Unicuspid Aortic Valve Presenting with Decompensated Critical Aortic Stenosis

Abstract

•UAV is a rare congenital anomaly that leads to severe symptomatic stenosis.•Echocardiography plays a critical role in the evaluation of aortic stenosis.•Correctly distinguishing between UAV and BAV is relevant in determining intervention.

Keywords: Aortic stenosis; Aortic valve; Unicommisural; Unicuspid.

Graphical abstract

Figure 1

Electrocardiogram on admission to outside hospital showing normal sinus rhythm with J-point elevation and ST-segment changes concerning for left ventricular strain and left ventricular hypertrophy.

Figure 2

(A) Close-up image from transthoracic echocardiographic parasternal long-axis view showing heavily calcified, minimally mobile aortic valve. (B) Color flow Doppler parasternal long-axis view showing eccentric jet of aortic insufficiency (AI). (C) Transthoracic five-chamber apical view, demonstrating extension of aortic valve calcification from fused cusps into the left ventricular outflow tract. (D) Five-chamber apical view with color Doppler showing AI with main eccentric jet hugging the septum. See Videos 5-8.

Figure 3

Gradient across aortic valve showing severely elevated maximal velocity of 6.4 m/sec.

Figure 4

(A) Transesophageal echocardiographic (TEE) midesophageal aortic valve (AV) short-axis view showing en face view of heavily calcified, severely stenotic unicommisural UAV with and without color Doppler. (B) TEE midesophageal AV short-axis view showing a different angulation of the open commissure with a single attachment point. (C) TEE midesophageal long-axis view showing heavily calcified aortic root with a classic calcification pattern into the left ventricular outflow tract (LVOT), as well as an eccentric aortic regurgitation jet. (D) Long-axis midesophageal view taken at 120° also displaying unusual pattern of extended calcification into the LVOT. See Videos 5-8.

Figure 5

Direct visualization of explanted UAV.

Figure 6

The number of points of commissural contact with the aortic root is a helpful way to identify the number of aortic valve cusps present. Red arrows indicate commissural attachment sites, with cusps numbered in the diastolic views. LCC, Left coronary cusp; RCC, right coronary cusp; TTE PSAX AV, transthoracic parasternal short-axis view focusing on the aortic valve.