Congenital Heart Defects

cyanotic

reduced amount of oxygen turning skin blue

acyanotic

blood is oxygenated but circulation is poor

what kind of shunt is cyanotic?

right to left

hypoxia

decreased level of O2 in the blood

what kind of shunt is acyanotic?

left to right

results of an acyanotic lesion

right side volume overload and increased PAP

eisenmengers syndrome

media and intima of pulmonary arteries and arterioles become thick and narrow

tell tale sign of eisenmengers syndrome

chronic pulmonary HTN

what kind of shunt is associated with eisenmengers syndrome?

acyanotic, left to right

how does pulmonary resistance affect a shunt?

it can change the flow direction

what happens to arteries with eisenmengers syndrome over time?

they thrombose and obstruct

pulmonary pressures with eisenmengers syndrome

>70 mmhg

shunt direction of an ASD

acyanotic, left to right

what secondary changes can a significant ASD cause?

enlargement, hypertrophy, paradoxical septal motion

what is enlargement caused from?

volume overload

what is hypertrophy caused by?

pressure overload

location of ostium secundum

site of fossa ovalis, middle of septum

location of ostium primum

near AV valves

most common ASD type

ostium secundum

location of sinus venosus

near IVC, most superior

best window to view an ASD

subcostal

how to diagnose an ASD

bubble study in apical 4 or PSAX, measure dimension, QP/QS

what defect dimension is best for closure device?

< 38 mm

closure device best suited for ostium secundum ASD

septal occluder

PFO

failure of flap valve to seal completely between the atria after birth

PFO affected population

1/4 people

what can cause the flap to open with a PFO?

increased chest pressure

how to diagnose a PFO

bubble study

aneurysmal septum measurement

> or equal to 1.5 cm excursion from midline

what is an aneurysmal septum a potential site for?

PFO

percentage of membranous VSD

70-80%

percentage of muscular VSD

20-30%

what would cause a muscular VSD?

CAD or MI

where is a muscular VSD found?

toward the apex

where is a membranous VSD found?

toward the valves

inlet VSD

at the valves

where would you see a perimembranous VSD in PSAX AO level?

between 9-12 o'clock

PDA

persistent latency of fetal vessel that connected PA and AO

what type of shunt is PDA?

left to right

effects of a PDA

volume overload in the pulmonary artery, LA, and LV

what can large left to right shunts cause?

failure and CHF symptoms

murmur associated with PDA

continuous

what does color look like on a PDA?

shunt shows a mosaic jet in SSN

PDA closure devices

plug or coil

where is a coarctation normally found?

distal to the left subclavian artery

percentage of patients with a bicuspid aortic valve with a coarctation

80-85%

percentage of patients with a PDA with a coarctation

50%

sign of a coarctation

elevated systolic pressure in the upper extremities

coarctation treatment

repair, balloon, or surgical resection

Ebstein's anomaly

tricuspid valve leaflets are displaced apically, part of the RV is atrialized

effects of ebsteins

TR and RHF

ebsteins anomaly diagnosis

TV insertion point is > 20mm away from MV annulus

ebsteins treatment

annuloplasty, valve replacement

persistent left superior vena cava

fetal remnant of the vena cava, left SVC connects with the CS

what will the coronary sinus look like with persistent left SVC?

dilated

how to diagnose persistent left SVC?

bubble study will appear in the CS before the RA

partial anomalous pulmonary venous return

pulmonary veins are connected to the RA instead of the LA

what is commonly associated with partial anomalous pulmonary venous return?

sinus venosus ASD

tetralogy of fallot

overriding aorta, VSD, RVH, pulmonary stenosis

what is an overriding aorta

the aorta is shifted to the right

diagnosis of TOF

DOE, clubbing of digits, chronic hypoxia

D TGA

aorta comes from RV and pulmonary artery comes from LV

what is a symptom of D TGA?

extreme hypoxia

which type of TGA is a medical emergency?

Dextro type

what is maintained with D TGA?

a PDA

what is used to keep a PDA open?

prostaglandins

mustard/senning procedure

treatment for D TGA, that reroutes blood flow

arterial switch procedure

treatment for D TGA, they cut and switch the great vessels

Type L TGA

ventricles are switched

another name for Levo or type L TGA

congenitally corrected TGA

why does TGA Levo happen?

looping error in week 3

what percentage of Levo TGA patients have a VSD?

70%

explain the valve orientation of type L TGA

the valves match the ventricle and have the normal amount of papillary muscles

truncus arteriosus

one great artery arises from the heart, there is no septum between the pulmonary artery and aorta

what will you always have with truncus arteriosus?

VSD

sub aortic membrane

membrane covering part of the aortic valve

sub aortic membrane affect on LV

hypertrophy (pressure overload)

pulmonic stenosis

abnormal pulmonary valve cusps

types of PS

valvular

subvalvular

supravalvular

another name for RVOT

infundibulum

endocardial cushion defect

middle portion of heart did not develop

another name for endocardial cushion defect

AV canal

what abnormalities will you see with endocardial cushion defect?

ostium primum ASD, cleft MV, RV overload

cleft MV

Hole/slit in AMVL

Hypoplastic Left Heart Syndrome

underdevelopment of the left side of the heart, with missing AV, MV, or LV

what needs to stay open with Hypoplastic Left heart?

PDA and PFO

Atretic Tricuspid Valve

failure of the development of the TV, ASD and VSD present

dextrocardia

heart displaced to the right

situs inversus

left-right reversal of organ placement

right arch abnormality

aortic arch is on the right side

Cor triatriatum

a membrane that divides an atrium, usually the left

what do you need to check when diagnosing Cor triatriatum?

if it is obstructive or not

abnormalities with trisomy 21

ASD, VSD, PDA, or TOF

when the extra chromosome is on a lower #...

more defects

turners syndrome

missing part of chromosome, only affecting females

ratio of a coarctation in someone with turners syndrome

1/3

signs of turner's syndrome

short stature, may not undergo puberty

Noonan's syndrome

distinct facial features, similar to turners

defects associated with Noonan's?

partial AV canal

PS

secundum ASD

diffuse hypertrophy

dilated aorta

constrictive pericardium

Kawasaki syndrome

febrile that causes CA issues in children under 5 years