Division of Prenatal Medicine, Gynecological Ultrasound and Fetal Surgery, Department of Obstetrics and Gynecology, University Hospital Cologne and Faculty of Medicine, University of Cologne, Cologne, Germany.
Department of Obstetrics and Prenatal Medicine, University of Bonn, Bonn, Germany.
Arch Gynecol Obstet. 2024 Apr;309(4):1353-1367. doi: 10.1007/s00404-023-06997-8. Epub 2023 Mar 27.
Restrictive foramen ovale (FO) in dextro-transposition of the great arteries (d-TGA) with intact ventricular septum may lead to severe life-threatening hypoxia within the first hours of life, making urgent balloon atrial septostomy (BAS) inevitable. Reliable prenatal prediction of restrictive FO is crucial in these cases. However, current prenatal echocardiographic markers show low predictive value, and prenatal prediction often fails with fatal consequences for a subset of newborns. In this study, we described our experience and aimed to identify reliable predictive markers for BAS.
We included 45 fetuses with isolated d-TGA that were diagnosed and delivered between 2010 and 2022 in two large German tertiary referral centers. Inclusion criteria were the availability of former prenatal ultrasound reports, of stored echocardiographic videos and still images, which had to be obtained within the last 14 days prior to delivery and that were of sufficient quality for retrospective re-analysis. Cardiac parameters were retrospectively assessed and their predictive value was evaluated.
Among the 45 included fetuses with d-TGA, 22 neonates had restrictive FO postnatally and required urgent BAS within the first 24 h of life. In contrast, 23 neonates had normal FO anatomy, but 4 of them unexpectedly showed inadequate interatrial mixing despite their normal FO anatomy, rapidly developed hypoxia and also required urgent BAS ('bad mixer'). Overall, 26 (58%) neonates required urgent BAS, whereas 19 (42%) achieved good O saturation and did not undergo urgent BAS. In the former prenatal ultrasound reports, restrictive FO with subsequent urgent BAS was correctly predicted in 11 of 22 cases (50% sensitivity), whereas a normal FO anatomy was correctly predicted in 19 of 23 cases (83% specificity). After current re-analysis of the stored videos and images, we identified three highly significant markers for restrictive FO: a FO diameter < 7 mm (p < 0.01), a fixed (p = 0.035) and a hypermobile (p = 0.014) FO flap. The maximum systolic flow velocities in the pulmonary veins were also significantly increased in restrictive FO (p = 0.021), but no cut-off value to reliably predict restrictive FO could be identified. If the above markers are applied, all 22 cases with restrictive FO and all 23 cases with normal FO anatomy could correctly be predicted (100% positive predictive value). Correct prediction of urgent BAS also succeeded in all 22 cases with restrictive FO (100% PPV), but naturally failed in 4 of the 23 cases with correctly predicted normal FO ('bad mixer') (82.6% negative predictive value).
Precise assessment of FO size and FO flap motility allows a reliable prenatal prediction of both restrictive and normal FO anatomy postnatally. Prediction of likelihood of urgent BAS also succeeds reliably in all fetuses with restrictive FO, but identification of the small subset of fetuses that also requires urgent BAS despite their normal FO anatomy fails, because the ability of sufficient postnatal interatrial mixing cannot be predicted prenatally. Therefore, all fetuses with prenatally diagnosed d-TGA should always be delivered in a tertiary center with cardiac catheter stand-by, allowing BAS within the first 24 h after birth, regardless of their predicted FO anatomy.
右旋-transposition 大动脉转位（d-TGA）伴完整室间隔的限制型卵圆孔（FO）可能导致出生后数小时内严重危及生命的缺氧，使紧急球囊房间隔造口术（BAS）成为必然。这些情况下，对限制型 FO 进行可靠的产前预测至关重要。然而，目前的产前超声心动图标志物预测价值较低，并且产前预测经常会导致部分新生儿出现致命后果。在本研究中，我们描述了我们的经验，并旨在确定 BAS 的可靠预测标志物。
我们纳入了 2010 年至 2022 年间在两个德国三级转诊中心诊断和分娩的 45 例孤立性 d-TGA 胎儿。纳入标准是：有先前的产前超声报告，有存储的超声心动图视频和静态图像，这些图像必须在分娩前最后 14 天内获得，并且具有足够的质量进行回顾性重新分析。我们回顾性评估了心脏参数及其预测价值。
在 45 例 d-TGA 胎儿中，22 例新生儿在产后出现限制型 FO，并在出生后 24 小时内需要紧急 BAS。相比之下，23 例新生儿 FO 解剖正常，但其中 4 例尽管 FO 解剖正常，但仍出现了不理想的房间隔混合，迅速出现缺氧，也需要紧急 BAS（“不良混合器”）。总体而言，26 例（58%）新生儿需要紧急 BAS，而 19 例（42%）获得了良好的氧饱和度，未进行紧急 BAS。在先前的产前超声报告中，有 11 例（50%的敏感性）正确预测了限制型 FO 伴随后的紧急 BAS，而有 19 例（83%的特异性）正确预测了正常 FO 解剖。在对存储的视频和图像进行当前的重新分析后，我们确定了三个限制型 FO 的高度显著标志物：FO 直径<7mm（p<0.01）、固定（p=0.035）和可移动（p=0.014）FO 瓣。限制型 FO 时，肺静脉的最大收缩期血流速度也显著增加（p=0.021），但无法确定可靠预测限制型 FO 的截断值。如果应用上述标志物，则所有 22 例限制型 FO 和所有 23 例正常 FO 解剖的病例都可以正确预测（100%的阳性预测值）。所有 22 例限制型 FO 的紧急 BAS 预测也都成功（100%的 PPV），但在 23 例正确预测正常 FO 的病例中自然会失败（“不良混合器”）（82.6%的阴性预测值）。
准确评估 FO 大小和 FO 瓣运动可以可靠地预测产后限制型和正常 FO 解剖。预测紧急 BAS 的可能性在所有限制型 FO 胎儿中也可靠地成功，但无法识别一小部分尽管 FO 解剖正常但仍需要紧急 BAS 的胎儿，因为无法预测足够的产后房间隔混合能力。因此，所有产前诊断为 d-TGA 的胎儿均应在具有心脏导管备用的三级中心分娩，以便在出生后 24 小时内进行 BAS。
