Pulmonary Stenosis

Question 1

What are the causes of pulmonary stenosis?

Answer 1

1. Congenital
2. Noonan’s syndrome
3. Congenital rubella
4. Carcinoid
5. Obstructing tumours
6. Infective endocarditis (bulky vegetation)
7. Rheumatic heart disease (very rare)

Question 2

What are the clinical signs of severe pulmonary stenosis?

Answer 2

1. Raised JVP with giant a waves
2. Left parasternal heave (right ventricular heave)
3. Thrill in pulmonary area
4. Widely split second heart sound
5. Quiet P2
6. Mid systolic crescendo-decrescendo murmur over pulmonary area +/- ejection click
7. Signs of heart failure

Question 3

What is Noonan’s syndrome?

Answer 3

Autosomal dominant condition in chromosome 12
Male phenotypic form of Turner’s syndrome, but with normal karyotype
Right sided cardiac lesions including pulmonary stenosis, ASD, VSD

Question 4

How would you investigate a patient with pulmonary stenosis?

Answer 4

ECG
- RVH
- RAH
- RAD

CXR
- Post-stenotic dilation of main PA
- Diminished pulmonary vascular markings
- Apex lifted off left hemidiaphragm

TTE: pulmonary valve peak systolic gradient, Doppler jet velocity
- Mild: < 36mmHg ( <3m/s)
- Moderate: 36-64mmHg (3-4m/s)
- Severe: > 64mmHg (>4m/s)

TEE - delineate RV outflow tract, infundibular hypertrophy

Question 5

How would you manage a patient with pulmonary stenosis?

Answer 5

Endocarditis prophylaxis
Mild: often require follow-up only
Moderate: follow-up
Severe: valvuloplasty, valve repair or valve replacement

Question 6

How would you grade the severity of pulmonary stenosis?

Answer 6

Trans-valvular gradient

Mild: <50mmHg
Moderate: 50-79mmHg
Severe:>80mmHg