Aortic valve stenosis

Question 1

What is aortic stenosis?

Answer 1

A congenital condition where kids are born with a narrow aortic valve that restricts blood flow from the left ventricle into the aorta. Severity varies between patients.

Question 2

What is the pathophysiology of aortic stenosis?

Answer 2

The aortic valve is normally made up of 3 leaflets, called the aortic sinuses of Valsalva, which allow blood to flow from the left ventricle into the aorta but prevent blood from flowing back into the left ventricle. Patients may have one, two, three, or four leaflets.

Question 3

What are the causes of aortic stenosis?

Answer 3

Causes include degenerative calcification (>65), bicuspid aortic valve (<65), William’s syndrome (supravalvular aortic stenosis), and post-rheumatic disease.

Question 4

How can a patient with aortic stenosis present?

Answer 4

• mild AS can be asymptomatic, discovered as an incidental murmur during a routine examination
• more significant AS can present with chest pain, fatigue, SOB, dizziness and syncope/presyncope
• symptoms can worsen on exertion as the outflow from the left ventricle can’t keep up with demand
• severe AS will present with heart failure within a few months of birth

Question 5

What signs are found on examination of aortic stenosis?

Answer 5

• ejection systolic murmur heard loudest at the aortic area, it has a crescendo-decrescendo character and radiates to the carotids
• ejection click just before the murmur
• palpable thrill during systole
• slow rising pulse and narrow pulse pressure

Question 6

What is the gold standard investigation for aortic stenosis?

Answer 6

The gold standard is an echocardiogram.

Question 7

What is the management for aortic stenosis?

Answer 7

• regular follow up needed with paediatric cardiologist with echo’s, ecg’s and exercise testing of monitor the progression (congenital aortic stenosis tends to be a progressive condition that worsens over time)
• patients with more significant AS may need to restrict physical activities
• percutaneous balloon aortic vavloplasty (inflating a balloon to stretch the valve via a catheter)
• surgical aortic valvotomy (open surgery via a midline sternotomy incision to widen the aortic valve)
• valve replacement (open surgery via midline sternotomy to replace the aortic valve)

Question 8

What are the complications of aortic stenosis?

Answer 8

• LV outflow tract obstruction
• heart failure
• ventricular arrhythmia
• bacterial endocarditis
• sudden death, often on exertion