Transcatheter Aortic Valve Implantation in a Patient with Unicuspid Aortic Valve

Abstract

Transcatheter aortic valve replacement (TAVR) in patients with bicuspid aortic valves has been successfully performed, but there is a lack of published experience in percutaneous treatment of patients with unicuspid valves and severe aortic stenosis. We describe a case of TAVR in such a patient. A 31-year-old woman with Turner syndrome-who had undergone coarctation repair via subclavian flap at age 7 days and an aortic valvotomy at age 6 weeks-presented with severe symptomatic aortic stenosis. She was deemed inoperable because of her severe pulmonary hypertension and numerous comorbidities; consequently, a 20-mm Edwards Sapien 3 Transcatheter Heart Valve was offered for compassionate use. Postdeployment angiography and transesophageal echocardiography and aortography revealed no aortic insufficiency. Transcatheter aortic valve replacement for unicuspid aortic valve stenosis is technically feasible. Before implantation, particular attention should be paid to the interplay between the large single leaflet, coronary ostia, and stented valve, to select the correct size and position of the device. Some degree of intraoperative aortic migration should be anticipated.

Keywords: Aortic valve/abnormalities/pathology; aortic valve stenosis/therapy; cardiac catheterization/instrumentation; heart defects, congenital/complications; heart valve diseases; heart valve prosthesis implantation/methods.

Fig. 1

Transesophageal echocardiogram shows severe aortic stenosis in the presence of a unicuspid aortic valve.

Fig. 2

Aortogram shows the inflated balloon with concomitant coronary flow.

Fig. 3

Postdeployment angiogram shows the valve in relation to the sinotubular junction and the aortic annulus. The presence of the aortic annulus can be inferred from the position of the pigtail catheter in the noncoronary cusp (in the absence of substantial calcification).

Fig. 4

Transesophageal echocardiogram shows the deployed valve in relation to the sinotubular junction (arrow) and the aortic annulus (arrowhead).

Fig. 5

Final angiogram shows the deployed valve in relation to the sinotubular junction and the aortic annulus. Note the absence of significant aortic regurgitation (suggested by the absence of contrast medium in the left ventricle).