Perioperative fetal hemodynamic changes in twin-twin transfusion syndrome and neurodevelopmental outcome at two years of age.

OBJECTIVE: To investigate whether perioperative fetal hemodynamic changes in twin-to-twin transfusion syndrome (TTTS) are associated with neurodevelopmental impairment (NDI) at two years. METHODS: Doppler parameters of three sonograms (day before, first day after and 1 week after laser surgery for TTTS) were assessed for correlation with neurodevelopmental outcome at two years (2008-2016). NDI was defined as: cerebral palsy, deafness, blindness, and/or a Bayley-III cognitive/motor developmental test-score > 2SD below the mean. RESULTS: Long-term outcome was assessed in 492 TTTS survivors. NDI was present in 5% (24/492). After adjustment for severe cerebral injury (present in 4%), associated with NDI were: middle cerebral artery peak systolic velocity (MCA-PSV) >1.5 multiples of the median (MoM) 1 day after surgery (odds ratio [OR] 4.96; 95% confidence interval [CI]: 1.17-21.05, P = .03), a change from normal umbilical artery pulsatility index (UA-PI) presurgery to UA-PI >p95 postsurgery (OR 4.19; 95% CI: 1.04-16.87, P = .04), a change from normal to MCA-PSV >1.5MoM (OR 4.75; 95% CI: 1.43-15.77, P = .01). CONCLUSION: Perioperative fetal hemodynamic changes in TTTS pregnancies treated with laser are associated with poor neurodevelopmental outcome. Prospective research on the cerebrovascular response to altered hemodynamic conditions is necessary to further understand the cerebral autoregulatory capacity of the fetus in relation to neurodevelopmental outcome.

Outcomes of hypoplastic left heart syndrome and fetal aortic valvuloplasty in a country with suboptimal postnatal management.

BACKGROUND: Fetal aortic stenosis (AoS) may progress to hypoplastic left heart syndrome (HLHS) in utero. There are currently no data, prenatal or postnatal, describing survival of fetuses or neonates with AoS or HLHS in a country with suboptimal postnatal management. STUDY DESIGN: Prospective cohort study performed in Mexico, including cases diagnosed with AoS and HLHS within a 6-year period. AoS patients fulfilling previously published criteria for evolving HLHS (eHLHS) were offered fetal aortic valvuloplasty. Outcome variables were perinatal mortality, postnatal management, type of postnatal circulation, and overall survival. RESULTS: Fifty-four patients were included: 16 AoS and 38 HLHS. Eighteen patients had associated anomalies and/or an abnormal karyotype. Seventy-four percent of HLHS received comfort measures, with only three cases reporting an attempt at surgical palliation, and one survivor of the first stage. Fetal aortic valvuloplasty was performed successfully in nine cases of eHLHS. Overall postnatal survival was 44% in AoS with fetal aortic valvuloplasty, and one case (ongoing) in the HLHS group. CONCLUSIONS: HLHS in Mexico carries more than a 95% risk of postnatal death, with little or no experience at surgical palliation in most centers. Fetal aortic valvuloplasty in AoS may prevent progression to HLHS and in this small cohort was associated with ≈50% survival.

Prenatal diagnosis of fetal congenitally corrected transposition of the great arteries.

BACKGROUND: Fetal congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac anomaly and the diagnosis remains a challenge for many sonographers in routine screening programs. We describe a new method of diagnosis by four-dimensional (4D) echocardiography and spatiotemporal image correlation (STIC). METHODS: We reviewed retrospectively 11 cases of fetal ccTGA complicated with ventricular septal defect. The four-chamber view (4CV) and the cardiac outflow tract views were obtained by conventional two-dimensional (2D) echocardiography. Atrioventricular connections were observed in the 4CV and ventriculoarterial connections in the cardiac outflow tract views. 4D volume data sets were acquired at the 4CV level and then analyzed by STIC. All volume data sets were displayed using the multiplanar modality, which allows simultaneous display of images in three orthogonal planes. The correlation of the three planes was used to reveal the spatial relationship of the two great arteries. The diagnosis was confirmed by postnatal echocardiography or by autopsy. RESULTS: By 4D echocardiography and STIC, the origin, course and relationship of the two great arteries could be revealed by moving the reference dot in the three correlated orthogonal planes: in ccTGA, the aorta could be seen to arise from the right ventricle, anterior and to the left of the pulmonary artery, and its course was upward and parallel to the pulmonary artery. CONCLUSIONS: 4D echocardiography and STIC technique can assist 2D echocardiography in the diagnosis of ccTGA by revealing the spatial relationship of the transposed great arteries, improving the understanding of the anomaly and facilitating the examination.

Tracheal diameter at birth in severe congenital diaphragmatic hernia treated by fetal endoscopic tracheal occlusion.

