congenital heart disease

pressure equation

P=QxR

pressure gradient = blood flow x resistance

williams syndrome is an abnormality in what gene

elastin

noncardiac s/s of williams syndrome

developmental delay

cocktail party personality

elfin facies

cardiac s/s of williams syndrome

supravalve aortic stenosis

pulmonary artery stenosis

renal artery stenosis

what genetic syndromes are associated with CHD

down syndrome

turner syndrome

william syndrome

noonan syndrome

turner syndrome

female with XO karyotype

what cardiac abnormalities are seen with turner syndrome

coarctation of the aorta

bicuspid aortic valve

noonan syndrome genetics

autosomal dominant (12q)

noncardiac s/s of noonan syndrome

turner phenotype

short stature, webbed neck, hypertelorism

developmental delay

what cardiac abnormalities are seen with noonan syndrome

pulmonary valve stenosis

hypertrophic cardiomyopathy

down syndrome genetics

Trisomy 21

noncardiac s/s of down syndrome

hypotonia

developmental delay

what cardiac abnormalities are seen with down syndrome

AV septal defect

VSD

lithium is associated with

Ebstein's anomaly

complications of VSD

CHF

pulmonary HTN

bacterial endocarditis

what happens to the majority of small VSD's

close spontaneously

murmur for small VSD

holosystolic murmur at LLSB due to turbulent flow through VSD

2nd heart sound for small VSD

normal

the magnitude of a shunt depends on

size of hole

downstream resistance

murmur for medium/large VSD

ejection murmur

2nd heart sound for medium/large VSD

loud single S2

small VSD is usually

asymptomatic

s/s of large VSD

CHF

failure to thrive in infancy

abnormal intracardiac pressures for small VSD?

no, normal

abnormal intracardiac pressures for large VSD?

yes, pulmonary HTN

what is seen on chest x ray for VSD

large heart

increased vascularity

what can be seen in echo for large VSD

dilated LA, LV

what is seen on EKG for VSD

LVH

+/- RVH

what is seen in the transitional circulation for VSD

PA pressure high

delayed fall in PVR

high PVR limits PBF

when is surgery done for VSD

large shunt (>2:1)

large VSD

uncontrolled CHF

what is a reliable sign for CHF secondary to a shunt

hepatomegaly

what is the fick principle

uptake/release of any substance by an organ is the product of blood flow to the organ and arterio-venous concentration difference of the substance

oxygen content

actual amount of oxygen bound to hemoglobin

oxygen capacity

Maximum amount of oxygen blood can carry

Qp/Qs ratio

what is the Qp/Qs ratio

ratio of pulmonary to systemic blood flow

what is a normal Qp/Qs ratio

1:1

complications of ASD

late in life

R heart failure

atrial arrhythmias

pulmonary HTN

paradoxical emboli

ASD murmur

systolic ejection murmur at 2nd LICS from inc flow across PA

diastolic murmur from inc flow across TV

S2 ASD

fixed splitting (pulmonary valve closure delayed)

EKG findings for ASD

RVH

CXR findings for ASD

cardiomegaly

increased PVM (papillary muscle)

echo findings for ASD

enlarged RV, PA

when should an ASD be surgically closed

Qp:Qs >2

EKG findings for PDA

LVH

+/- RVH

other cardiac exam findings for ASD

RV heave

murmur for PDA

Continuous machine-like murmur

diastolic rumble (large shunt)

other cardiac exam findings for PDA

LV impulse

wide pulse pressure

echo findings for PDA

dilated LV and LA

can see ductus

CXR findings for large PDA

cardiomegaly with inc pulm vasc markings

large PDA can lead to what complications

CHF

pulmonary HTN

endocardial cushion defects are associated with

Down syndrome or trisomy 21

AV septal defect

Failure of superior and inferior endocardial cushions of AV canal to fuse

one common valve

AV septal defect is associated with

down syndrome

CXR findings for AV septal defect

huge heart

EKG findings for AVSD

left axis deviation

what is seen on physical exam for cyanotic heart disease

clubbing

kid is blue

EKG findings for TOF

RVH

CXR findings for TOF

boot shaped heart

dec pulm vasc

TOF is not associated with

CHF

severe TOF may be dependent on

PDA

cardio exam findings for TOF

RV tap

single S2

murmur TOF

ejection murmur

Hypercyanotic spells

AKA tet spells, a marker of TOF

dehydration, inc contractility --> worsening obstruction, less PBF

murmur disappears

LOC, seizure

what do pts with TOF do to relieve their s/s during a tet spell

squatting

inc SVR to reduce R to L shunt

treatment for a tet spell

O2

morphine

phenylephrine

B blocker

volume expansion

what can be given close a PDA

indomethacin

Presentation of transposition of great vessels

cyanosis unresponsive to oxygen

"happy blue kid"

cardiac findings for transposition of the great vessels

murmur

single S2

what is useful in kids with transposition of the great arteries

PGE1

treatment for transposition of the great vessels

balloon atrial septostomy

material switch

what other defects are required for tricuspid atresia

ASD

VSD

Qp:Qs for tricuspid atresia

< 1

treatment for tricuspid atresia

fontan operation

what is required for the Fontan operation

low PVR

unobstructed PA's

what is the Fontan operation

divert SVC and IVC flow directly into PA

the use of oxygen may worsen what defect

truncus arteriosus

O2 causes pulmonary vasodilation = dec PVR = more blood into lungs than aorta

s/s of truncus arteriosus

early CHF

early pulmonary vascular disease

what is dilated in truncus arteriosus

LA, LV

murmur associated with truncus arteriosus

ejection murmur (lots of blood through truncal valve)

diastolic murmur (lots of blood through mitral valve)

what other defect is needed in TAPVC

ASD

severe obstructive lesion can lead to

heart failure

low CO

syncope

sudden death

complication of pulmonary valve stenosis

RHF

pulmonic stenosis leads to hypertrophy of

RV

murmur for pulmonic stenosis

harsh SEM

click

pulmonic stenosis is associated with

Noonan syndrome

complications of aortic stenosis

endocarditis

sudden death

CHF

aortic stenosis leads to hypertrophy of

LV

aortic stenosis murmur

harsh SEM

click

treatment for pulmonic and aortic stenosis

balloon valvuloplasty

murmur for coarctation of the aorta

systolic murmur ULSB

critical coarctation of the aorta is dependent on

PDA

hypoplastic L heart is dependent on

PDA