Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, Medical Faculty of the Johannes Kepler University, Linz, Austria.
Institute of Prenatal Medicine, Kepler University Hospital, Medical Faculty of the Johannes Kepler University, Linz, Austria.
Ultrasound Obstet Gynecol. 2022 May;59(5):633-641. doi: 10.1002/uog.24792. Epub 2022 Apr 11.
To review our experience with fetal aortic valvuloplasty (FAV) in fetuses with critical aortic stenosis (CAS) and evolving hypoplastic left heart syndrome (eHLHS), including short- and medium-term postnatal outcome, and to refine selection criteria for FAV by identifying preprocedural predictors of biventricular (BV) outcome.
This was a retrospective review of all fetuses with CAS and eHLHS undergoing FAV at our center between December 2001 and September 2020. Echocardiograms and patient charts were analyzed for pre-FAV ventricular and valvular dimensions and hemodynamics and for postnatal procedures and outcomes. The primary endpoints were type of circulation 28 days after birth and at 1 year of age. Classification and regression-tree analysis was performed to investigate the predictive capacity of pre-FAV parameters for BV circulation at 1 year of age.
During the study period, 103 fetuses underwent 125 FAVs at our center, of which 87.4% had a technically successful procedure. Technical success per fetus was higher in the more recent period (from 2014) than in the earlier period (96.2% (51/53) vs 78.0% (39/50); P = 0.0068). Eighty fetuses were liveborn after successful intervention and received further treatment. BV outcome at 1 year of age was achieved in 55% of liveborn patients in our cohort after successful FAV, which is significantly higher than the BV-outcome rate (23.7%) in a previously published natural history cohort fulfilling the same criteria for eHLHS (P = 0.0015). Decision-tree analysis based on the ratio of right to left ventricular (RV/LV) length combined with LV pressure (mitral valve regurgitation maximum velocity (MR-Vmax)) had a sensitivity of 96.97% and a specificity of 94.44% for predicting BV outcome without signs of pulmonary arterial hypertension at 1 year of age. The highest probability for a BV outcome was reached for fetuses with a pre-FAV RV/LV length ratio of < 1.094 (96.4%) and for those fetuses with a RV/LV length ratio ≥ 1.094 to < 1.135 combined with a MR-Vmax of ≥ 3.14 m/s (100%).
FAV could be performed with high success rates and an acceptable risk with improving results after a learning curve. Pre-FAV RV/LV length ratio combined with LV pressure estimates were able to predict a successful BV outcome at 1 year of age with high sensitivity and specificity. © 2022 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
回顾我们在患有严重主动脉瓣狭窄(critical aortic stenosis,CAS)和进行性左心发育不良综合征(hypoplastic left heart syndrome,HLHS)的胎儿中进行胎儿主动脉瓣成形术(fetal aortic valvuloplasty,FAV)的经验,包括短期和中期的新生儿预后,并通过识别术前预测双心室(biventricular,BV)结局的指标来完善 FAV 的选择标准。
这是一项对 2001 年 12 月至 2020 年 9 月期间在我们中心接受 FAV 的所有患有 CAS 和 HLHS 的胎儿的回顾性研究。对胎儿的术前心室和瓣膜尺寸以及血流动力学以及产后程序和结局进行了超声心动图和患者图表分析。主要终点是出生后 28 天和 1 岁时的循环类型。分类回归树分析用于研究术前参数对 1 岁时 BV 循环的预测能力。
在研究期间,103 例胎儿在我们中心接受了 125 次 FAV,其中 87.4%的手术技术成功。与早期相比,近期(2014 年以后)的技术成功率更高(96.2%(51/53)比 78.0%(39/50);P=0.0068)。80 例成功干预后的胎儿存活并接受了进一步治疗。在我们的队列中,55%的活产婴儿在成功进行 FAV 后达到了 1 岁时的 BV 结局,这明显高于符合 eHLHS 相同标准的先前发表的自然病史队列的 BV 结局发生率(23.7%)(P=0.0015)。基于右心室与左心室(RV/LV)长度比与左心室压力(二尖瓣反流最大速度(MR-Vmax))的决策树分析,在预测 1 岁时无肺动脉高压的 BV 结局方面,具有 96.97%的敏感性和 94.44%的特异性。对于 RV/LV 长度比<1.094 的胎儿(96.4%)和 RV/LV 长度比≥1.094 至<1.135 并结合 MR-Vmax≥3.14m/s(100%)的胎儿,达到 BV 结局的可能性最高。
FAV 可以在高成功率和可接受的风险下进行,学习曲线后结果改善。术前 RV/LV 长度比与 LV 压力估计相结合能够以高灵敏度和特异性预测 1 岁时的成功 BV 结局。© 2022 作者。超声在妇产科由 John Wiley & Sons Ltd 代表国际妇产科超声学会出版。