AV valves, cleft septal tv, cleft anterior mv, makes AV canal.)
- sinus venosus (anomalous pulmonary return of RUPV most common, svc more common than IVC, IVC associated with scimitar syndrome)
- coronary sinus (associated with persistent left SVC
AV canal defect
made up of both primum ASD and inlet VSD. If one is present then it is just a partial AV canal defect
scimitar syndrome
associated with inferior sinus venosus defect
scimitar vein from hypoplastic RLL of lung to IVC
aortopulmonary collaterals to hypoplastic RLL
coronary sinus asd
essentially an unroofing of coronary sinus that allows LA blood in and then into RA. assocaiated with left svc
ostium secundum ASD
defect is caudad from crista terminalis in the fossa ovalis
occurs in septum primum
associated with MVP
ostium primum asd
associated with trisomy 21 and av canal defect (complete or partial, as well as av valve clefts)
Associated with conduction abnormalities. Can get complete heart block after repair
defect in endocardial cushions
sinus venosus
SVC - RUPV anomaly, more cephalad than crista terminalis, can be seen at 0 degrees very high between LA and RA
coronary sinus asd
Dilated coronary sinus > 1cm
Inject contrast into left arm which shows up in sinus before RA.
VSDs
anatomic: relative to crista supraventricularis (infracristal vs supracristal). Subpulmonic is suprascristal , AKA outlet or subarterial or type 1
membranous or subaortic or type 2 or perimembranous or conoventricular and is most common type
Inlet or type 3
muscular/trabecular or type 4
membranous vsd
most common type
para, peri, infracristal, subaortic, conoventricular
Type 2
can be repaired through RA, close to TR in RV I/O
conoventricular is very similar, between tricuspid and pulmonic valve in RV inflow outflow view
Associated with prolapse of rcc and ai. Lv septal aneurysm where tricuspid leaflet tries to plug hole and can prolapse into lv.
Outlet vsd
Type 1, supracristal, subpulmonic, subarterial, doubly committed, conal, intraconal
Most likely to have rcc prolapse and AR
Least common VSD
Inlet vsd
Av canal vsd, associated with primum asd. av septal defect vsd, endocardical cushion defect vsd
Trisomy 21, cleft leaflets
More posterior , don’t see anterior structures on echo.
primum asd = partial av canal
primum + restricted VSD = transitional av canal
primum +non restrictive inlet vsd = complete av canal
Muscular vsd
Trabecular defect, difficult to locate for surgeon. Inferior and posterior
Anterior or posterior malalignment vsd
Seen in tof , shift of ivs to right and anterior. Synonym to membranous vsd
25% of patients with TOF have right aortic arch
View to tell membranous from others
RV I/O
What is significant problem in hypoplastic left heart syndrome
aortic insufficiency
unobstructed pulmonary vein and interatrial flow
tetralogy of fallot
pulmonic stenosis
overriding aorta
membranous vsd (anterior and rightward malalignment)
RVH
Also can see anomalous origin of the LAD, right aortic arch, asd
Not associated with bicuspid aortic valve or coarctation of aorta
Types of av canal
complete - non restrictive inlet vsd and one fibrous ring around both av valves
transitional - restrictive inlet vsd and one fibrous ring
partial - just a primum ASD with 2 fibrous rings
bicuspid AV
most common congenital defect
associated with aortic aneurysms and dissections
Gerbode defect
LVOT to RA shunt
fabry disease
x linked recessive lysosomal storage disease from a galactosidase deficiency
similar to amyloidosis with biventricular abnormalities and reduced diastolic and systolic function
problems of asd and vsd
asd- RV volume overload
VSD- LV volume overload (PV is open and volume goes to lungs) more likely to cause pHTN
warden procedure
used for sinus venosus ASD
Take SVC and reroute it to RAA . Suture line allowing RUPV to drain to LA
Ebsteins
large anterior “sail like” leaflet with septal leaflet that is inserted low in ventricle. Associated with WPW
aorto pulmonary window vs pda
window is much larger and has diastolic runoff into the PA from aorta. Can look like severe AI on PWD
williams syndrome
high risk of sudden death after induction
associated with supravalvular aortic stenosis
chromosome 7 defect (elastin gene- non elastic) cocktail personality, elfen facies
stenotic coronary ostia, thick LV , ischemia
HD goals High SVR, low normal sinus rhythm, full preload, maintain contractility
HD goals for TOF
same as HOCM
slow HR, full preload, increased SVR, low contractility
cardiac situs
thoraco abdominal situs - solitus, inversus, ambiguous
cardiac position-levocardia, mesocardia, dextrocardia (apex position)
segment by segment analysis- Atria, AV valves, ventricles, outflow tracts, semilunar valves
look for other abnormalities
right atrium
has pectinate muscles outside RAA, and RAA is broad based, triangular
Has IVC, SVC, CS, crista terminalis
left atrium
pectinate muscles only in the left atrium, longer and skinnier
septum primum in left atrium, pulmonary veins
AV valves
Generally RV has TV and LV has MV
tricuspid has 3 and mitral has 2 leaflets
Tricuspid is usually more apically inserted
LV vs RV
LV has smoother walls, two pap muscles, no muscular outflow tract, and fibrous continuity between the MV and AV
RV wraps around heart, muscular conus outflow, TV more apically inserted, moderator band, complicated papillary muscles
L transposition of great arteries
Systemic pressures on RV and 90% have abnormal TV and other lesions, associated with heart block
Left side has subpulmonic stenosis and connected to pulmonic valve
Right side connected to aortic valve
also referred as double discordance or ventricular inversion or congenitally corrected TGA
In both D and L TGA aortic and pulmonic valves are in same plane
Counsel against pregnancy with EF < 40% and HF class 3/4- increased volume contributes to RV failure
PA banding decreases TR and trains LV before switch procedure
D TGA
Sub aortic conus with fibrous continuity between aortic and mitral valve
Aorta coming off RV and PA coming off LV
reverse differential cyanosis- higher saturations in feet after birth (post ductal sats>preductal sats)
Historically repaired with atrial switch procedure (still have systemic RV, arrythmias, leaks, etc.)
Now repaired with arterial switch procedure - have to reanastomose coronary arteries.
Rarely associated with other non cardiac abnormalities, 50% have vsd
LTGA vs DTGA path
RA-MV-LV-PV-PA-LA-TV-RV-AV-Ao = LTGA
LA-MV-LV-PV-PA (PDA) - RA-TV-RV-AV-Ao = DTGA
Digeorge syndrome associations
cotruncal malformations like persistent truncus arteriosus
VSD
TOF
turner syndrome associations
Bicuspid AV, coarctation of aorta, Hypoplastic left heart syndrome, aortic stenosis
What is lutembacher syndrome
mitral stenosis + asd
