Pulmonary valve stenosis

Question 1

What is the pulmonary valve?

Answer 1

• the pulmonary valve usually consists of 3 leaflets that open and close to let blood out and prevent blood from returning to the heart
• these leaflets can develop abnormally, becoming thickened or fused
• this leads to a narrow opening between the RV and the pulmonary artery
• = congenital pulmonary valve stenosis

This condition is known as congenital pulmonary valve stenosis.

Question 2

What can congenital pulmonary valve stenosis be associated with?

Answer 2

• often without any associations
• TOF
• william syndrome
• noonan syndrome
• congenital rubella syndrome

Question 3

How does congenital pulmonary valve stenosis present?

Answer 3

It is often completely asymptomatic and discovered incidentally on routine baby check. More significant cases can present with fatigue on exertion, shortness of breath (SOB), dizziness, and fainting.

Question 4

What are the signs of congenital pulmonary valve stenosis?

Answer 4

• ejection systolic murmur heard loudest at the pulmonary area
• palpable thrill in the pulmonary area
• right ventricular heave due to RV hypertrophy
• raised JVP with giant A waves

Question 5

What is the diagnosis for congenital pulmonary valve stenosis?

Answer 5

The diagnosis is made using an echocardiogram.

Question 6

What is the management for congenital pulmonary valve stenosis?

Answer 6

• patients with mild pulmonary stenosis without symptoms generally don’t require any intervention. They’re followed up by a cardiologist with a watching and waiting approach
• if the patient is symptomatic or the valve is more significantly stenosed, balloon valvuloplasty via a venous catheter is performed → this involves inserting a catheter under x-ray guidance into the femoral vein → through the IVC and right side of the heart → to the pulmonary valve & dilating the valve by inflating the balloon
• if this isn’t appropriate or fails, open heart surgery is performed