Pulmonary Valve Stenosis

Question 1

What is the Pulmonary Valve?

Answer 1

• The pulmonary valve consists of three leaflets that open and close to let blood out
• These leaflets can develop abnormally becoming thickened or fused
• There is a narrowing between the right ventricle and the pulmonary artery

Question 2

What are the associations with pulmonary valve stenosis?

Answer 2

• Tetralogy of Fallot
• William Syndrome
• Noonan Syndrome
• Congenital Rubella Syndrome

Question 3

How does Pulmonary Valve Stenosis Present?

Answer 3

• Normally completely asymptomatic
• discovered incidentally during routine baby checks

More significant pulmonary valve stenosis:
- fatigue on exertion
- SOB
- dizziness
- fainting

Question 4

What are the signs of Pulmonary Valve Stenosis?

Answer 4

• Ejection Systolic murmur loudest at the pulmonary area - second intercostal space, left sternal border
• Palpable thrill in the pulmonary area
• Right Ventricular heave due to right ventricular hypertrophy
• Raised JVP with giant A waves

Question 5

What is the managment for Pulmonary Valve Stenosis?

Answer 5

• gold standard = ECHO
• mild pulmonary stenosis without intervention do not require any intervention and are followed up with a watching and waiting approach
• symptomatic or valve is stenosed = balloon valvuloplasty via a venous catheter
• Involves inserting a catheter under xray guidance into the femoral vein, through the inferior vena cava and right side of the heart to the pulmonary valve
• Dilating the valve by inflating the balloon
• If valvuloplasty is not appropriate or fails open-heart surgery can be performed