Outflow obstruction

Question 1

What is aortic stenosis?

Answer 1

the aortic valve leaflets are partly fused together
there may be 1 to 3 aortic leaflets, leading to restrictive exit from the LV
aortic stenosis may not be associated with an isolated lesion, there may be mitral valve stenosis and coarctation of the aorta and these must be excluded

Question 2

what are the clinical features of aortic stenosis?

Answer 2

present with an asymptomatic murmur

severe: reduced exercise tolerance, chest pain, syncope
neonatal: heart failure, duct dependent circulation

Question 3

What are the physical signs of aortic stenosis?

Answer 3

small volume
slow rising pulse
carotid thrill
ejection systolic murmur maximal at upper right sternal edge
delayed soft aortic second sound
apical ejection click

Question 4

What investigations are performed is aortic stenosis is suspected?

Answer 4

CXR: normal or post stenotic dilatation of the aorta

ECG: LVH, deep S wave in V2 and tall R in V6, downgoing T wave suggests severe stenosis

Question 5

Management of aortic stenosis

Answer 5

regular clinical and echo assessment is required to determine when to intervene
symptomatic children: balloon valvotomy
children with significant stenosis often require aortic valve replacement

Question 6

What is Pulmonary stenosis

Answer 6

leaflets are partly fused together leading to restrictive exit from the RV

Question 7

What are the clinical features of pulmonary stenosis?

Answer 7

asymptomatic

clinically diagnosed

Question 8

Physical signs of pulmonary stenosis

Answer 8

an ejection systolic murmur best heard at upper left sternal edge, thrill may be present

an ejection click

soft or absent p2

severe lesion: prolonged RV impulse with delayed valve closure

Question 9

pulmonary stenosis investigations

Answer 9

CXR: normal or post stenotic dilatation

ECG: RVH, upright T wave in V1

Question 10

pulmonary stenosis management

Answer 10

transcatheter balloon dilatation required when pressure across the pulmonary valve becomes markedly increased

Question 11

coarctation of the aorta

Answer 11

not duct dependent, uncommon lesion

Question 12

clinical features of coarctation of the aorta

Answer 12

asymptomatic
systemic HTN in right arm
ejection systolic murmur upper right sternal edge
collateral supply towards posterior
radio-femoral delay due to collateral supply

Question 13

investigations for coarctation of the aorta

Answer 13

CXR: ‘rib notching’ due to large collateral intercostal arteries.
3 sign, visible notch at site of coarctation

ECG: LVH, deep S wave in V2 and tall R in V6, downgoing T wave

palpate for absent femoral pulses in children

Question 14

management for coarctation of the aorta

Answer 14

echo, when severe insert stent

Question 15

what is interruption of the aortic arch?

Answer 15

severe coarctation with no connection between the aorta proximally and distally
VSD also usually present
presents in neonatal period
features of duct dependent circulation

Question 16

what is hypoplastic left heart syndrome?

Answer 16

underdevelopment of the entire left side of the heart
mitral valve is small
LV is diminutive 
aortic valve is abnormal
usually coarctation

Question 17

hypoplastic left heart syndrome features and management

Answer 17

features:
detected antenatally at USS
very sick at birth with duct dependent circulation
absent PP’s

management:
neonatal operation known as Norwood procedure, followed by Glenns
Prostaglandin for duct dependent lesions