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Virtual Pediatric Hospital: Paediapaedia: Pulmonary Stenosis (PS) Paediapaedia: Cardiovascular Diseases

Pulmonary Stenosis (PS)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Usually asymptomatic with a systolic thrill associaed with an ejection type murmur heard best over the pulmonary area.

Etiology/Pathophysiology:
Can occur with the ventricle (infundibular stenosis), at the pulmon valve (valvular stenosis), or in the pulmonary artery (peripheral stenosis). Isolated valvular stenosis is much more common. Can occur by itself or in a complex isolated valvular stenosis. It is due to fusion of the 3 cusps of the valve. The stenosis leads to increased right ventricle end systolic pressure and right ventricular hypertrophy and then right ventricular dilation when the right ventricle decompensates.

Pathology:
Not applicable

Imaging Findings:
On the chest film post stenotic dilation of the main pulmonary artery is seen, especially in the left pulmonary artery because the jet of blood shoots straight into it. The heart is normal is size as is the pulmonary vascularity. Right ventricular hypertrophy does not change the size of the right ventricle but an upturned cardiac apex can be seen due to superior displacement of the left ventricle by the hypertrophied right ventricle.

DDX:

References:
See References Chapter.

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