Anderson Robert H, Crucean Adrian, Spicer Diane E
Biosciences Division, Newcastle University, Newcastle upon Tyne NE1 7RU, UK.
Department of Paediatric Cardiac Surgery, Birmingham Women's and Children's Hospital, Birmingham B4 6NH, UK.
J Cardiovasc Dev Dis. 2023 Mar 23;10(4):133. doi: 10.3390/jcdd10040133.
As yet, there is no agreed definition for the so-called "hypoplastic left heart syndrome". Even its origin remains contentious. Noonan and Nadas, who as far as we can establish first grouped together patients as belonging to a "syndrome" in 1958, suggested that Lev had named the entity. Lev, however, when writing in 1952, had described "hypoplasia of the aortic outflow tract complex". In his initial description, as with Noonan and Nadas, he included cases with ventricular septal defects. In a subsequent account, he suggested that only those with an intact ventricular septum be included within the syndrome. There is much to commend this later approach. When assessed on the basis of the integrity of the ventricular septum, the hearts to be included can be interpreted as showing an acquired disease of fetal life. Recognition of this fact is important to those seeking to establish the genetic background of left ventricular hypoplasia. Flow is also of importance, with septal integrity then influencing its effect on the structure of the hypoplastic ventricle. In our review, we summarise the evidence supporting the notion that an intact ventricular septum should now be part of the definition of the hypoplastic left heart syndrome.
迄今为止，对于所谓的“左心发育不全综合征”尚无公认的定义。甚至其起源也存在争议。据我们所知，努南（Noonan）和纳达斯（Nadas）在1958年首次将患者归为一个“综合征”类别，他们认为是列夫（Lev）命名了这个病症。然而，列夫在1952年撰写文章时，描述的是“主动脉流出道复合体发育不全”。在他最初的描述中，和努南与纳达斯一样，他纳入了室间隔缺损的病例。在随后的一篇报道中，他建议该综合征仅应包括室间隔完整的病例。后一种方法有很多值得称赞之处。当根据室间隔的完整性进行评估时，纳入的心脏可被解释为显示出胎儿期获得性疾病。认识到这一事实对于那些试图确定左心室发育不全遗传背景的人来说很重要。血流也很重要，室间隔的完整性会影响其对发育不全心室结构的作用。在我们的综述中，我们总结了支持以下观点的证据：完整的室间隔现在应成为左心发育不全综合征定义的一部分。
