Supravalvar aortic stenosis with supravalvar pulmonary stenosis and peripheral vascular stenoses.
A non-dysmorphic 10 month old female was discovered at surgery to have severe vasculopathy of both the systemic and pulmonary arteries. These findings were confirmed by pathologic examination. Follow-up angiography has confirmed multiple sites of vascular obstruction which appear to be worsening. Angioplasty has only partially relieved these obstructions. The pathology and possible etiology are reviewed.
D021921:Aortic Stenosis, Supravalvular / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery*; D003251:Constriction, Pathologic / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D016491:Peripheral Vascular Diseases / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery*; D011666:Pulmonary Valve Stenosis / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery*