Hi, this is Tom, and in this episode I'm going to be going through congenital pulmonary valve stenosis.

And you can find written notes at zero to finals.com and in the zero to finals pediatrics book.

And you can find flashcards and questions to train your knowledge at members.0.0.0.com.

So let's jump straight in. The pulmonary valve lets blood flow from the right ventricle to the

pulmonary arteries and prevents blood from flowing back into the right ventricle. It usually consists of three leaflets.

When these leaflets develop abnormally and become thickened or fused,

it can result in narrowing of the valve.

This is called congenital pulmonary valve stenosis.

Congenital pulmonary valve stenosis may be associated with tetralogy of fallow,

William syndrome, nunan syndrome, and congenital rubella syndrome.

Let's talk about the presentation.

Pulmonary stenosis is often asymptomatic and patients won't have any symptoms. More significant pulmonary valve stenosis can present with

fatigue on exertion, shortness of breath, dizziness, and syncope, or loss of consciousness.

Signs on examination may include an ejection systolic murmur which is heard loudest in the

pulmonary area, which is the second intercostal space left sternal border, a palpable thrill in the

pulmonary area, a right ventricular heave due to right ventricular hypertrophy or thickening

of the right ventricle, a raised JVP with giant A waves, and a widely split second heart sound.

The narrow pulmonary valve prolongs the time that it takes for the right ventricle to empty.

By contrast, the left ventricle empties relatively quickly through a healthy aortic valve.

Closure of the pulmonary valve is delayed and occurs noticeably later than the closure of the

aortic valve. This causes a widely split second heart sound, meaning that two separate sounds are heard rather than the

usual single second heart sound. Finally, let's go through management. An echocardiogram can be used

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