Obstructive lesions

Question 1

What are the types of pulmonary stenosis?

Answer 1

1. Valvular- usually seen in Noonan’s
2. Isolated infundibular- rare, ass with large VSD and TOF
3. Abberent hypertrophied muscular bands
4. Supravalvular PS-
   - –Single (main PA) or
   - –multiple (several small branches), associated with Williams, Noonans, Alagille, Ehler’s-danlos, Silver-Russell or congenital rubella

Question 2

What are clinical features of Pulmonary Stenosis?

Answer 2

1. Asymptomatic if mild
2. Exertional dyspnoea
3. Right heart failure
4. Arrhythmias, later in life
5. Newborns- poor feeding, tachypnoea and cyanosis

Question 3

Examination findings in pulmonary stenosis?

Answer 3

Precordium. RV tap and systolic thrill in ULSE
Heart sounds. ES click in ULSE only with PVS, S2 split widely
Murmur. Ejection systolic, ULSE, transmits to back (not carotid)
Hepatomegaly if CHF present

Question 4

ECG findings in PS?

Answer 4

RVH and RAD in moderate
RAH and RVH with strain in severe
LVH in neonates with critical PS

Question 5

CXR findings in PS?

Answer 5

Normal heart size usually

Post stenotic dilatation of pulmonary artery

Question 6

What are the management options for PS?

Answer 6

1. Nothing if mild (pressure gradient

Question 7

What are the types of Aortic stenosis?

Answer 7

Valvular - M:F 4:1. Uni/Bi/Tricuspid with stenosis
Sub valvular - Discrete, tunnel like or idiopathic hypertrophic
Supravalvular; Ass with Williams (low IQ, facies, high Ca, multiple PA stenoses)

Question 8

History findings of AS?

Answer 8

1. Neonates- CHF and duct dependant circulation
2. Mild-moderate: Asymptomatic to mild exertional dyspnoea
3. Exertional chest pain, easy fatigueability, syncope in severe

Question 9

Examination findings in Aortic stenosis?

Answer 9

Narrow pulse pressure in severe AS
Supravalvular AS may have higher BP in R arm then left
Systolic thrill in URSE
Ejection click
Paradoxically split S2 in severe AS
Murmur- Ejection systolic, aortic area, radiation to neck, carotid thrill

Question 10

ECG findings in AS?

Answer 10

LVH

Question 11

CXR findings in AS?

Answer 11

Usually normal heart size

Dilated ascending aorta or prominent knob- post stenotic dilatation

Question 12

How is AS managed?

Answer 12

Neonate- Valvotomy and then valve replacement

Older- If symptomatic or pressure gradient >50mmHg

Question 13

What is a COA associated with?

Answer 13

M>F 2:1
Bicuspid aortic valve (50-85%)
Mitral valve anomaly (10%)
VSD
Turner's (30%)
Berry aneurysm 10%

Question 14

How does COA present?

Answer 14

Bimodal

1. Neonates with duct dependant circulation
2. Asymptomatic infants and children. Weakness or pain in legs after exercise

Question 15

ECG findings in COA?

Answer 15

RVH in infants

LVH in older children

Question 16

CXR findings in COA?

Answer 16

Cardiomegaly with increased pulmonary vascular markings

Rib notching

Question 17

What is pulmonary stenosis associated with?

Answer 17

Noonan’s ad Maternal Warfarin use