Assessment of Unicuspid Aortic Valve Stenosis Using Multimodality Imaging, X-ray Radiography and Raman Analysis

Abstract

Unicuspid aortic valve (UAV) is an extremely rare form of congenital cardiac malformation, leading to aortic stenosis (AS), aortic regurgitation (AR), or both. We report the case of a 55-year-old man with unicommissural UAV associated with severe AS and mild AR using different multimodality imaging approaches. The excised UAV isolated after aortic valve replacement exhibited an eccentric "teardrop" opening with a slit-shaped unicommissural structure. Raman spectroscopic results indicated that 3 unevenly distributed components were deposited on the surface of the UAV, in which calcium hydroxyapatite and type-B carbonate apatite were the predominate components deposited on the surface, leading to severe AS formation. (Int Heart J 2019; 60: 482-488)

A Rare Case of Unicuspid Aortic Valve with Postoperative Heart Block

“…A TTE is unlikely to show enough detail to differentiate a unicuspid from a bicuspid aortic valve. Rarely, the diagnosis is made on intraoperative visualization of the valve [2,4]. As seen in our case, a TTE was only able to identify the presence of aortic stenosis, a follow-up TEE raised suspicion of UAV which was subsequently confirmed during the open surgical procedure.…”

“…A review by Mookadam et al reported that nearly 41% of the cases manifested with isolated aortic stenosis and 28% had aortic stenosis with or without regurgitation [3]. While a majority of UAV cases are diagnosed on autopsy or pathological examination of the excised valve, the diagnostic modality best suited to clinically diagnose UAV is a three-dimensional TEE [4]. A TTE is unlikely to show enough detail to differentiate a unicuspid from a bicuspid aortic valve.…”