Abstract：
OBJECTIVE: To summarize echocardiographic characteristics of the anatomy and hemodynamic and clinical outcomes in fetuses with isolated pulmonary stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS).
METHODS: This was a single-center retrospective study of fetuses with isolated PS or PA/IVS. Echocardiographic variables and clinical outcomes after delivery were evaluated and compared.
RESULTS: Between 2016 and 2021, 115 livebirths with isolated PS or PA/IVS were included. Proportion of fetuses with mild, moderate and critical PS and PA/IVS was 41.7 %, 18.3 %, 26.1 % and 13.9 %. Fetuses with more severe PS had worse anatomic and hemodynamic profiles. Specifically, the cardiothoracic ratio, pulmonary valve (PV) velocity, degree and velocity of tricuspid regurgitation increased as PS severity increased; and the pulmonary artery/aorta ratio, right ventricle/left ventricle long-axis (TV/MV) ratio, tricuspid valve/mitral valve annulus (TV/MV) ratio, and tricuspid valve inflow duration/cardiac cycle ratio decreased as PS severity increased (P <0.001 for all). PV velocity ≥2 m/s predicted PV pressure ≥40 mm Hg after delivery, with an AUC of 0.81; TV/MV ratio combined with RV/LV ratio predicted clinical outcomes, with an AUC of 0.88. Live births with more severe PS had higher mortality rate (mild 0 vs. moderate 0 vs. critical 11 % vs. PA-IVS 36 %) and lower rate of developing bi-ventricles (mild 100 % vs. moderate 95 % vs. critical 89 % vs. PA-IVS 36 %).
CONCLUSIONS: Findings of this study help better understand the anatomy and hemodynamic and clinical outcomes in fetuses with isolated PS or PA/IVS, which could have implications for prenatal counseling and prediction of fetal outcome.
METHODS: This was a single-center retrospective study of fetuses with isolated PS or PA/IVS. Echocardiographic variables and clinical outcomes after delivery were evaluated and compared.
RESULTS: Between 2016 and 2021, 115 livebirths with isolated PS or PA/IVS were included. Proportion of fetuses with mild, moderate and critical PS and PA/IVS was 41.7 %, 18.3 %, 26.1 % and 13.9 %. Fetuses with more severe PS had worse anatomic and hemodynamic profiles. Specifically, the cardiothoracic ratio, pulmonary valve (PV) velocity, degree and velocity of tricuspid regurgitation increased as PS severity increased; and the pulmonary artery/aorta ratio, right ventricle/left ventricle long-axis (TV/MV) ratio, tricuspid valve/mitral valve annulus (TV/MV) ratio, and tricuspid valve inflow duration/cardiac cycle ratio decreased as PS severity increased (P <0.001 for all). PV velocity ≥2 m/s predicted PV pressure ≥40 mm Hg after delivery, with an AUC of 0.81; TV/MV ratio combined with RV/LV ratio predicted clinical outcomes, with an AUC of 0.88. Live births with more severe PS had higher mortality rate (mild 0 vs. moderate 0 vs. critical 11 % vs. PA-IVS 36 %) and lower rate of developing bi-ventricles (mild 100 % vs. moderate 95 % vs. critical 89 % vs. PA-IVS 36 %).
CONCLUSIONS: Findings of this study help better understand the anatomy and hemodynamic and clinical outcomes in fetuses with isolated PS or PA/IVS, which could have implications for prenatal counseling and prediction of fetal outcome.
摘要：
目的：总结孤立性肺动脉狭窄（PS）或室间隔完整的肺动脉闭锁（PA/IVS）胎儿的解剖和血流动力学特征以及临床结局的超声心动图特征。
方法：这是一项单中心回顾性研究，研究对象是分离的PS或PA/IVS胎儿。评估并比较分娩后的超声心动图变量和临床结局。
结果：在2016年至2021年之间，纳入了115例分离PS或PA/IVS的活产。轻度胎儿的比例，中度和临界PS和PA/IVS为41.7%，18.3%,26.1%和13.9%。PS较严重的胎儿的解剖和血液动力学特征较差。具体来说,心胸比率,肺动脉瓣（PV）速度，随着PS严重程度的增加，三尖瓣反流的程度和速度增加；肺动脉/主动脉比值，右心室/左心室长轴（TV/MV）比，三尖瓣/二尖瓣环(TV/MV)比值,随着PS严重程度的增加，三尖瓣流入持续时间/心动周期比值降低（全部P<0.001）。PV速度≥2m/s，交付后预测PV压力≥40mmHg，AUC为0.81;TV/MV比值结合RV/LV比值预测临床结局,AUC为0.88。PS更严重的活产婴儿死亡率更高(轻度0vs.温和0vs.临界11%与PA-IVS36％）和较低的双心室发育率（轻度100％与适度的95%与临界89%与PA-IVS36%）。
结论：这项研究的结果有助于更好地了解患有孤立性PS或PA/IVS的胎儿的解剖结构和血流动力学以及临床结局。这可能对产前咨询和预测胎儿结局有影响。
方法：这是一项单中心回顾性研究，研究对象是分离的PS或PA/IVS胎儿。评估并比较分娩后的超声心动图变量和临床结局。
结果：在2016年至2021年之间，纳入了115例分离PS或PA/IVS的活产。轻度胎儿的比例，中度和临界PS和PA/IVS为41.7%，18.3%,26.1%和13.9%。PS较严重的胎儿的解剖和血液动力学特征较差。具体来说,心胸比率,肺动脉瓣（PV）速度，随着PS严重程度的增加，三尖瓣反流的程度和速度增加；肺动脉/主动脉比值，右心室/左心室长轴（TV/MV）比，三尖瓣/二尖瓣环(TV/MV)比值,随着PS严重程度的增加，三尖瓣流入持续时间/心动周期比值降低（全部P<0.001）。PV速度≥2m/s，交付后预测PV压力≥40mmHg，AUC为0.81;TV/MV比值结合RV/LV比值预测临床结局,AUC为0.88。PS更严重的活产婴儿死亡率更高(轻度0vs.温和0vs.临界11%与PA-IVS36％）和较低的双心室发育率（轻度100％与适度的95%与临界89%与PA-IVS36%）。
结论：这项研究的结果有助于更好地了解患有孤立性PS或PA/IVS的胎儿的解剖结构和血流动力学以及临床结局。这可能对产前咨询和预测胎儿结局有影响。
