congenital defects of the rt heart

most common cause of congenital pulmonary stenosis

congenital malformation of cusps

acquired pulmonic stenosis is _____ seen in adults

rarely

when might acquired pulmonic stenosis be seen

in patients with carcinoid syndrome or rheumatic disease

congenital pulmonary stenosis is most commonly seen in

children

as a result to obstruction of flow what is commonly demonstrated in the rt heart

rt ventricular hypertrophy

valvular stenosis is commonly associated with

• maternal rubella

• clubbing

• turner syndrome

• noonan syndrome

• polycythemia vera

patients w moderate/severe pulmonic stenosis should have it corrected before becoming pregnant - why

increase in flow volume w pregnancy can cause significant complications

symptoms of pulmonic stenosis

• dyspnea on exertion

• sob

• chest pain

• syncope

• fatigue

best views to demonstrate ps

left parasternal and subcostals, psax offers best insonation angle

with ps, m mode will show a significant increase in

the a wave

how will leaflets look w ps

thickened w systolic doming

how will the heart look w ps

• rt ventricular hypertrophy

• ra dilation

• dilated main pa

mild, moderate, severe psv for ps

mild - .9 to 3 m/s

moderate - 3 to 4 m/s

severe - over 4 m/s

what ppg indicates severe ps

over 64 mmHg

this is associated with

tetralogy of fallot

this is associated with

william syndrome

pulmonary atresia

absence of pulmonary valve opening, pulmonary artery is supplide by the ductus arteriosus

in pulmonary atresia, if there is no vsd, how does blood get to the left heart from the right

a pfo or small asd needs to be patent

how does blood move w pulmonary atresia and vsd

deoxygenated blood moves from ra to rv, through vsd to lv, exits through aorta, a pda then moves blood from descending aorta to pulmonary arteries (left to right shunt)

how does blood move w pulmonary atresia and no vsd

deoxygenated blood moves from ra through atrial septum to la and then lv, blood exits the lv through aorta, then a pda moves blood from descending aorta to the pulmonary arteries (left to right shunt)

overall how will the heart appear w pulmonary atresia

• small, slit like rv

• dilated left heart structures

• flow reversal mpa = flow from pda backfills mpa

pseudotruncus

pulmonary atresia w vsd and overriding aorta

how is pseudotruncus differentiated from truncus arteriosus

pulmonary branch artery origins must be located

surgical correction options for pulmonary atresia

blalock-taussig shunt

fontan procedure

blalock-taussig procedure

connects the subclavian artery to the pulmonary artery to provide blood flow to the lungs

fontan procedure

venous return goes directly to the lungs

part 1 - glenn shunt: connect svc to rt pulmonary artery

part 2 - ivc connected by a conduit to rt pulmonary artery

tricuspid atresia

absence of opening in the tricuspid valve, causing a lack of direct communication between the rt atrium and rt ventricle

how does tricuspid atresia appear on us

• echogenic plate-like structure is documented btween ra and rv

• hypoplasic rv w dilated ra

• pfo must be present to allow flow

tricuspid atresia is best evaluated in what views

apical, subcostal 4

surgical correction options for tricuspid atresia

• palliative pulmonary artery banding

• norwood procedure

palliative pulmonary artery banding

may be performed first to reduce flow to lungs in cases w a large vsd, allows patient to wait until they are older for correction

norwood procedure

part 1 - atrial septectomy, blalock taussig shunt

part 2 - glenn shunt: connect svc to rt pulmonary artery

part 3 - fontan procedure: ivc connected by conduit to rt pulmonary artery

ebstein anomaly

congenital malformation where tricuspid leaflets are more inferior and toward the apex of the heart

symptoms of ebstein anomaly

• dyspnea

• cyanosis

• pedal edema

• hepatomegaly

• ascites

best view to diagnose ebstein

apical 4

mitral valve and tricuspid valve insertion points are normally under _____ apart

20 mm or 8 mm/m²

how does ebstein anomaly appear on us

• tv annulus in normal position, only leaflets displaced

• small rt ventricle

• very large rt atrium

• small d shaped left ventricle

what is ebstein anomaly associated with

• secundum asd (rt to left flow)

• 30% also have wolff-parkinson-white

how is ebstein anomaly treated

• cone procedure to repair tv

• glenn shunt used in cases that have functional pulmonary atresia due to malpositioned leaflets

eisenmenger syndrome

normal shunt flow is from rt to left, but chronic shunt flow can lead to increased rt heart pressures. eventually right ventricular pressures rise to exceed left and the shunt flow reverses

w eisenmenger syndrome, rvsp will be

over 120 mmHg

what does eisenmenger syndrome lead to

cyanosis and rt heart failure

most common cause of eisenmenger syndrome

vsd

persistent left svc

blood from left arm drains directly into coronary sinus through brachiocephalic vein

primary finding of persistent left svc

significantly dilated coronary sinus

how to see persistent left svc on us

plax view will demonstrate dilated coronary sinus between la and descending ao

how is persistent left svc imaged

image at ssn longitudinally, tilt left to view vertical vein adjacent to the aortic arch

if bubbles are used to confirm persistent left svc, what will happen in a positive study

bubbles will fill the coronary sinus before the rt atrium

aortopulmonary window

direct connection between the ASCENDING aorta and mpa

how does aortopulmonary window appear w doppler

diastolic flow reversal in descending thoracic aorta

continuous murmur

symptoms of aortopulmonary window

• respiratory infection

• tachypnea

• tachycardia

what does aortopulmonary window lead to

chf

pulmonary htn

what kind of shunting does aortopulmonary window cause

left to right unless eisenmenger syndrome is present

best view for evaluating aortopulmonary window

psax

patent ductus arteriosus

ductus arteriosus doesn’t close after birth, should close after 2 to 3 weeks, causing blood to be shunted through the ductus from the descending aorta to the pulmonary artery

what is pda associated w

premature birth

how does pda sound

continuous, high pitched machinery type murmur

symptoms of pda

cyanosis, esp in lower extremities

best view to demonstrate pda

high psax

what does pda cause in the heart

• left ventricular volume overload

• dilated hyperkinetic left ventricle

• dilated left atrium

• may see diastolic flow reversal in descending thoracic aorta

when can normal cardiac pressures be assumed

if the gradient revealed from shunt flow is equivalent to 100 mmHg, which is the normal different in pressure between pulmonary artery and aorta

treatment for pda

medical closure for newborns w indomethacin or device closure w a coil, occluder, or plug