Pediatric Supravalvar Aortic Stenosis Differential Diagnoses

Updated: Jan 27, 2021
  • Author: Anita Krishnan, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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DDx

Diagnostic Considerations

Supravalvar aortic stenosis (SVAS) should be considered in the differential diagnosis of children or infants presenting with a suspicious murmur or with signs of syncope, fatigue, angina, exercise intolerance, or left ventricular hypertrophy or dysfunction. Additionally, a cardiac evaluation should be performed with this diagnosis in mind in an infant or child with Williams syndrome.

It should be differentiated from valvar and subvalvar aortic stenosis. This differentiation can be made through the use of two-dimensional and pulse-wave echocardiography to delineate the level of obstruction and through measurements of the aorta above the level of the sinotubular junction. Cardiac catheterization or MRI is frequently indicated after the diagnosis of SVAS is made in order to evaluate for coronary involvement or stenosis of the arch vessels. [15]

Differential Diagnoses