Imaging, Treatment Options, Patient Selection, and Outcome Considerations for Patients With Bicuspid Aortic Valve Disease

Abstract

Transcatheter aortic valve replacement has emerged as a safe and effective alternative to surgical aortic valve replacement for patients with severe symptomatic aortic stenosis across the spectrum of surgical risks based on a series of foundational randomized clinical trials. Of note, patients with bicuspid aortic valve (BAV) disease were excluded from all these pivotal randomized trials, leaving a significant knowledge gap because BAVs are commonly encountered in patients referred for aortic valve surgery or intervention. In this comprehensive review, we aim to provide heart teams with a detailed insight into how to approach patients with BAV disease, focusing on imaging and characterization of bicuspid valves, an overview of surgical approaches, and an understanding of the current data behind the role of transcatheter aortic valve replacement for patients with BAV disease.

Keywords: bicuspid aortic valve; severe aortic stenosis; surgical aortic valve replacement; transcatheter aortic valve replacement.

Graphical abstract

Figure 1

(A) Parasternal echocardiographic imaging depicting systolic doming of the aortic valve leaflets (arrow). (B) Unequal sinuses of Valsalva observed using parasternal long-axis imaging. (C) Unequal sinuses observed using aortic short-axis imaging

Figure 2

Sievers and Schmidtke classification of the morphotypes of the bicuspid valve. Based on the presence and number of raphae, bicuspid aortic valves are classified into 3 categories. Type 0, no raphe (5%-7%); type 1, presence of 1 raphe with fusion of any 2 cusps (right-left cusp fusion [70%-80%], right-noncusp fusion [20%-30%] left-noncusp fusion [3%-6%]); and type 2, presence of 2 raphae. Partial-fusion bicuspid aortic valves (forme fruste) with small or mini raphae with partial fusion of the cusps, which is not part of the Sievers classification but is in the International Consensus Statement classification.

Figure 3

(A) Three-dimensional imaging of bicuspid aortic valve. (B) Multiplanar reconstruction of the aortic annulus with annular measurements.

Figure 4

(A) Three-dimensional assessment of aortic regurgitation. (B) Multiplanar alignment and measurement of 3-dimensional vena contracta area. VCA, vena contracta area.

Figure 5

(A) Parasternal imaging with measurement of the aortic root at the sinus, sinotubular junction, and ascending aorta using leading-edge-to-leading-edge measurement (yellow lines). (B) Pulse wave Doppler of the ascending aorta showing diastolic flow reversal in a patient with aortic regurgitation. (C) Doppler showing flow acceleration in the descending aorta in a patient with concomitant coarctation of the aorta.

Figure 6

Imaging demonstrating key measurements obtained during computed tomographic assessment for baseline and before the procedure.

Figure 7

Computed tomography imaging of the aorta demonstrating (A) aortopathy affecting the tubular ascending aorta, (B) coarctation of the aorta (arrow), and (C) aneurysmal dilatation of both the aortic root and ascending aorta.

Figure 8

Magnetic resonance imaging of aortic root thickness, aortic dimensions, and coronary height assessment.

Figure 9

Four-dimensional flow model (time-resolved, phase-contrast, 3-dimensional flow). (A) Flow through trileaflet aortic valve stenosis. (B) Turbulent flow through a bicuspid aortic valve with an aneurysm.

Central Illustration

Algorithm highlighting high-risk features on preassessment and the need for careful consideration prior to interventional planning. AV, aortic valve; CMR, cardiac magnetic resonance; CT, computed tomography; LVOT, left ventricular outflow tract; TAVR, transcatheter aortic valve replacement; TEE, transesophageal echocardiography.