A case of adult unicuspid aortic valve stenosis that underwent emergent aortic valve replacement for severe heart failure

Abstract

A 68-year-old woman was admitted to our hospital for progressive respiratory distress and orthopnea. Marked elevation of serum concentrations of BNP and H-troponin, and findings of lung congestion with bilateral pleural effusion on chest X-p, suggested severe congestive heart failure. Transthoracic echocardiography showed a marked decrease in left ventricular wall motion, and the ejection fraction was 23%. The aortic valve showed pure severe stenosis and the effective orifice area was 0.3 cm². Using color Doppler echocardiography, the orifice of the aortic valve showed only one commissure, indicating unicuspid valve. Coronary angiography showed no significant stenosis. Owing to the poor response to medical treatment for heart failure, emergent aortic valve and ascending aortic replacement was performed. The anatomical surgical finding of the aortic valve showed that the valve orifice was a 2×12-mm slit-like shape with one commissure, suggesting unicuspid aortic valve. Adult aortic unicuspid valve is extremely rare, and it is difficult to diagnose by transthoracic echocardiography. Here, we report a rare case of unicuspid aortic valve diagnosed by means of precise observation with transthoracic echocardiography.