National registry of thoracoamniotic shunting using a double-basket catheter: A post-marketing surveillance registry of 295 patients with fetal hydrothorax.

OBJECTIVE: We report on the 1-year outcomes of fetuses who underwent thoracoamniotic shunting (TAS) with a double-basket catheter for fetal hydrothorax (FHT) using a national post-marketing surveillance registry. METHODS: This surveillance study was conducted for cases enrolled between 2011 and 2019. Preoperative, operative, and postoperative findings and outcomes had to be reported through the national surveillance website. RESULTS: Forty-three institutes enrolled 295 patients. In 60 patients, associated abnormalities were diagnosed after birth. Among the 235 patients with isolated hydrothorax, the survival rate at 1 year of age was 70.5%. Among these patients, 89% did not require oxygen or other respiratory support. The 1-year survival rate in complicated cases was 27%. Trisomy 21 was the most common associated problem (40%). Catheter associated problems, including dislocation (25%), obstruction (24%), and preterm rupture of the membranes (6%) were observed, but there were no severe maternal complications. TAS before 29.5 weeks of gestation (odds ratio [OR] 0.16) and skin edema and ascites (OR 0.06) were risk factors for death at one year, whereas appropriate shunt location for >28 days (OR 4.2) was a protective factor. CONCLUSION: We report a favorable survival rate in fetuses with isolated FHT treated with this double-basket catheter.

Fetal open spinal dysraphism repair through a mini-hysterotomy: Influence of gestational age at surgery on children's ability to walk.

OBJECTIVE: To analyze the impact of gestational age (GA) at the time of fetal open spinal dysraphism (OSD) repair through a mini-hysterotomy on the ability of children to walk. METHODS: Children who underwent in utero repair of OSD and had formal neurological assessment after 2.5 years of age were compared regarding their ability to walk in relation to pre-surgical predictors. RESULTS: Sixty-nine children fulfilled the inclusion criteria. Among them, 63.7% (44/69) were able to walk with or without orthesis. Fetal OSD correction performed earlier in gestation (from 19.7 to 26.9 weeks) was associated with a higher probability of walking with or without orthesis (p = 0.033). The median GA at delivery was 35.3 weeks. Multivariate binary logistic regression showed that the upper anatomical level of the OSD ( L5) (p < 0.004; OR: 10.31 [95% CI: 2.07-51.28]) and GA at the time of fetal surgery (p = 0.026; OR = 0.68 [95% CI: 0.48-0.95]) were independent predictors of the postnatal ability to walk with or without orthesis. CONCLUSION: Fetuses with OSD who were operated on earlier in pregnancy (range: 19.7-26.9 weeks), were more likely to walk with or without orthesis.

Serial neurosonography in fetuses with congenital heart defects shows mild delays in cortical development.

INTRODUCTION: Neurodevelopmental delay is more common in children born with congenital heart defects (CHD), even with optimal perinatal and peri-operative care. It is hypothesized that fetuses with CHD are prone to neurological impairment in utero due to their cardiac defect, possibly leading to delayed cortical development. METHODS: Cerebral cortical maturation was assessed with advanced neurosonographic examinations every 4 weeks in fetuses with CHD and compared to control fetuses. Five different primary fissures and four areas were scored (ranging 0-5) by blinded examiners using a cortical maturation scheme. RESULTS: Cortical staging was assessed in 574 ultrasound examinations in 85 CHD fetuses and 61 controls. Small differences in grading were seen in Sylvian and cingulate fissures. (Sylvian fissure: -0.12 grade, 95% CI (-0.23; -0.01) p = 0.05, cingulate fissure: -0.24 grade, 95% CI (-0.38; -0.10) p = <0.001. Other cortical areas showed normal maturation as compared to control fetuses. CONCLUSION: Small differences were seen in three of the nine analyzed cortical areas in CHD fetuses, in contrast to previous reports on progressive third-trimester delay. The clinical implications of the small differences however, remain unknown.

Fetal therapy using rapamycin for a rapidly enlarging, obstructive, cervical lymphatic malformation: a case report.

