Department of Pediatric Cardiology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
Department of Cardiovascular Surgery, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
Pediatr Neonatol. 2020 Feb;61(1):92-99. doi: 10.1016/j.pedneo.2019.06.013. Epub 2019 Jul 4.
Sinus venosus defect (SVD) is an unusual type of interatrial communication (IAC) and is virtually always associated with partial anomalous pulmonary venous drainage (PAPVD) of the right pulmonary veins (RPV) to the superior vena cava (SVC) or right atrium (RA). However, its definite morphogenesis is still elusive, and diagnostic fallibility continues.
We conducted a retrospective review of the echocardiograms, cardiac catheterization data, computed tomographic findings, and surgical notes of 44 children with surgery-confirmed isolated SVD from 1977 to 2016. We investigated the location of the IAC and its boundaries within the atrial septum and its anatomic relationship with the adjacent structures, including the anomalously draining RPV. We also tried to explore any possible associated abnormalities which might be implicated in the morphogenesis of SVD.
Two distinct types of IAC were defined. Forty patients had an IAC that was located posterosuperior to the intact fossa ovalis (superior type), and all were associated with PAPVD of the right upper and often the right middle pulmonary veins to the SVC. The remaining 4 patients had an IAC that was located posterior to the intact fossa ovalis (inferior type), and all were associated with PAPVD of all the RPV to the RA. Another consistently associated abnormality was a defect between the anomalously draining RPV posteriorly and the SVC or RA anteriorly. All these 44 patients underwent successful surgical baffling the associated PAPVD via the IAC into the left atrium.
A defect between the RPV posteriorly and the SVC or RA anteriorly will result in SVD, and an unusual type of IAC, and PAPVD of the RPV to the SVC or RA. The IAC is not a true atrial septal defect in the atrial septum proper, but it actually represents the left atrial orifice of the unroofed RPV.
静脉窦缺损（SVD）是一种不同寻常的房间隔缺损（IAC）类型，几乎总是与右肺静脉（RPV）部分异常肺静脉回流（PAPVD）至上腔静脉（SVC）或右心房（RA）相关。然而，其明确的形态发生仍然难以捉摸，诊断错误仍然存在。
我们回顾性分析了 1977 年至 2016 年间 44 例经手术证实的孤立性 SVD 患儿的超声心动图、心导管数据、计算机断层扫描结果和手术记录。我们研究了 IAC 的位置及其在房间隔内的边界及其与邻近结构的解剖关系，包括异常引流的 RPV。我们还试图探讨任何可能与 SVD 形态发生有关的相关异常。
定义了两种不同类型的 IAC。40 例患者的 IAC 位于完整卵圆窝后上方（上型），均伴有右上和右中肺静脉异常引流至 SVC。其余 4 例患者的 IAC 位于完整卵圆窝后下方（下型），均伴有所有 RPV 异常引流至 RA。另一个始终存在的相关异常是异常引流的 RPV 后部与 SVC 或 RA 前部之间的缺陷。所有这 44 例患者均成功地通过 IAC 将相关的 PAPVD 经房间隔旁路手术引流至左心房。
RPV 后部与 SVC 或 RA 前部之间的缺陷会导致 SVD、一种不同寻常的 IAC 和 RPV 至 SVC 或 RA 的 PAPVD。IAC 不是真正的房间隔缺损，而是未覆盖的 RPV 的左心房口。
