<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.

Objectives Congenital diaphragmatic hernia (CDH) can cause a significant mass effect in the fetal thorax, displacing the heart into the opposite hemithorax. In left‐sided CDH (L‐CDH), this is associated with smaller left‐sided cardiac structures and reduced left‐ventricular cardiac output (LVCO). The effect of these physiologic changes on cerebral blood flow is not well understood. We sought to describe the middle cerebral artery (MCA) pulsatility index (PI), a measure of cerebrovascular impedance, in fetuses with L‐CDH and those with right‐sided CDH (R‐CDH) compared with unaffected fetuses, and the relationship between MCA‐PI and LVCO. We hypothesized that MCA‐PI would be lower in fetuses with L‐CDH and similar in those with R‐CDH compared to controls, and that MCA‐PI would be correlated with LVCO. Methods We identified all fetuses with CDH evaluated at The University of California San Francisco, San Francisco, CA, USA from 2011 to 2018. Fetal echocardiograms and ultrasound scans were reviewed. Umbilical artery and MCA Doppler examinations were assessed to calculate pulsatility indices. Ventricular outputs were calculated using Doppler‐derived stroke volume and fetal heart rate. Lung‐to‐head ratio (LHR), estimated fetal weight, biparietal diameter (BPD) and head circumference (HC) were obtained from fetal sonograms. Measurements in fetuses with CDH, according to the side of the defect, were compared with those in unaffected, gestational age‐matched controls. A subset of CDH survivors had available data on neurodevelopmental outcome, as assessed using the Bayley Scales of Infant Development, 3rd edition. Results A total of 64 fetuses with CDH (L‐CDH, n = 53; R‐CDH, n = 11) comprised the study groups, with 27 unaffected fetuses serving as controls. Mean gestational age at evaluation was similar between the three groups. Compared to controls, fetuses with L‐CDH had significantly lower LVCO expressed as a percentage of combined cardiac output (CCO) (32%; 95% CI, 29–35% vs 38%; 95% CI, 33–42%; P = 0.04) and lower MCA‐PI Z‐score (−1.3; 95% CI, −1.7 to −1.0 vs 0.08; 95% CI, −0.5 to 0.6; P < 0.001), while they did not differ between the R‐CDH group and controls. There was a strong positive association between LVCO as a percentage of CCO and MCA‐PI Z‐score in the overall cohort of CDH and control fetuses (P = 0.01). BPD and HC were similar between the three groups. At neurodevelopmental follow‐up, mean cognitive, motor and language scores in the CDH group were within 1 SD of those in the general population. Conclusion MCA‐PI values are significantly lower in fetuses with L‐CDH as compared to controls, and lower LVCO was correlated with lower MCA vascular impedance. The neurodevelopmental effect of changes in MCA‐PI in response to decreased LVCO is unknown, although, on average, CDH survivors had neurodevelopmental scores in the normal range. This may reflect a fetal compensatory mechanism in response to diminished antegrade cerebral blood flow. © 2020 International Society of Ultrasound in Obstetrics and Gynecol...

What are the novel findings of this work?Our findings reveal that differences in underlying fetal cardiovascular physiology lead to varying cerebrovascular response to maternal hyperoxygenation and that there are impairments in cerebral autoregulation in certain congenital heart disease groups. What are the clinical implications of this work?Given the recent interest in studying maternal hyperoxygenation as a therapeutic tool to optimize brain development in fetuses with congenital heart disease, our findings provide important physiologic data that should be incorporated in future clinical trials.

Neonates with congenital heart disease demonstrate different neurobehavioral performance than typically developing neonates. Evaluation of neonatal neurobehavioral performance provides an opportunity to identify neurodevelopmental variability early. Identification of neurobehavioral performance variability allows targeted interactions and therapy.