WHAT'S ALREADY KNOWN ABOUT THIS TOPIC?: Fetal lymphatic malformations (LMs) can be detected on prenatal ultrasound and until recently, therapeutic options were limited. Recently the mammalian target of rapamycin inhibitor rapamycin has emerged as a safe, effective therapy for children with LMs and multiple studies have demonstrated improved efficacy if started early. WHAT DOES THIS STUDY ADD?: We report the first in-utero therapy with rapamycin for a rapidly enlarging, obstructive, fetal cervical LM. Fetal therapy with rapamycin was safe and effective in managing this severe malformation, despite rapamycin being started only in the last 6.5 weeks of pregnancy. We speculate that had rapamycin been commenced earlier, the reduction in mass size might have been even greater.

Fetal Cystoscopy and Vesicoamniotic Shunting in Lower Urinary Tract Obstruction: Long-Term Outcome and Current Technical Limitations.

BACKGROUND: In utero therapeutic approaches for lower urinary tract obstruction (LUTO) have been developed to salvage the fetal kidney function. OBJECTIVE: The aim of this work was to report the long-term survival, nephrological, and urological outcome of children treated prenatally for LUTO using operative fetal cystoscopy (FC) and vesicoamniotic shunting (VAS) or both. METHODS: A retrospective study of 48 procedures (23 FC, 25 VAS) was performed on 33 patients (between 2008 and 2018). Reviewed data included prenatal management and clinical follow-up by a pediatric nephrologist and a pediatric urologist. Both intention-to-treat and per-protocol analyses were conducted. RESULTS: The median follow-up was 3.6 years (0.5-7) for FC and 2.5 years (1.1-5.1) for VAS. There was no difference between FC and VAS in terms of survival (92 vs. 83%, p = 1), complication rate (74 vs. 92%, p = 0.88), or chronic kidney disease (58 vs. 50%, p = 1). The number of procedures was higher in the VAS group: 1.7 (1-3) versus 1.1 (1-2), p = 0.01. With a 30% rate of technical failure, FC added diagnostic value in 3 out of 21 cases. CONCLUSIONS: No difference was found between FC and VAS regarding survival, long-term kidney function, or urological outcome. Despite overly optimistic reports on FC, it lacks reproducibility due to posterior-urethra inadequate visualization and inappropriate instrumentation.

Fetus in Fetu: Lessons Learned from a Large Multicenter Cohort Study.

INTRODUCTION: Fetus in fetu (FIF) is an extremely rare condition of abnormal twinning during embryogenesis. Most publications are single case reports. We describe the combined experience of four large tertiary referral centers with FIF which were not previously reported or published, and thereby draw conclusions to establish criteria for the workup, diagnosis, and management including intraoperative risk. MATERIALS AND METHODS: A survey was forwarded to a national pediatric surgery group which includes members from all pediatric surgery centers in the country enquiring about unpublished cases of FIF encountered over a 20-year interval. The cohort was analyzed for age of presentation, type of presentation, diagnostic workup, surgical management, and outcome. RESULTS: From 1998 to 2018, a total of 10 FIF cases were included in the study. Mean age of presentation was 4 months. Computed tomography and ultrasound were the main preoperative diagnostic modality in our cohort. Resection of the mass was curative in nine cases. Two cases in which the FIF was in direct topographic proximity to the biliary tree suffered severe intraoperative or lethal postoperative complications. CONCLUSION: Complete excision of FIF is the treatment of choice and generally results in excellent long-term quality of life. Mortality is rare and may be associated with biliary involvement and retroperitoneal right upper quadrant location of the FIF tends to be associated with increased risk in excision, and there is also a possible association with the presence of immature elements in the pathology report.

Trend in ventricle size during pregnancy and its use for prediction of ventriculoperitoneal shunt in fetal open neural tube defect.

