Transcatheter aortic valve replacement in patients with quadricuspid aortic valve in a single center

Abstract

Background: Quadricuspid aortic valve (QAV) is a rare congenital malformation that can present with aortic regurgitation or aortic stenosis (AS)), requiring surgical treatment. Transcatheter aortic valve replacement (TAVR) is an alternative treatment for older patients and its prognosis for QAV therapy remains challenging. We sought to examine our early experience with TAVR in patients with QAV.

Materials and methods: Prospectively collected data were retrospectively reviewed in patients with QAV undergoing TAVR in our institution.

Results: Five patients with QAV and AR or AS were treated with TAVR between January 2016 and January 2022. The mean age was 73.8 years (range 69-82 years), and the median Society of Thoracic Surgeons score was 7.51% (range 2.668-18.138%). Two patients had type B and three had either type A, D, or F according to the Hurwitz and Roberts classification for QAV. Four patients with pure aortic regurgitation underwent transapical TAVR using the J-Valve system, and the patient with severe AS underwent transfemoral TAVR using the Venus-A system. Procedural success was achieved in all five patients. Trivial paravalvular leak was only detected in one case after the procedure, and one patient received a permanent pacemaker due to high-degree atrioventricular block three days later. The median follow-up period was 18 (12-56) months. After discharge, no deaths occurred during the 1 year follow-up. All patients improved by ≥1 New York Heart Association functional class at 30 days; four patients were in functional class ≤II later in the follow-up period. All patients' heart failure symptoms improved considerably.

Conclusion: Our early experience with TAVR in QAV demonstrates these procedures to be feasible with acceptable early results. Further follow-up is necessary to determine the long-term outcomes of this modality.

Clinical trial registration: [ClinicalTrials.gov], identifier [NCT02917980].

Keywords: aortic regurgitation; aortic stenosis; quadricuspid aortic valve; transcatheter; transcatheter aortic valve replacement.

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Copyright © 2022 Liu, Zhai, Mao, Xu, Ma, Li, Jin and Yang.

Figures

FIGURE 1

Anatomical structures of the quadricuspid aortic valves of five patients demonstrated with computed tomography angiography (CTA), 3-dimensional (3D) simulation, and 3D printing models.

FIGURE 2

Procedural details of transcatheter aortic valve replacement (TAVR) with the self-expandable J-Valve prosthesis in a patient with a quadricuspid aortic valve with pure aortic regurgitation. (A) 2-Dimensional transesophageal echocardiography (TEE) with color Doppler showed aortic regurgitation in the quadricuspid aortic valve before the procedure. (B) Preoperative angiography showed four sinuses and aortic regurgitation. (C) Postoperative angiography showed the final position of a 29 mm J-Valve prosthesis without a paravalvular leak after deployment. (D) 2-dimensional TEE with color Doppler showed no aortic regurgitation postprocedure.

FIGURE 3

Procedural details of transcatheter aortic valve replacement (TAVR) with the self-expandable Venus-A prosthesis in a patient with quadricuspid aortic valve with aortic stenosis (AS) and regurgitation. (A) 2-Dimensional transesophageal echocardiography (TEE) with color Doppler showed aortic regurgitation in the quadricuspid aortic valve before the procedure. (B) Angiography showed a satisfactory position of the THV prosthesis before deployment. (C) Angiography showed the final position of a 32 mm Venus-A prosthesis without a paravalvular leak after deployment. (D) 2-Dimensional TEE with color Doppler showed no aortic regurgitation postprocedure.

FIGURE 4

Hurwitz and Roberts classification of the quadricuspid aortic valve (QAV). (A) QAV with four equal-sized cusps. (B) QAV with three equal-sized cusps and one smaller cusp. (C) QAV with two equal larger cusps and two equal smaller cusps. (D) QAV with one larger cusp, two mid-sized cusps, and one smaller cusp. (E) QAV with one larger cusp and three equal-sized smaller cusps. (F) QAV with two equal-sized large cusps and two unequal smaller cusps. (G) QAV with four unequal-sized cusps.