Objective: To investigate the echocardiographic features, consistency of diagnosis between fetal and postnatal periods and postnatal clinical outcomes of fetal pulmonary valve stenosis (PS) with different degrees. Methods: This study was a retrospective cohort study comprising 108 cases of fetal PS diagnosed during the fetal period and followed up postnatally at Xinhua Hospital, Shanghai Jiaotong University School of Medicine from November 2012 to February 2023. Echocardiographic characteristics, including morphological and hemodynamic features were collected for all fetuses who were then were followed up to at least 6 months after birth. One-way analysis of variance and Kruskal-Wallis test were used to compare the differences in the echocardiographic features among fetuses with different degrees of PS. Subsequently, McNemar test was used to assess the consistency of diagnosis between the fetal and postnatal periods. Furthermore, Logistic regression analysis was applied to explore the risk factors for neonatal intervention in fetuses with moderate PS and the receiver operating characteristic (ROC) curve was utilized to ascertain the optimal cut-off value for continuous variables. Results: The age of the mothers of the 108 fetuses at the initial assessment was (30.8±4.0) years, and the gestational age was 26.5 (24.6, 30.0) weeks. The fetuses were categorized into mild (17 cases), moderate (49 cases), and severe groups (42 cases) based on the initial echocardiographic features. Mild PS was characterized by valve thickening and hyperechogenicity combined with systolic flow acceleration or dilation of main pulmonary artery. Moderate PS exhibited both restricted valve motion and a colorful blood flow pattern at the valve orifice. The peak flow velocities of fetuses with moderate and critical PS were notably higher than those in the mild group ((2.66±0.86) and (2.77±1.30) vs. (1.43±0.59)m/s, F=14.52, P<0.001). In critical PS, all cases showed retrograde ductal flow, with a significantly higher proportion of a small right ventricle compared to the mild and moderate PS (42.9% (18/42) vs. 0 and 2.0% (1/49), χ2=31.73, P<0.001). The proportion of severe tricuspid regurgitation was also higher (35.7% (15/42) vs. 0 and 10.2% (5/49), χ2=36.94, P<0.001). Compared to mild and severe PS, the consistency of diagnosis between fetal and postnatal periods in moderate PS was lower (40.8% (20/49) vs.13/17 and 80.3% (35/42), χ2=12.45, P=0.006). The systolic flow velocity was identified as an independent risk factor for neonatal intervention in fetuses with moderate PS (OR=7.21, 95%CI2.11-24.62). A flow velocity of ≥2.18 m/s in second trimester and ≥3.15 m/s in third trimester indicated the necessity of neonatal intervention for fetal moderate PS. Among the 108 fetuses, 68 underwent surgical intervention and all survived. Additionally, 39 fetuses were regularly followed up. A sole non-surgical fatality occurred, leading to a 6-month survival rate of 99.1% (107/108). Conclusions: Various degrees of fetal PS demonstrate distinctive morphological and hemodynamic alterations in echocardiography. The disparity in severity between the postnatal and fetal stages requires ongoing monitoring for fetal PS. The prognosis for fetal PS is generally favorable.
目的: 探讨胎儿不同程度肺动脉瓣狭窄(PS)的超声心动图特征、胎儿期与生后诊断一致性及生后结局。 方法: 回顾性队列研究。选择2012年11月至2023年2月于上海交通大学医学院附属新华医院行胎儿期诊断并生后随访的108例PS胎儿为研究对象。收集PS胎儿形态学及血流动力学等超声心动图特征,且所有胎儿至少随访至生后6月龄。采用单因素方差分析和Kruskal-Wallis检验等比较不同程度PS胎儿的超声心动图特征差异。采用McNemar检验分析胎儿期与生后诊断一致性。应用Logistic回归分析中度PS胎儿需新生儿期干预的危险因素,并利用受试者工作特征(ROC)曲线探索连续变量的最佳截断值。 结果: 108例胎儿首诊时孕母年龄(30.8±4.0)岁,胎龄26.5(24.6,30.0)周。根据首诊超声心动图特征将胎儿PS分为轻度(17例)、中度(49例)和危重型(42例)。轻度PS胎儿超声心动图特征包括瓣膜增厚、回声增强伴流速增快或MPA扩张,中度PS同时伴瓣膜开放活动受限,瓣口呈五彩血流。中度和危重型PS胎儿过瓣流速明显快于轻度组[(2.66±0.86)和(2.77±1.30)比(1.43±0.59)m/s,F=14.52,P<0.001]。危重型PS均为动脉导管血流逆灌注,其右心室偏小比例明显高于轻度和中度组[42.9%(18/42)比0和2.0%(1/49),χ2=31.73,P<0.001],且重度三尖瓣反流的比例也更高[35.7%(15/42)比0和10.2%(5/49),χ2=36.94,P<0.001]。与轻度和危重型PS胎儿相比,中度PS胎儿期与生后诊断一致率更低[40.8%(20/49)比13/17和83.3%(35/42),χ2=12.45,P=0.006]。过瓣流速是中度PS胎儿生后需新生儿期干预的独立危险因素(OR=7.21,95%CI 2.11~24.62),且胎儿中期过瓣流速≥2.18 m/s、胎儿晚期过瓣流速≥3.15 m/s提示中度PS胎儿需新生儿期干预。108例胎儿生后68例接受手术治疗后均存活,39例定期随访,1例未手术死亡,生后6个月存活率为99.1%(107/108)。 结论: 不同程度的胎儿PS在超声心动图中表现出特征性的形态学和血流动力学改变。生后严重程度与胎儿期差异较大,应对胎儿PS行定期随访。胎儿PS的整体预后较好。.