Michelena Hector I
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, USA.
Ann Cardiothorac Surg. 2022 Jul;11(4):402-417. doi: 10.21037/acs-2022-bav-24.
The congenital bicuspid aortic valve (BAV) condition is a valvulo-aortopathy with heterogeneous phenotypic expressions and clinical outcomes. A multitude of pre-existing classification systems, some extensive and some succinct, utilize combinations of numbers and/or letters to describe the condition. These diverse nomenclature systems are used according to the random preference of clinicians and researchers, generating confusion in clinical practice and research alike, effectively creating a barrier of communication at multiple levels. Based on imaging, pathology, surgery and clinical history evidence-based principles, and in utilizing the English language, the international nomenclature and classification consensus on the congenital BAV and its aortopathy offers a simple nosologic and phenotypic format that covers all possible phenotypes and clinical presentations of the bicuspid valvulo-aortopathy. From the nosology perspective, the valvulo-aortopathy has three major clinical presentations: (I) typical valvulo-aortopathy; (II) complex valvulo-aortopathy; and (III) undiagnosed or uncomplicated valvulo-aortopathy. From the valvular perspective, the congenital BAV has three major phenotypic expressions: (I) the fused BAV; (II) the 2-sinus BAV; and (III) the partial fusion (forme fruste) BAV. From the aortopathy perspective, the condition has three major phenotypic expressions: (I) ascending phenotype; (II) root phenotype; and (III) extended phenotypes. The international consensus is intended for universal use by Clinicians (pediatric and adult), echocardiography sonographers and physicians, cardiovascular advanced-imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists and researchers encompassing clinical and basic research areas. When new landmark research is available, this international consensus may be subject to change in accordance with evidence-based data.
先天性二叶式主动脉瓣(BAV)疾病是一种瓣膜-主动脉病变,具有异质性的表型表达和临床结局。众多现有的分类系统,有的详尽,有的简洁,使用数字和/或字母组合来描述该疾病。这些多样的命名系统根据临床医生和研究人员的随机偏好使用,在临床实践和研究中均造成了混乱,实际上在多个层面形成了沟通障碍。基于影像学、病理学、手术和临床病史的循证原则,并使用英语,关于先天性BAV及其主动脉病变的国际命名和分类共识提供了一种简单的疾病分类学和表型形式,涵盖了二叶式瓣膜-主动脉病变所有可能的表型和临床表现。从疾病分类学角度来看,瓣膜-主动脉病变有三种主要临床表现:(I)典型瓣膜-主动脉病变;(II)复杂瓣膜-主动脉病变;(III)未诊断或无并发症的瓣膜-主动脉病变。从瓣膜角度来看,先天性BAV有三种主要表型表达:(I)融合型BAV;(II)双窦型BAV;(III)部分融合(不完全型)BAV。从主动脉病变角度来看,该疾病有三种主要表型表达:(I)升主动脉型;(II)主动脉根部型;(III)扩展型。该国际共识旨在供临床医生(儿科和成人)、超声心动图检查技师和医生、心血管高级影像学专家、介入心脏病学家、心血管外科医生、病理学家、遗传学家以及涵盖临床和基础研究领域的研究人员普遍使用。当有新的里程碑式研究出现时,这一国际共识可能会根据循证数据进行更改。