Divisions of Cardiothoracic Surgery, University of Illinois College of Medicine at Chicago, 840 S Wood St Suite 417(MC 958), Chicago, IL, 60612, USA.
Rush University Medical College, Chicago, USA.
Pediatr Cardiol. 2021 Jun;42(5):993-1001. doi: 10.1007/s00246-021-02541-0. Epub 2021 May 28.
Unicuspid aortic valve (UAV) stenosis is a rare condition accounting for 5% of non-rheumatic aortic stenosis. The diagnosis can be difficult to make prior to surgical intervention and transesophageal echocardiography has been demonstrated to be more accurate in making the diagnosis compared to transthoracic echocardiography. The presence of a posteriorly located aortic orifice on the short-axis views, with one or two visible raphe anteriorly; the absence of commissures (acommissural); or the presence of a lone commissure (unicommissural) between the left and noncoronary, or the left and right cusps suggests the diagnosis. Patients with UAV are predominantly males and present with stenosis about a decade earlier than those with the more prevalent bicuspid aortic valves (BAV). They more commonly present with aortic annular dilatation and have fewer comorbidities at presentation compared to patients with BAV. Surgical management of UAV stenosis includes aortic valve replacement through standard open heart surgery or percutaneous transcatheter aortic valve replacement (TAVR), aortic valve repair either by bicuspidization, tricuspidization or trileaflet reconstruction, or the Ross procedure. Patients with UAV stenosis require less concomitant coronary or other cardiac procedures when they need surgical intervention, but are about a decade younger at the time of their death. UAV stenosis is a distinct congenital anomaly with a different natural course than BAV. Surgical management should be individualized based on the patient's age at presentation, aortoannular anatomy, and associated cardiac conditions.
单瓣化主动脉瓣狭窄（UAV）是一种罕见的疾病，占非风湿性主动脉瓣狭窄的 5%。在手术干预之前，诊断可能很困难，与经胸超声心动图相比，经食管超声心动图在诊断方面更为准确。在短轴视图上，主动脉瓣口位于后位，有一个或两个可见的前嵴；无交界（无交界）；或左瓣和非冠状动脉瓣之间存在一个单独的交界（单交界），或左瓣和右瓣之间存在一个单独的交界，提示诊断。UAV 患者主要为男性，比更常见的二叶式主动脉瓣（BAV）患者狭窄早约 10 年。与 BAV 患者相比，他们更常出现主动脉瓣环扩张，且在就诊时并发症较少。UAV 狭窄的手术治疗包括通过标准开胸手术或经皮经导管主动脉瓣置换术（TAVR）进行主动脉瓣置换，主动脉瓣修复通过双瓣化、三叶化或三叶瓣重建，或 Ross 手术。当需要手术干预时，UAV 狭窄患者需要同时进行冠状动脉或其他心脏手术的情况较少，但在死亡时年龄小约 10 岁。UAV 狭窄是一种独特的先天性异常，其自然病程与 BAV 不同。应根据患者的就诊年龄、主动脉瓣环解剖结构和相关心脏状况，对手术治疗进行个体化。
