Hello and welcome to the zero to finals podcast. My name is Tom and in this episode I'm going

to be talking to you about congenital pulmonary valve stenosis. And if you want to follow along

with written notes on this topic, you can follow along at zero definals.com slash pulmonary stenosis

or in the cardiology section of the 0.0.2 finals

pediatrics book. So let's get straight into it. The pulmonary valve sits between the right

ventricle and the pulmonary artery. It usually consists of three leaflets that open and close to let blood out into the pulmonary artery and prevent blood returning to the right ventricle.

These leaflets can develop abnormally, becoming thickened or fused.

This results in a narrow opening between the right ventricle and the pulmonary artery,

and this is called congenital pulmonary valve stenosis.

Congenital pulmonary valve stenosis often occurs without any associations, but it can be associated with other conditions such as tetralogy of fallow,

William syndrome, noonan syndrome, and congenital rubella syndrome.

Let's talk about the presentation.

Often pulmonary valve stenosis is completely asymptomatic and is discovered as an incidental

finding of a murmur during routine baby checks.

More significant pulmonary valveowsdenosis can present with

symptoms of fatigue on exertion, shortness of breath, dizziness and fainting. There's a few signs to

look for on examination. Typically the murmur is an ejection systolic murmur heard loudest at the

pulmonary area, which is the

second intercostal space left sternal border. There may be a palpable thrill in the pulmonary area.

Right ventricular heave can be present due to right ventricular hypertrophy, due to that right

ventricle becoming thicker and stronger

trying to pump blood against that narrow pulmonary valve, and a raised JVP can be present with

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