From Divisions of Cardiovascular Diseases (M.Y.C.T., N.M.A., V.T.N., P.A.P.) and Anatomic Pathology (W.D.E.), Mayo Clinic, Rochester, MN; and Division of Cardiovascular Diseases, Mayo Clinic, Scottsdale, AZ (M.M.A., T.Z.N.).
Circulation. 2016 Jan 19;133(3):312-9. doi: 10.1161/CIRCULATIONAHA.115.017743. Epub 2015 Dec 3.
Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect. This study sought to determine QAV frequency in a large echocardiography database, to characterize associated cardiovascular abnormalities, and to describe long-term outcomes.
Fifty patients (mean ± SD age, 43.5 ± 21.8 years at the time of the index diagnosis; female sex, 52%) received a diagnosis of QAV between January 1, 1975, and March 14, 2014 (frequency, 0.006%). The QAV was type A in 32% and type B in 32% (Hurwitz and Roberts classification). Aortic dilatation was present in 29% of the patients, and 26% had moderate or severe aortic valve regurgitation at the index diagnosis. Stenosis affected only 8% of the valves and was mild. Other findings, including abnormalities of other cardiac valves, septal defects, persistent left superior vena cava, and patent ductus arteriosus, were present in 32% of patients. During a mean ± SD follow-up of 4.8 ± 5.6 years, 8 patients underwent aortic valve surgery, with severe aortic valve regurgitation being the surgical indication in 7 patients. One patient with mild to moderate aortic valve regurgitation underwent aortic valve repair for obstruction of the left coronary ostium by the accessory cusp of QAV. No infective endocarditis or aortic dissection was found. Overall survival was 91.5% and 87.7% at 5 and 10 years.
Aortic dilatation and other structural cardiac abnormalities were relatively common among patients with QAV. Aortic valve regurgitation was the predominant hemodynamic abnormality and the indication for aortic valve surgery in most patients who received surgery. Long-term survival was excellent.
四叶式主动脉瓣（QAV）是一种罕见的先天性心脏缺陷。本研究旨在确定大型超声心动图数据库中 QAV 的频率，以明确其相关心血管异常，并描述其长期预后。
1975 年 1 月 1 日至 2014 年 3 月 14 日期间，共有 50 例患者（索引诊断时的平均年龄±标准差为 43.5±21.8 岁；女性占 52%）被诊断为 QAV（发生率为 0.006%）。根据 Hurwitz 和 Roberts 分类，QAV 中 A 型占 32%，B 型占 32%。29%的患者存在主动脉扩张，26%的患者在索引诊断时就有中度或重度主动脉瓣反流。仅有 8%的瓣膜存在狭窄，且狭窄程度较轻。其他发现，包括其他心脏瓣膜异常、间隔缺损、永存左上腔静脉和动脉导管未闭，在 32%的患者中存在。在平均随访 4.8±5.6 年后，8 例患者接受了主动脉瓣手术，7 例患者的手术指征是严重主动脉瓣反流。1 例存在轻至中度主动脉瓣反流的患者因 QAV 的副瓣阻塞左冠状动脉开口而行主动脉瓣修复术。无感染性心内膜炎或主动脉夹层。总体生存率分别为 91.5%和 87.7%，在 5 年和 10 年时。
在 QAV 患者中，主动脉扩张和其他结构性心脏异常相对常见。主动脉瓣反流是大多数接受手术患者的主要血流动力学异常和手术指征。长期生存率良好。
