Multiplane transoesophageal echocardiography is the only definitive ultrasound approach in adult supravalvular aortic stenosis

This report describes a rare case of isolated supravalvular aortic stenosis (SVAS) in a 28 year-old female patient. A congenital heart defect, diagnosed at birth, was until 1994 suspected to be a valvular aortic stenosis (VAS). Cardiac catheterization led to the diagnosis of supravalvular aortic stenosis, which could easily be confirmed by multiplane but not by monoplane transoesophageal echocardiography (TEE). Precordial examinations had not revealed the vitium, probably because the SVAS is a rare malformation of the ascending aorta, but with multiplane TEE the aortic narrowing could be imaged clearly and pressure gradients comparable to those found with invasive measurements were established. The advantages of this non-invasive method for diagnosis and preoperative preparation are discussed in detail.