Case history
Case history #1
A full-term 3.3 kg newborn girl is found to have a systolic ejection murmur shortly after birth. She is clinically asymptomatic and fully saturated while breathing room air. She has no dysmorphic features.
Case history #2
A 7-year-old boy with a normal karyotype and dysmorphic features such as webbing of neck, short stature, and pectus carinatum is incidentally found to have a prominent main pulmonary artery and cardiomegaly on chest x-ray for respiratory complaints.
Other presentations
Pulmonary stenosis (PS) may also present as critical PS in neonates. Critical PS is associated with severe right ventricular outflow obstruction and right-to-left shunting of blood at the atrial level, resulting in cyanosis. The systolic murmur may be variable, is usually prominent, but may be surprisingly soft if the cardiac output is limited and there is limited blood flow across the stenotic valve. PS may also rarely be associated with other systemic diseases including intracardiac tumours, neurofibromatosis, and extrinsic compression lesions (neoplasms).
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