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首页> 外文期刊>European Heart Journal - Case Reports >Aortic stenosis of a bicuspid aortic valve in a patient with Klippel–Feil syndrome: a case report
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Aortic stenosis of a bicuspid aortic valve in a patient with Klippel–Feil syndrome: a case report

机译：KLIPPEL-FEIL综合征患者双裂性主动脉瓣主动脉狭窄：案例报告

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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. Anaesthetic review of cervical spine imaging showed fusion of the C2–C6 vertebral bodies and a desiccated bulging disc at C4–C5 but no significant foraminal narrowing in the lower cervical spine. Valve replacement with a mechanical aortic prosthesis resulted in an uneventful recovery and the patient was discharged home to follow-up. Discussion We report the first case of severe aortic valve stenosis requiring AVR in a Klippel–Feil patient, in whom the aortic valve was confirmed to be bicuspid. This report provides further evidence of an association of KFS with BAoV and strengthens the case for screening and follow-up of KFS patients for BAoV and other cardiovascular pathologies, the consequences of which may be serious.
机译：背景技术Klippel-Feil综合征（KFS）是颈椎的罕见先天性异常，其与许多心血管畸形有关，包括主动脉，双囊主动脉瓣和主动脉瘤和主动脉瘤的缩生。以前尚未报道患有KFS患者中BAOV主动脉狭窄的手术管理。案例摘要A 54岁的白种人妇女，已知KFS呈现给她当地医院的选修胆囊切除术。偶然发现了一种喷射的收缩杂音在术前次疗法上，这被证实是由于令人严重的令人严重的宝伐。胆囊切除术被取消，患者被称为我们的中心，并根据症状和预后的地面接受外科主动脉瓣置换（AVR）。宫颈脊柱成像的麻醉综述显示C4-C6椎体的融合和C4-C5的干燥凸出盘，但在下颈椎中没有显着的传染性变窄。阀门替代机械主动脉假体导致恢复不变，患者被排放回家。讨论，我们报告了在Klippel-Feil患者中需要AVR的严重主动脉瓣狭窄的第一种情况，其中主动脉瓣被证实是双裂的。本报告提供了进一步证明KFS与BAOV协会的证据，并加强了KFS患者的筛查和其他心血管病理学的筛选和随访，其后果可能是严重的。

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