OBJECTIVES: Fetal surgery for repair of open neural tube defect (ONTD) typically results in decreased need for a ventriculoperitoneal shunt (VPS). Our objectives were to determine the trend in ventricle size (VS) during pregnancy and whether VS and change in VS, as assessed by ultrasound, were predictive of the need for VPS in pregnancy with ONTD. METHODS: This was a retrospective analysis of prospectively collected data of consecutive pregnancies with ONTD, evaluated in a single center from January 2012 to May 2018. Two groups were identified: the first consisted of pregnancies that underwent in-utero repair (IUR) and the second those that had postnatal repair (PNR). Penalized B splines were used to determine the trend in VS, across 2-week gestational-age (GA) epochs, between 24 and 36 weeks of gestation. VS at each GA epoch and the change in VS between each GA epoch were compared between the IUR and PNR groups. To determine whether VS at any GA was predictive of VPS, receiver-operating-characteristics (ROC) curves were used and the optimal cut-off at each GA epoch was identified. Univariate analysis and multiple logistic regression were used for further analysis. RESULTS: ONTD was diagnosed in 110 fetuses, of whom 69 underwent IUR and 41 had PNR. Fetuses in the IUR group were more likely to have Chiari II malformation (100.0% vs 82.9%; P < 0.01), lower GA at delivery (34.9 ± 3.2 vs 37.1 ± 2.1 weeks; P < 0.01) and lower rates of VPS within the first year postpartum (36.2% vs 61.0%; P = 0.02) compared with the PNR group. In both groups, VS increased steadily with GA from the initial evaluation to delivery. In the IUR group, there was a significant change in VS between the 24 + 0 to 25 + 6-week and the 26 + 0 to 27 + 6-week epochs (2.3 (95% CI, 0.4-4.1) mm; P = 0.02). There was a positive trend in the change in VS at later GAs, but this was not significant. Although there was no significant change in VS in the PNR group before 30 weeks, there was a positive trend after that time. On multivariate analysis, each week of advancing GA was associated with a mean increase of 0.74 mm in VS (P < 0.0001) in both groups. VS was not associated with the level or type of lesion, but presence of Chiari II malformation was associated with a mean increase of 5.88 mm (P < 0.0001) in VS in both the IUR and PNR groups. VS was modestly predictive of need for VPS in both groups, with area under ROC curves between 0.68 and 0.76 at the different GA epochs. Change in VS between the first and last measurements was also modestly predictive of the need for VPS, with better performance in the PNR group. CONCLUSIONS: VS increased with advancing GA in all fetuses with ONTD, although in the IUR group this increase occurred immediately after fetal surgery and in the PNR group it occurred after 30 weeks of gestation. In-utero surgery was associated with a decreased rate of VPS and was more predictive of need for VPS than was VS. Postnatal factors resulting in increased need for VPS in the PNR group need to be assessed further. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

The first national survey of anesthesia techniques for fetal therapies in Japan.

PURPOSE: The aim of this study was to survey the frequency of various anesthetic techniques used in the anesthetic management of both the mother and fetus during fetal therapies in Japan. METHODS: We sent a postal survey to the institutions with physicians who held membership of the Japan Society of Fetal Therapy to describe maternal and fetal anesthetic management during fetal therapies performed from January 2016 to March 2017. The therapies included were thoracoamniotic shunting (TAS), intrauterine transfusion (IUT), radiofrequency ablation (RFA), fetoscopic laser photocoagulation (FLP), fetoscopic endotracheal occlusion (FETO), and ex utero intrapartum treatment (EXIT). Survey respondents were asked to specify the standard anesthetic technique used in each of these procedures done during the study period. RESULTS: The most common anesthetic techniques used in each therapy were sedation/analgesia with local anesthesia in TAS (31%), local anesthesia alone in IUT (47%), neuraxial anesthesia in RFA (50%), FLP (66%) and FETO (100%), and general endotracheal anesthesia in EXIT. Fetal analgesia was utilized in 61% of TAS, 33% of IUT, 10% of RFA, 22% of FLP, 100% of FETO, and 50% of EXIT. In all fetal therapies, the most common route of administration for fetal anesthesia was maternal administration. CONCLUSION: In this first published description of the frequency of various anesthetic techniques used during fetal therapies in Japan, we found that anesthetic techniques varied depending on the degree of invasiveness to the mother and fetus. Fetal anesthesia was not always performed, and the most common route for fetal anesthesia was maternal administration.

Effect of Prenatal Repair of Myelomeningocele on Urological Outcomes at School Age.

