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Echo Boards > congenital heart disease > Flashcards

congenital heart disease Flashcards

1
Q

Types of ASDs

A
  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
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2
Q

AV canal defect

A

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

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3
Q

scimitar syndrome

A

associated with inferior sinus venosus defect

scimitar vein from hypoplastic RLL of lung to IVC

aortopulmonary collaterals to hypoplastic RLL

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4
Q

coronary sinus asd

A

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

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5
Q

ostium secundum ASD

A

defect is caudad from crista terminalis in the fossa ovalis

occurs in septum primum
associated with MVP

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6
Q

ostium primum asd

A

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

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7
Q

sinus venosus

A

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

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8
Q

coronary sinus asd

A

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

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9
Q

VSDs

A

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

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10
Q

membranous vsd

A

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.

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11
Q

Outlet vsd

A

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

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12
Q

Inlet vsd

A

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

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13
Q

Muscular vsd

A

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

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14
Q

Anterior or posterior malalignment vsd

A

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

25% of patients with TOF have right aortic arch

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15
Q

View to tell membranous from others

A

RV I/O

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16
Q

What is significant problem in hypoplastic left heart syndrome

A

aortic insufficiency
unobstructed pulmonary vein and interatrial flow

17
Q

tetralogy of fallot

A

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

18
Q

Types of av canal

A

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

19
Q

bicuspid AV

A

most common congenital defect
associated with aortic aneurysms and dissections

20
Q

Gerbode defect

A

LVOT to RA shunt

21
Q

fabry disease

A

x linked recessive lysosomal storage disease from a galactosidase deficiency

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

22
Q

problems of asd and vsd

A

asd- RV volume overload

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

23
Q

warden procedure

A

used for sinus venosus ASD

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

24
Q

Ebsteins

A

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

25
Q

aorto pulmonary window vs pda

A

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

26
Q

williams syndrome

A

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

27
Q

HD goals for TOF

A

same as HOCM

slow HR, full preload, increased SVR, low contractility

28
Q

cardiac situs

A

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

29
Q

right atrium

A

has pectinate muscles outside RAA, and RAA is broad based, triangular

Has IVC, SVC, CS, crista terminalis

30
Q

left atrium

A

pectinate muscles only in the left atrium, longer and skinnier

septum primum in left atrium, pulmonary veins

31
Q

AV valves

A

Generally RV has TV and LV has MV

tricuspid has 3 and mitral has 2 leaflets

Tricuspid is usually more apically inserted

32
Q

LV vs RV

A

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

33
Q

L transposition of great arteries

A

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

34
Q

D TGA

A

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

35
Q

LTGA vs DTGA path

A

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

36
Q

Digeorge syndrome associations

A

cotruncal malformations like persistent truncus arteriosus
VSD
TOF

37
Q

turner syndrome associations

A

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

38
Q

What is lutembacher syndrome

A

mitral stenosis + asd

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