congenital heart disease

Question 1

Types of ASDs

Answer 1

1. secundum (most common, associated with MVP)
2. primum (associated with cleft AV valves, cleft septal tv, cleft anterior mv, makes AV canal.)
3. sinus venosus (anomalous pulmonary return of RUPV most common, svc more common than IVC, IVC associated with scimitar syndrome)
4. coronary sinus (associated with persistent left SVC

Question 2

AV canal defect

Answer 2

made up of both primum ASD and inlet VSD. If one is present then it is just a partial AV canal defect

Question 3

scimitar syndrome

Answer 3

associated with inferior sinus venosus defect

scimitar vein from hypoplastic RLL of lung to IVC

aortopulmonary collaterals to hypoplastic RLL

Question 4

coronary sinus asd

Answer 4

essentially an unroofing of coronary sinus that allows LA blood in and then into RA. assocaiated with left svc

Question 5

ostium secundum ASD

Answer 5

defect is caudad from crista terminalis in the fossa ovalis

occurs in septum primum
associated with MVP

Question 6

ostium primum asd

Answer 6

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

Question 7

sinus venosus

Answer 7

SVC - RUPV anomaly, more cephalad than crista terminalis, can be seen at 0 degrees very high between LA and RA

Question 8

coronary sinus asd

Answer 8

Dilated coronary sinus > 1cm
Inject contrast into left arm which shows up in sinus before RA.

Question 9

VSDs

Answer 9

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

Question 10

membranous vsd

Answer 10

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.

Question 11

Outlet vsd

Answer 11

Type 1, supracristal, subpulmonic, subarterial, doubly committed, conal, intraconal
Most likely to have rcc prolapse and AR
Least common VSD

Question 12

Inlet vsd

Answer 12

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

Question 13

Muscular vsd

Answer 13

Trabecular defect, difficult to locate for surgeon. Inferior and posterior

Question 14

Anterior or posterior malalignment vsd

Answer 14

Seen in tof , shift of ivs to right and anterior. Synonym to membranous vsd

25% of patients with TOF have right aortic arch

Question 15

View to tell membranous from others

Answer 15

RV I/O

Question 16

What is significant problem in hypoplastic left heart syndrome

Answer 16

aortic insufficiency
unobstructed pulmonary vein and interatrial flow

Question 17

tetralogy of fallot

Answer 17

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

Question 18

Types of av canal

Answer 18

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

Question 19

bicuspid AV

Answer 19

most common congenital defect
associated with aortic aneurysms and dissections

Question 20

Gerbode defect

Answer 20

LVOT to RA shunt

Question 21

fabry disease

Answer 21

x linked recessive lysosomal storage disease from a galactosidase deficiency

similar to amyloidosis with biventricular abnormalities and reduced diastolic and systolic function

Question 22

problems of asd and vsd

Answer 22

asd- RV volume overload

VSD- LV volume overload (PV is open and volume goes to lungs) more likely to cause pHTN

Question 23

warden procedure

Answer 23

used for sinus venosus ASD

Take SVC and reroute it to RAA . Suture line allowing RUPV to drain to LA

Question 24

Ebsteins

Answer 24

large anterior “sail like” leaflet with septal leaflet that is inserted low in ventricle. Associated with WPW

Question 25

aorto pulmonary window vs pda

Answer 25

window is much larger and has diastolic runoff into the PA from aorta. Can look like severe AI on PWD

Question 26

williams syndrome

Answer 26

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

Question 27

HD goals for TOF

Answer 27

same as HOCM slow HR, full preload, increased SVR, low contractility

Question 28

cardiac situs

Answer 28

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

Question 29

right atrium

Answer 29

has pectinate muscles outside RAA, and RAA is broad based, triangular Has IVC, SVC, CS, crista terminalis

Question 30

left atrium

Answer 30

pectinate muscles only in the left atrium, longer and skinnier septum primum in left atrium, pulmonary veins

Question 31

AV valves

Answer 31

Generally RV has TV and LV has MV tricuspid has 3 and mitral has 2 leaflets Tricuspid is usually more apically inserted

Question 32

LV vs RV

Answer 32

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

Question 33

L transposition of great arteries

Answer 33

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

Question 34

D TGA

Answer 34

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

Question 35

LTGA vs DTGA path

Answer 35

RA-MV-LV-PV-PA-LA-TV-RV-AV-Ao = LTGA LA-MV-LV-PV-PA (PDA) - RA-TV-RV-AV-Ao = DTGA

Question 36

Digeorge syndrome associations

Answer 36

cotruncal malformations like persistent truncus arteriosus VSD TOF

Question 37

turner syndrome associations

Answer 37

Bicuspid AV, coarctation of aorta, Hypoplastic left heart syndrome, aortic stenosis

Question 38

What is lutembacher syndrome

Answer 38

mitral stenosis + asd