Paediatric aortic stenosis

Question 1

What is congenital aortic valve stenosis?

Answer 1

Patients with congenital aortic valve stenosis are born with a narrow aortic valve that restricts blood flow from the left ventricle into the aorta. The severity of the stenosis varies between patients and will determine the symptoms.

Question 2

Aortic valve morphology in patients with congenital aortic valve stenosis?

Answer 2

The aortic valve is normally made up of three leaflets, called the aortic sinuses of Valsalva. Patients with aortic stenosis may have one, two, three or four leaflets.

Question 3

How does congenital aortic valve stenosis present?

Answer 3

Mild aortic stenosis can be completely asymptomatic, discovered as an incidental murmur during a routine examination. More significant aortic stenosis can present with symptoms of fatigue, shortness of breath, dizziness and fainting.

Symptoms are typically worse on exertion as the outflow from the left ventricle cannot keep up with demand.

Severe aortic stenosis will present with heart failure within months of birth.

Question 4

Examination findings aortic stenosis?

Answer 4

The key examination finding is an ejection systolic murmur heard loudest at the aortic area, which is the second intercostal space, right sternal border. It has a crescendo-decrescendo character and radiates to the carotids.

Other signs that may be present on examination are:

Ejection click just before the murmur
Palpable thrill during systole
Slow rising pulse and narrow pulse pressure

Question 5

Management for congenital aortic stenosis?

Answer 5

The gold standard investigation for establishing a diagnosis is an echocardiogram.

Congenital aortic stenosis tends to be a progressive condition that worsens over time. Patients need regular follow-up under a paediatric cardiologist, with echocardiograms, ECGs and exercise testing to monitor the progression of the condition. Patient with more significant stenosis may need to restrict physical activities.

Options for treating the stenosis are:

Percutaneous balloon aortic valvoplasty
Surgical aortic valvotomy
Valve replacement

Question 6

Complications of congenital aortic stenosis?

Answer 6

Left ventricular outflow tract obstruction
Heart failure
Ventricular arrhythmia
Bacterial endocarditis
Sudden death, often on exertion