Aortic stenosis of a bicuspid aortic valve in a patient with Klippel-Feil syndrome: a case report.

ABSTRACT:

Background

Klippel-Feil syndrome (KFS) is a rare congenital anomaly of the cervical spine, which is associated with a number of cardiovascular malformations, including coarctation of the aorta, bicuspid aortic valve (BAoV), and aortic aneurysm. Operative management of aortic stenosis of a BAoV in a patient with KFS has not been previously reported.

Case summary

A 54-year-old Caucasian woman with known KFS presented to her local hospital for elective cholecystectomy. An ejection systolic murmur was found incidentally on preoperative workup, which was confirmed to be due to a severely stenosed BAoV. The cholecystectomy was cancelled, and the patient was referred to our centre and accepted for surgical aortic valve replacement (AVR) based on symptomatic and prognostic grounds.

SUBMITTER: Hammond RFL 

PROVIDER: S-EPMC7319826 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature