Hogan Whitnee J, Grinenco Sofia, Armstrong Aimee, Devlieger Roland, Dangel Joanna, Ferrer Queralt, Frommelt Michele, Galindo Alberto, Gardiner Helena, Gelehrter Sarah, Herberg Ulrike, Howley Lisa, Jaeggi Edgar, Miranda Joana, Morris Shaine A, Oepkes Dick, Pedra Simone, Peterson Renuka, Sholler Gary, Simpson John, Strainic James, Vigneswarran Trisha V, Wacker-Gussmann Annette, Moon-Grady Anita J
University of California-San Francisco, San Francisco, California, USA,
Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.
Fetal Diagn Ther. 2020 Jul 7:1-9. doi: 10.1159/000508045.
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.
Describe fetal and maternal characteristics of those being evaluated for FCI, including pregnancy/neonatal outcome data using the International Fetal Cardiac Intervention Registry (IFCIR).
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.
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%).
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.
对于室间隔完整的肺动脉闭锁（PAIVS）和重度肺动脉狭窄（PS），已开展侵入性胎儿心脏介入治疗（FCI），有小型单机构系列报道了其技术和生理方面的成功。我们展示了首个多中心经验。
描述接受FCI评估者的胎儿和母体特征，包括使用国际胎儿心脏介入注册中心（IFCIR）的妊娠/新生儿结局数据。
我们查询了IFCIR中2001年1月至2018年4月评估的PAIVS/PS病例，并回顾了母体/胎儿特征、手术细节、妊娠和新生儿结局。使用标准描述性统计方法分析数据。
在注册中心的84对母体/胎儿中，58例在孕龄中位数为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（TV）大小、TV反流和肺动脉瓣通畅性方面特征各异。55%的病例出现胎儿并发症，包括7例死亡和2例胎儿延迟丢失。在FCI成功的病例中，从介入到出生，TV的绝对测量值每周增加0.32（±0.17）mm。在60例已知结局的活产儿中，FCI成功后具有双心室循环的比例更高（87%对43%）。
我们的数据表明胎儿治疗PAIVS/PS可能有益，尽管技术上不成功的手术率和与手术相关的并发症，包括胎儿丢失率都很高。FCI标准差异极大，使得与未干预患者直接比较具有挑战性且可能存在偏差。需要为PAIVS/PS胎儿制定更统一的FCI标准，以避免不必要的手术，仅让最有可能受益的胎儿接受手术，并能够与未干预患者进行比较。
