Publication Date

4-1-2017

Journal

The Texas Heart Journal

DOI

10.14503/THIJ-15-5602

PMID

28461798

Publication Date(s)

April 2017

Language

English

PMCID

PMC5408626

PubMedCentral® Posted Date

4-1-2017

PubMedCentral® Full Text Version

Post-Print

Published Open-Access

yes

Keywords

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

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.

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