OBJECTIVE: To investigate tracheal dimensional differences seen at birth following fetal endoscopic tracheal occlusion (FETO) in cases of severe congenital diaphragmatic hernia (CDH) and to report on their clinical follow-up. PATIENTS AND METHODS: In chest X-rays, taken within 48 h after birth, we measured the tracheal diameter at the level of the tracheal entry into the chest, 1 cm above the level of the carina and at middistance between these sites in 37 fetuses with severe CDH treated by FETO. These measurements were compared with those in 74 preterm and term neonates with no congenital lung abnormalities. RESULTS: In the CDH group, compared to the controls, the tracheal diameter corrected for gestational age was significantly larger at all three levels of the trachea. Regression analysis showed that significant predictors of the tracheal diameter at the level of tracheal entry into the chest were the observed to expected (o/e) lung area to head circumference ratio (LHR) and the duration of tracheal occlusion. In the CDH group, postnatal follow-up until the age of 22 months (1-70) showed that 5 of 24 neonates had an effort-induced barking cough. CONCLUSION: A large number of infants with severe CDH surviving after FETO have a degree of tracheomegaly that is associated with the severity of CDH as assessed by pre-FETO LHR. This tracheomegaly does not constitute an obvious clinical problem.

Long-term outcome after intrauterine laser treatment for twin-twin transfusion syndrome.

Twin-twin transfusion syndrome (TTTS) is a severe complication occurring in about 10% of monochorionic twin pregnancies. The chronic unbalanced transfusion of blood across placental vascular communications from the donor to the recipient twin may lead to impairment of various organ systems in the affected twins. In Hamburg, Germany, since 1995 patients with TTTS were treated with fetoscopic laser coagulation as the first causal therapeutic strategy. All survivors after laser surgery were followed up in the University Children's Hospital in Bonn, Germany. In this article, we summarize long-term follow-up studies from our German study population and compare our results with data from the literature.

The outcome and prognostic factors of twin-twin transfusion syndrome following fetoscopic laser surgery.

OBJECTIVES: To evaluate the outcome and preoperative risks of twin-twin transfusion syndrome (TTTS) following fetoscopic laser surgery (FLS). METHODS: A retrospective cohort study of a series of 181 consecutive cases of TTTS before 26 weeks' gestation subjected to FLS at four centers in Japan between July 2002 and December 2006. RESULTS: The chances of survival of at least one twin at 28 days of age and 6 months of age were 91.2% and 90.1%, respectively. The rate of major neurological complications in survivors at 6 months of age was 4.7%. Preoperative findings that were significant risk factors for death were as follows: (1) being donor [odds ratio (OR): 3.01, 95% confidence interval (CI): 1.24-7.31, P = 0.015]; (2) reversed (OR: 11.78, CI: 3.05-45.55, P < 0.001) and absent (OR: 3.95, CI: 1.66-9.43, P = 0.002) end-diastolic velocity in the umbilical artery (EDV-UA) of the donor; and (3) reversed blood flow in the ductus venosus of the recipient (OR: 2.35, CI: 1.04-5.29, P = 0.040). CONCLUSIONS: FLS leads to high survival rates and low neurological morbidity for fetuses in TTTS. FLS is an effective therapeutic option for TTTS before 26 weeks of gestation. Preoperative Doppler findings of the umbilical artery and the ductus venosus are useful in predicting prognosis following FLS.

Comparison of prenatal and postnatal treatments of spina bifida in Poland--a non-randomized, single-center study.

OBJECTIVE: The aim of this study was a comparison of the outcomes of intrauterine myelomeningocele (MMC) repairs (IUMR) in type II Chiari malformation (II CM) fetuses with clinical data of newborns and infants operated on postnatally. METHODS: The study group (SG) comprised 46 pregnant women whose type II CM children underwent IUMR, while 47 pregnant women whose type II CM children were operated on postnatally constituted the control group (CG). A total of 24 SG and 20 CG patients reached the endpoint of the study. RESULTS: High incidence of prelabor rupture of membranes (24 (52.2%), CI: 3.74 (1.69-8.26) (p < 0.001) was noted in the group of prenatal surgeries as compared to controls. The need for ventriculoperitoneal shunt implantation was statistically significantly lower (p < 0.008) in the group of children after IUMR as compared to controls (5 (27.8%) and 16 (80%), respectively, CI: 0.35 (0.16-0.75). None of the postnatally treated CG children can walk without adaptive equipment. In contrast, two children from the SG (2 (11.1%) CI: 1.86 (1.00-3.48) p < 0.05) are able to walk independently. CONCLUSIONS: Prenatal MMC closure significantly lowers further adverse evolution of the II CM. Further studies are needed, especially on preventive measures for preterm labor and iatrogenic preterm prelabor rupture of membranes (iPPRM) in the postoperative course of IUMR.