胎儿肺动脉闭锁伴动脉导管相关的肺动脉分支狭窄的产前特征。
Prenatal features of ductus arteriosus-related branch pulmonary stenosis in fetal pulmonary atresia.
机构信息
Fetal & Neonatal Cardiology Program, Division of Cardiology, Department of Pediatrics, Stollery Children's Hospital, Women's & Children's Health Research Institute and Mazankowski Alberta Heart Institute, University of Alberta, Edmonton, AB, Canada.
出版信息
Ultrasound Obstet Gynecol. 2021 Sep;58(3):411-419. doi: 10.1002/uog.23576. Epub 2021 Aug 4.
OBJECTIVE
Ductus arteriosus (DA)-related branch pulmonary stenosis (PS), due to ductal tissue migration into the proximal pulmonary artery (PA) ipsilateral to the DA, is common in newborns with pulmonary atresia (PAtr) and contributes significantly to their mortality and morbidity. We sought to define fetal echocardiographic predictors of DA-PS in PAtr.
METHODS
This was a study of all neonates diagnosed prenatally with PAtr and a DA-dependent pulmonary circulation, with a DA that joined the underbelly of the arch, who had undergone surgical or catheter intervention in our hospital between 2009 and 2018. The postnatal echocardiograms and clinical records were reviewed to confirm the presence or absence of DA-PS based on the need for angioplasty at initial intervention and/or development of proximal PA stenosis post intervention. Fetal echocardiograms were examined for the features of DA-PS.
RESULTS
Of 53 fetuses with PAtr, 34 (64%) had analyzable images, including 20/34 (59%) with and 14/34 (41%) without DA-PS. An inability to visualize the branch PAs in the same plane, largely associated with abnormal DA insertion into the ipsilateral PA (85% of cases), had sensitivity, specificity and positive (PPV) and negative (NPV) predictive values of 75%, 100%, 100% and 74%, respectively, for the prediction of postnatal DA-PS. The mean branch PA posterior bifurcation angle was more obtuse in cases with DA-PS compared to cases without DA-PS (117° ± 17° vs 79° ± 17°, P < 0.001), and an angle of > 100°, the preoperative cut-off observed previously in affected newborns, had a sensitivity, specificity, PPV and NPV of 88%, 79%, 82% and 85%, respectively. The receiver-operating-characteristics curve revealed an angle of ≥ 105° to have a sensitivity and specificity of 88% and 93%, respectively, for prenatal prediction of DA-PS. The presence of one or both features (inability to image in the same plane and the posterior bifurcation angle of ≥ 105°) had a sensitivity, specificity, PPV and NPV of 100%, 93%, 95% and 100%, respectively.
CONCLUSION
An inability to visualize the branch PAs in the same plane, associated with abnormal insertion of the DA in most cases, and/or the presence of a posterior PA bifurcation angle of ≥ 105° are predictive features of postnatal DA-PS in fetuses with PAtr. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.
目的
动脉导管（DA）相关的分支肺动脉狭窄（PS）是由于导管组织迁移到同侧 DA 的近端肺动脉（PA）引起的，在伴有肺动脉闭锁（PAtr）的新生儿中很常见，并且对其死亡率和发病率有显著影响。我们试图确定与 PAtr 相关的胎儿超声心动图预测因素。
方法
这是一项对 2009 年至 2018 年间在我院接受手术或导管介入治疗的所有产前诊断为 PAtr 和依赖 DA 的肺循环且 DA 与弓的下腹部相连的新生儿进行的研究。回顾术后超声心动图和临床记录，根据初始介入时是否需要球囊扩张和/或介入后近端 PA 狭窄的发展来确定是否存在 DA-PS。对胎儿超声心动图进行 DA-PS 的特征检查。
结果
在 53 例 PAtr 胎儿中，有 34 例（64%）有可分析的图像，其中 20/34 例（59%）和 14/34 例（41%）有或无 DA-PS。不能在同一平面上观察到分支 PA ，主要与 DA 异常插入同侧 PA（85%的病例）有关，其对预测出生后 DA-PS 的敏感度、特异度、阳性预测值（PPV）和阴性预测值（NPV）分别为 75%、100%、100%和 74%。与无 DA-PS 组相比，DA-PS 组的分支 PA 后分叉角更钝角（117°±17° vs 79°±17°，P<0.001），且>100°的术前切点（先前在受影响的新生儿中观察到）的敏感度、特异度、PPV 和 NPV 分别为 88%、79%、82%和 85%。受试者工作特征曲线显示，≥105°的角度对产前预测 DA-PS 的敏感度和特异度分别为 88%和 93%。存在一个或两个特征（不能在同一平面成像和后 PA 分叉角≥105°）的敏感度、特异度、PPV 和 NPV 分别为 100%、93%、95%和 100%。
结论
在伴有 PAtr 的胎儿中，不能在同一平面上观察到分支 PA，与大多数情况下 DA 的异常插入有关，和/或存在后 PA 分叉角≥105°，是出生后 DA-PS 的预测特征。© 2020 年国际妇产科超声学会。
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