PURPOSE: We investigated longer term urological outcomes in patients enrolled in the Management of Myelomeningocele Study (MOMS). MATERIALS AND METHODS: Women who participated in the original trial were asked for consent for followup for their child at age 6 years or older in a single comprehensive study visit to a MOMS center. Participating children underwent urological and radiologic procedures to provide objective evidence of current bladder functioning. Primary urological outcome was defined as any among need for clean intermittent catheterization, vesicostomy, urethral dilatation or augmentation cystoplasty. RESULTS: A total of 156 children were evaluated, with a mean age of 7.4 years. Overall 62% vs 87% in the prenatal and postnatal surgery groups, respectively, were placed on clean intermittent catheterization (RR 0.71, 95% CI 0.58-0.86, p <0.001). Voiding status was significantly different between the groups (p <0.001) as 24% in the prenatal group vs 4% in the postnatal group (RR 5.8, 95% CI 1.8-18.7) were reported to be voiding volitionally. Augmentation cystoplasty, vesicostomy and urethral dilation did not differ between the 2 groups. Aside from a larger post-void residual urodynamic catheterization volume, there were no other statistical differences in videourodynamic data or findings on renal/bladder ultrasound. CONCLUSIONS: Prenatal closure of myelomeningocele resulted in less reported clean intermittent catheterization at school age and the mechanism for this is unclear. Although most children are in diapers or on clean intermittent catheterization, parental reports showed children who underwent prenatal closure may be more likely to void volitionally than the postnatal group. Despite these findings, urological outcomes alone should not be the sole impetus to perform in utero closure in children with spina bifida.

Characteristics of referred patients with twin-twin transfusion syndrome who did not undergo fetal therapy.

INTRODUCTION: Abundant research has reported twin-twin transfusion syndrome (TTTS) outcomes following fetal therapy. Our research describes TTTS patients who did not undergo fetal therapy. METHODS: Records from TTTS pregnancies evaluated at 16 to 26 gestational weeks were reviewed between January 2006 and March 2017. The study population comprised subjects who did not undergo fetal therapy. Based on initial consultation, patients were grouped as nonsurgical vs surgical candidates. TTTS progression and perinatal outcomes were assessed. RESULTS: Of 734 TTTS patients evaluated, 68 (9.3%) did not undergo intervention. Of these, 62% were nonsurgical candidates and 38% were surgical candidates. Nonsurgical candidates were ineligible for treatment because of fetal demise or maternal factors (placental abruption, severe membrane separation, and preterm labor). Of surgical candidates, 11 underwent expectant management, eight elected pregnancy termination, and seven planned fetal intervention but had a complication before the procedure. TTTS progression occurred in 10 (15.2%) of 66 cases. Neonatal survival in 64 cases was as follows: in 41 (64%), no survivors; in 11 (17.2%), one survivor; and in 12 (18.8%), two survivors. CONCLUSION: Nine percent of referred TTTS patients did not undergo fetal therapy, with many ineligible because of morbidity between referral and consultation. Studies of TTTS should acknowledge this subgroup and circumstances leading to lack of treatment.

Amniotic membrane and placental histopathological findings after open and fetoscopic prenatal neural tube defect repair.

OBJECTIVES: To describe and compare placental and amniotic histology in women who underwent a fetoscopic myelomeningocele repair to those who underwent an open hysterotomy myelomeningocele repair. Also, we intended to compare findings from both prenatal repair groups to age-matched control pregnant patients. METHODS: Placental and membrane histopathology from 43 prenatally repaired spina bifida cases (17 fetoscopic and 26 open) and 18 healthy controls were retrospectively assessed. Quantitative assessment of histopathology included apoptosis count and maternal and fetal underperfusion scores. Qualitative assessment included the detection of pigmented macrophages and/or signs of placental/amniotic inflammation. Associations between the duration of surgery or the duration of CO2 insufflation and quantitative histological parameters were tested. RESULTS: Fetoscopic surgery cases did not show significant differences in any of the studied parameters when compared against controls. No differences were detected either when compared with open repaired cases, except for lower proportion of pigmented laden macrophages in the fetoscopic group (11.8% vs 61.5%, P < 0.01). No associations between the duration of surgery or the duration of CO2 exposure and any of the quantitative histological parameters were detected. CONCLUSIONS: These preliminary results support the lack of detrimental effects of the use of heated and humidified CO2 gas for uterine insufflation to fetal membranes and placenta.

Fetal myelomeningocele surgery: Only treating the tip of the iceberg.

OBJECTIVE: Fetal myelomeningocele (fMMC) surgery improves infant outcomes when compared with postnatal surgery. Surgical selection criteria and the option of pregnancy termination, however, limit the number of cases that are eligible for prenatal surgery. We aimed to quantify what proportion of cases could ultimately benefit from fetal therapy. METHODS: We retrospectively reviewed all cases of fMMC referred to a large tertiary care center over a 10-year period and assessed their eligibility for fetal surgery, pregnancy termination rates, and actual uptake of the surgery. RESULTS: Of 158 cases, 67 (42%) were ineligible for fetal surgery based on surgical exclusion criteria. Eleven fetuses (7%) had chromosomal anomalies, 10 of which (91%) had other anomalies on ultrasound. Thirty-four patients had a combination of maternal and fetal contraindications. Of the remaining 91 eligible cases (58%), 45 (49%) pregnancies were terminated, leaving only 46 (29% of initial 158 cases) as potential candidates for fetal repair. Actual uptake of fetal surgery was 15% (n = 14 of 91), but this increased after a national program was started. CONCLUSION: Only a minority of fMMC cases will ultimately undergo fetal surgery. These numbers support the centralization of care in expert centers.

