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Year : 2015  |  Volume : 25  |  Issue : 3  |  Page : 93-95

Isolated supravalvar aortic stenosis without William's syndrome

1 Department of Adult Cardiology, Philippine Heart Center, Quezon City, Metro Manila, Philippines
2 Section of Non Invasive Cardiology, Philippine Heart Center, Quezon City, Metro Manila, Philippines

Correspondence Address:
Lucky Romero Cuenza
Department of Adult Cardiology, Philippine Heart Center, East Avenue 0850, Quezon City, Metro Manila
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2211-4122.166089

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Supravalvular aortic stenosis, characterized by narrowing of the ascending aorta above the valve, is the least common form of left ventricular outflow tract obstruction and is usually associated with William's syndrome. We present a case of a 27-year-old male with isolated supravalvar aortic stenosis (SVAS) presenting with heart failure. This case underscores the fact that in rare cases sporadic SVAS can occur in isolation without the classic findings of William's syndrome and highlighting the importance of integration of clinical and echocardiographic recognition for definitive management.

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