Citro Rodolfo, Cecconi Moreno, La Carrubba Salvatore, Bossone Eduardo, Antonini-Canterin Francesco, Nistri Stefano, Chirillo Fabio, Dentamaro Ilaria, Bellino Michele, Posteraro Alfredo, Giorgi Mauro, Petrella Licia, Monte Ines, Manuppelli Vincenzo, Mantero Antonio, Carerj Scipione, Benedetto Frank, Colonna Paolo
Heart Department, University Hospital "San Giovanni di Dio e Ruggi d'Aragona," Salerno, Italy.
Department of Cardiovascular Science, University Hospital "Ospedali Riuniti Ancona," Ancona, Italy.
J Cardiovasc Echogr. 2018 Apr-Jun;28(2):78-89. doi: 10.4103/jcecho.jcecho_5_18.
Bicuspid aortic valve (BAV) is the most common congenital heart disease, affecting 0.5%-2% of the general population. It is associated not only with notable valvular risk (aortic stenosis and/or regurgitation, endocarditis) but also with aortopathy with a wide spectrum of unpredictable clinical presentations, including aneurysmal dilation of the aortic root and/or ascending thoracic aorta, isthmic coarctation, aortic dissection, or wall rupture.
The REgistro della Valvola Aortica Bicuspide della Società Italiana di ECocardiografia e CArdiovascular Imaging is a retrospective (from January 1, 2010)/prospective, multicenter, observational registry, expected to enroll 3000 patients with definitive diagnosis of BAV made by transthoracic and/or transesophageal echocardiography, computed tomography, cardiovascular magnetic resonance, or at surgery. Inclusion criteria were definitive diagnosis of BAV. Patients will be enrolled regardless of the presence and severity of aortic valve dysfunction or aortic vessel disease and the coexistence of other congenital cardiovascular malformations. Exclusion criteria were uncertain BAV diagnosis, impossibility of obtaining informed consent, inability to carry out the follow-up. Anamnestic, demographic, clinical, and instrumental data collected both at first evaluation and during follow-up will be integrated into dedicated software. The aim is to derive a data set of unselected BAV patients with the main purpose of assessing the current clinical presentation, management, and outcomes of BAV.
A multicenter registry covering a large population of BAV patients could have a profound impact on the understanding of the natural history of this disease and could influence its management.
二叶式主动脉瓣（BAV）是最常见的先天性心脏病，影响着0.5%-2%的普通人群。它不仅与显著的瓣膜风险（主动脉狭窄和/或反流、心内膜炎）相关，还与具有广泛不可预测临床表现的主动脉病变相关，包括主动脉根部和/或升主动脉瘤样扩张、峡部缩窄、主动脉夹层或管壁破裂。
意大利超声心动图和心血管成像学会的二叶式主动脉瓣登记研究是一项回顾性（从2010年1月1日起）/前瞻性、多中心、观察性登记研究，预计纳入3000例经胸和/或经食管超声心动图、计算机断层扫描、心血管磁共振或手术确诊为BAV的患者。纳入标准为确诊BAV。无论主动脉瓣功能障碍或主动脉血管疾病的存在和严重程度以及其他先天性心血管畸形的共存情况如何，患者均可纳入。排除标准为BAV诊断不确定、无法获得知情同意、无法进行随访。首次评估和随访期间收集的病史、人口统计学、临床和器械数据将整合到专用软件中。目的是得出一组未经选择的BAV患者数据集，主要目的是评估BAV的当前临床表现、管理和结局。
一项覆盖大量BAV患者的多中心登记研究可能会对理解该疾病的自然史产生深远影响，并可能影响其管理。
