Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota.
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota.
Am J Cardiol. 2019 Mar 15;123(6):967-971. doi: 10.1016/j.amjcard.2018.12.010. Epub 2018 Dec 19.
Unicuspid aortic valve (UAV) is a rare malformation that is often difficult to distinguish from a bicuspid aortic valve (BAV) with commissural fusion by echocardiography or intraoperative surgical inspection. This study assessed the accuracy of intraoperative surgical inspection and two-dimensional echocardiography in diagnosing UAV compared to a gold standard of pathological diagnosis. The Mayo Clinic echocardiographic database, tissue registry database and electronic medical record were searched for all patients assigned a diagnosis of UAV by any technique. Transthoracic (TTE), transesophageal (TEE) echocardiographic, and surgical diagnoses were compared to pathological diagnosis as the standard. A clinical diagnosis of UAV was applied to 380 patients by 1 or more method and in 196 (52%) a pathologic evaluation was available to compare to the clinical description given by TTE, TEE, or surgical inspection. Of these 196 patients, only 58 (30%) had a pathological diagnosis of UAV; the majority were found to be BAVs by pathologic evaluation (n = 132, 67%). For diagnosing UAV, the sensitivity and specificity were 15% and 87% for TTE, 28%, and 82% for TEE, and 52% and 51% for surgical inspection, respectively. Valves with bicuspid morphology and extensive commissural fusion were frequently misclassified as UAV by all methods. In conclusion, intraoperative surgical inspection and echocardiography have limitations for diagnosing UAV due to difficulties in accurately assigning a correct morphological diagnosis, which suggests that the current understanding of the natural history of UAV may be inaccurate.
单瓣主动脉瓣（UAV）是一种罕见的畸形，通过超声心动图或术中手术检查，通常难以将其与融合嵴的二瓣主动脉瓣（BAV）区分开来。本研究评估了术中手术检查和二维超声心动图在诊断 UAV 方面的准确性，并与病理诊断的金标准进行了比较。通过任何技术诊断为 UAV 的患者，在梅奥诊所超声心动图数据库、组织登记数据库和电子病历中进行了搜索。经胸（TTE）、经食管（TEE）超声心动图和手术诊断与病理诊断作为标准进行了比较。通过一种或多种方法对 380 例患者进行了 UAV 的临床诊断，其中 196 例（52%）有病理评估结果，可与 TTE、TEE 或手术检查的临床描述进行比较。在这 196 例患者中，仅有 58 例（30%）有 UAV 的病理诊断；通过病理评估发现大多数为 BAV（n=132，67%）。对于诊断 UAV，TTE 的敏感性和特异性分别为 15%和 87%，TEE 为 28%和 82%，手术检查为 52%和 51%。具有二瓣形态和广泛融合嵴的瓣膜经常被所有方法错误地分类为 UAV。总之，由于难以准确地进行正确的形态学诊断，术中手术检查和超声心动图在诊断 UAV 方面存在局限性，这表明目前对 UAV 自然史的理解可能不准确。
