Supplementary Material for: Fetal Cardiac Intervention for Pulmonary Atresia with Intact Ventricular Septum: International Fetal Cardiac Intervention Registry

<b><i>Introduction:</i></b> Invasive fetal cardiac intervention (FCI) for pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (PS) has been performed with small single-institution series reporting technical and physiological success. We present the first multicenter experience. <b><i>Objectives:</i></b> Describe fetal and maternal characteristics of those being evaluated for FCI, including pregnancy/neonatal outcome data using the International Fetal Cardiac Intervention Registry (IFCIR). <b><i>Methods:</i></b> We queried the IFCIR for PAIVS/PS cases evaluated from January 2001 to April 2018 and reviewed maternal/fetal characteristics, procedural details, pregnancy and neonatal outcomes. Data were analyzed using standard descriptive statistics. <b><i>Results:</i></b> Of the 84 maternal/fetal dyads in the registry, 58 underwent pulmonary valvuloplasty at a median gestational age of 26.1 (21.9–31.0) weeks. Characteristics of fetuses undergoing FCI varied in terms of tricuspid valve (TV) size, TV regurgitation, and pulmonary valve patency. There were fetal complications in 55% of cases, including 7 deaths and 2 delayed fetal losses. Among those who underwent successful FCI, the absolute measurement of the TV increased by 0.32 (±0.17) mm/week from intervention to birth. Among 60 liveborn with known outcome, there was a higher percentage having a biventricular circulation following successful FCI (87 vs. 43%). <b><i>Conclusions:</i></b> Our data suggest a possible benefit to fetal therapy for PAIVS/PS, though rates of technically unsuccessful procedures and procedure-related complications, including fetal loss were substantial. FCI criteria are extremely variable, making direct comparison to nonintervention patients challenging and potentially biased. More uniform FCI criteria for fetuses with PAIVS/PS are needed to avoid unnecessary procedures, expose only fetuses most likely to sustain a benefit, and to enable comparisons to be made with nonintervention patients.

<b><i>引言：</i></b>针对室间隔完整型肺动脉闭锁（pulmonary atresia with intact ventricular septum, PAIVS）与重度肺动脉狭窄（critical pulmonary stenosis, PS）的侵入性胎儿心脏介入术（fetal cardiac intervention, FCI）此前仅在少数单中心研究中开展，相关报道仅涉及技术与生理学层面的成功案例。本研究首次呈现多中心的实践经验。
<b><i>研究目的：</i></b>本研究依托国际胎儿心脏介入注册研究（International Fetal Cardiac Intervention Registry, IFCIR），旨在描述接受胎儿心脏介入术评估的胎儿与母体特征，并纳入妊娠及新生儿结局数据。
<b><i>研究方法：</i></b>我们检索了IFCIR数据库中2001年1月至2018年4月期间纳入评估的PAIVS/PS病例，回顾分析了母体与胎儿特征、手术操作细节、妊娠及新生儿结局，并采用标准描述性统计学方法对数据进行分析。
<b><i>研究结果：</i></b>该注册研究中共纳入84组母胎对子病例，其中58例在中位胎龄26.1（21.9~31.0）周时接受了肺动脉瓣成形术。接受FCI的胎儿在三尖瓣（tricuspid valve, TV）尺寸、三尖瓣反流及肺动脉瓣通畅性方面存在异质性。55%的病例出现胎儿并发症，包括7例胎儿死亡及2例迟发性胎儿丢失。在成功接受FCI的病例中，从手术至分娩期间，三尖瓣的绝对尺寸每周增加0.32±0.17 mm。在60例结局明确的活产儿中，成功接受FCI者后续实现双心室循环的比例更高（87% vs 43%）。
<b><i>研究结论：</i></b>本研究数据提示，针对PAIVS/PS的胎儿治疗或可带来获益，但手术技术失败率及与手术相关的并发症（包括胎儿丢失）仍处于较高水平。目前FCI的入选标准差异极大，这使得与未接受干预的患者进行直接对比存在难度，且可能引入偏倚。未来亟需建立针对PAIVS/PS胎儿的统一FCI入选标准，以避免不必要的手术操作，仅将最有可能获益的胎儿纳入干预范围，并实现与未干预患者的对照研究。