Current Management of Congenital Pulmonary Airway Malformations: A "European Pediatric Surgeons' Association" Survey.

AIM: To define current management of congenital pulmonary airway malformation (CPAM). METHODS: A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire. MAIN RESULTS: Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%).Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively. CONCLUSION: Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.

Área maternofetal y neonatal - BCNatal / Maternal-fetal and neonatal area - BCNatal

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Fetal Aortic Valvuloplasty: Experience and Results of Two Tertiary Centers in Spain.

OBJECTIVE: Fetal aortic valvuloplasty (FAV) may avoid progression of critical aortic stenosis (CAS) to hypoplastic left ventricle, improving the options for biventricular circulation (BVC). We describe the results of FAV in 2 referral centers in Spain. METHODS: We analyzed all FAVs performed in the period 2007-2015. The selection of candidates, the technique, and postnatal management were made following an agreed protocol. A descriptive analysis of survival, type of circulation after birth, and complications was made, considering all deaths in the first 48 h after FAV as FAV-related. RESULTS: FAV was performed in 28 fetuses at a median gestational age (GA) of 23 weeks (range, 20-32). FAV was technically successful in 22 (78.6%), of whom 11 were born alive and with intention to treat. Eight (72.7%) resulted in BVC and 3 (27.3%) in univentricular circulation. The rate of FAV-related deaths was 32%. These patients underwent FAV earlier than live-born fetuses (median GA at FAV 22 weeks [range, 20.0-25.0] vs. 24.5 weeks [range, 21.0-32.0], respectively, p = 0.031). CONCLUSIONS: A significant proportion of fetuses with CAS who undergo technically successful FAV have BVC postnatally. However, FAV implies a high risk of fetal death, which highly depends on the GA at which this intervention is required.

Contemporary management of neonatal alloimmune thrombocytopenia: good outcomes in the intravenous immunoglobulin era: results from the Australian neonatal alloimmune thrombocytopenia registry.

OBJECTIVE: To describe the natural history, antenatal and postnatal therapy, and clinical outcomes of Australian patients with fetomaternal/neonatal alloimmune thrombocytopenia (NAIT) recorded in the Australian NAIT registry. METHODS: Analysis of registry data of Australian mothers treated antenatally for NAIT and any fetus/newborn with thrombocytopenia (TCP) and maternal human platelet antigen (HPA) antibodies. RESULTS: Ninety four potential cases (91 pregnancies; three twin pregnancies) were registered between December 2004 and September 2015 with 76 confirmed or treated as NAIT. NAIT was frequently unanticipated (44 cases, 58%), whilst 32 cases (42%) were anticipated due to personal or family history. In 70/76 cases, the diagnosis of NAIT was made based on HPA antibody results; anti-HPA-1a was most commonly detected (58/70, 82%), followed by anti-HPA-5b (5/70, 7%). Intracranial haemorrhage (ICH) was detected in seven cases (9%). Maternal antenatal therapy resulted in improved clinical outcomes. For antenatally treated cases, whilst 10/29 (34%) neonates had severe TCP, only one ICH was detected. CONCLUSIONS: This study provides data on contemporary "real world" management of Australian mothers and babies with NAIT. Antenatal IVIG therapy was associated with better neonatal outcomes. Maternal side-effects and treatment costs were substantial.

Prenatal diagnosis of congenital malformations for the better and for the worse.

