Severe unicuspid aortic valve stenosis: VACTERL association

Description

A 24-year-old man with a known history of horseshoe kidney leading to end-stage renal disease, scoliosis and small ventricular septal defect consistent with VACTERL association, who presented with worsening dyspnoea on exertion. Transthoracic echocardiogram (TTE) showed a unicuspid aortic valve (UAV) with severe aortic stenosis (AS) (valve area of 0.6 cm² and mean gradient of 44 mm Hg). TTE also showed moderate aortic insufficiency and severely reduced left ventricular systolic function with estimated ejection fraction of 20%. Transoesophageal echocardiography (TEE) confirmed a UAV with single commissure located anteriorly (figure 1A–D; online  and ) and severe AS (figure 1B, C). In preparation for surgical repair, chest CT angiogram showed dilated ascending aorta measuring 4 cm with no evidence of aortic dissection. The patient underwent aortic valve replacement surgery with 21 mm CarboMedics mechanical valve. TEE showed a well-seated prosthesis without evidence of paravalvular leak (figure 1E). The patient...

http://ift.tt/2wNNI2a