BACKGROUND: Prenatal detection of congenital malformations gives an opportunity to positively influence prenatal and postnatal management, survival and morbidity, as well as to allow parental choice and psychological preparation. OBJECTIVE: To examine the available evidence regarding the impact of detection and underdetection of fetal anomalies as well as that of fetal therapy on neonatal outcome, parental psychological wellness and neonatal care organization. METHODS: Literature search of PubMed. RESULTS AND CONCLUSIONS: The impact of prenatal diagnosis on neonatal survival and morbidity is dependent upon the type and severity of the anomalies inasmuch as upon the evaluation criteria chosen. We discuss the various pitfalls in the design of trials explaining why only few studies, targeting selected anomalies, have shown improved outcomes associated with prenatal diagnosis of congenital malformations. Fetal therapy applies mainly to conditions that are lethal either in utero or at birth if untreated before birth. It has rarely been evaluated with the highest level of evidence; this may be explained by a poor acceptability of randomization between prenatal intervention and expectancy up until delivery.

Current Selection Criteria and Perioperative Therapy Used for Fetal Myelomeningocele Surgery.

OBJECTIVE: To determine the current maternal and fetal selection criteria and operative approaches used at centers performing fetal myelomeningocele surgery. METHODS: The 17 principal investigators participating in the Fetal Myelomeningocele Consortium were asked to participate in an anonymous online survey regarding the current practice of maternal-fetal surgery for neural tube defect repair and results were tabulated. The 35-question survey related to diagnostic testing, inclusion and exclusion criteria, and clinical management. RESULTS: Sixty-five percent (11/17) of principal investigators responded to the survey and not all centers responded to all 35 questions. All centers continue to use magnetic resonance imaging in their preoperative evaluation. Diagnostic testing from amniocentesis is varied: 5 of 11 (45%) require amniotic fluid α-fetoprotein, 4 of 10 (40%) amniotic fluid acetylcholinesterase, and 8 of 11 (73%) DNA microarray. There is also variation from the Management of Myelomeningocele Study with regard to body mass index (BMI) (1/11; 9% would offer surgery with BMIs higher than 35), maternal medical risk factors (surgery would be offered for controlled pregestational diabetes [3/10 (30%)]), hepatitis C with negative viral load (4/11 [36%]), and human immunodeficiency virus with an undetectable viral load (1/10 [10%] or an obstetric history [3/11 (27%)] would offer surgery with a history of preterm delivery on progesterone). Ten of 11 (91%) centers did not consider ventriculomegaly of 18 mm and 9 of 11 (82%) centers did not consider lack of leg movement as an exclusion criteria. Nuances in the perioperative and intraoperative management were also reported, including 5 of 11 (45%) use intraoperative echocardiography and alterations in postoperative tocolytics. CONCLUSION: Variation in practice patterns for offering and performing maternal-fetal surgery for myelomeningocele repair exists among centers. Ongoing evaluation of inclusion and exclusion criteria as well as operative techniques is warranted to ensure continued safety, effectiveness, and beneficence.

International Fetal Cardiac Intervention Registry: A Worldwide Collaborative Description and Preliminary Outcomes.

BACKGROUND: Invasive fetal cardiac intervention (FCI) has been reported in single-institution series, promoting technical and physiologic success. OBJECTIVES: This study describes the creation of an international registry of cases presenting for FCI, intended to compile technical and outcome data from a multicenter cohort. METHODS: For this initial analysis, the entire database of the International Fetal Cardiac Intervention Registry (IFCIR) was queried for details of diagnoses, procedures, and outcomes. Maternal-fetal dyads from January 2001 through June 2014 were included. RESULTS: Eighteen institutions submitted data by data harvest. Of 370 cases entered, 245 underwent FCI: 100 aortic valvuloplasties from a previous single-center report (excluded from additional reporting here), an additional 86 aortic and 16 pulmonary valvuloplasties, 37 atrial septal cases, and 6 unclassified cases. FCI did not appear to affect overall survival to hospital discharge. Among live-born infants with a fetal diagnosis of aortic stenosis/evolving hypoplastic left heart syndrome, more than twice as many were discharged with biventricular circulation after successful FCI versus those meeting institutional criteria but without any or successful FCI (42.8% vs. 19.4%, respectively). When fetal deaths were counted as treatment failures, the percentages were similar: biventricular circulation at discharge was 31.3% versus 18.5% for those discharged with univentricular palliation. Survival to discharge for live-born fetuses with atrial restriction was similar to that of those undergoing technically successful versus unsuccessful FCI (63.6% vs. 46.7%, respectively), although criteria for diagnosis were nonuniform. CONCLUSIONS: We describe the contents of the IFCIR and present post-natal data to suggest potential benefit to fetal therapy among pregnancies considered for possible intervention and support proposals for additional work.