J Degenhardt

Percutaneous minimal-access fetoscopic surgery for spina bifida aperta. Part II: maternal management and outcome

To assess maternal morbidity and outcome in women undergoing minimal‐access fetoscopic surgery for spina bifida aperta.

Fetal Loss Rate and Associated Risk Factors After Amniocentesis, Chorionic Villus Sampling and Fetal Blood Sampling

PURPOSE To assess the total and procedure-related fetal loss rate and associated risk factors following amniocentesis (AC), chorionic villus sampling (CVS) and fetal blood sampling (FBS). MATERIALS AND METHODS We performed a retrospective analysis of patients with invasive diagnostics from 1993 to 2011 in two tertiary referral centers. We aimed to classify pregnancy loss after an invasive procedure and included the time after the invasive procedure and the result of targeted ultrasound/karyotype analysis in the analysis. Fetal losses occurring within two weeks after an invasive procedure were classified as procedure-related. RESULTS After excluding 1553 pregnancies with abnormal karyotype, fetal malformations and multiple insertions, 6256 cases were retrieved for final analysis. The total fetal loss rate was 1.5 %. The procedure-related fetal loss rate was 0.4 % for AC, 1.1 % for CVS and 0.4 % for FBS. Maternal vaginal bleeding in the first trimester was significantly associated with an increased procedure-related fetal loss rate (p= 0.008). The number of invasive procedures declined during the study period with increasing numbers of CVS in the first trimester. CONCLUSION In our population the procedure-related fetal loss rate was 0.4 % after AC and 1.1 % and 0.4 % after CVS and FBS, respectively. Different gestational ages at the time of invasive procedures might account in part for those differences. Vaginal bleeding during the first trimester is associated with increased procedure-related fetal loss. Overall, declining numbers of invasive procedures are the result of changing attitudes toward invasive procedures and more sophisticated noninvasive prenatal screening programs over the last 20 years.

Idiopathic Constriction of the Fetal Ductus Arteriosus Three Cases and Review of the Literature

Premature constriction or closure of the ductus arteriosus can occur during fetal life. It is a rare phenomenon and has been described secondary to medication or structural lesions or as idiopathic constriction. Premature closure of the ductus arteriosus can lead to progressive right heart dysfunction with tricuspid regurgitation, congestive heart failure, fetal hydrops, and intrauterine death. This series describes diagnosis of fetal ductus arteriosus constriction of unknown etiology in 3 cases, prenatal management, and outcomes. Constriction of the ductus arteriosus can be diagnosed prenatally with careful interrogation of the ductal arch using pulsed Doppler sonography and complete fetal echocardiography. Close monitoring is mandatory to rule out development of right heart failure and to determine the intervention time.

Noninvasive fetal genotyping of human platelet antigen-1a using targeted massively parallel sequencing

Fetal human platelet antigen (HPA) genotyping is required to determine whether the fetus is at risk and whether prenatal interventions to prevent fetal bleeding are required in pregnant women with a history of fetal and neonatal alloimmune thrombocytopenia (FNAIT). Methods for noninvasive genotyping of HPA alleles with the use of maternal plasma cell‐free DNA were published recently but do lack internal controls to exclude false‐negative results.

Fetal diagnosis of hypoplastic left heart, associations and outcomes in the current era.

PURPOSE Hypoplastic left heart (HLH) is one of the most common forms of cardiac abnormality detectable during gestation by fetal echocardiography. Antenatal diagnosis allows for appropriate counseling and time to consider treatment options. We report the actual outcome data after fetal diagnosis of HLH. MATERIALS AND METHODS Retrospective analysis of the outcome in all cases with HLH from 1994 - 2011 presenting in fetal life at two tertiary referral centers for prenatal diagnosis and pediatric cardiology. RESULTS 105 cases were included and the overall survival is 40.9 % (43/105) after prenatal diagnosis. There was an 81.1 % survival rate in infants undergoing surgery and a 64.1 % survival rate from an intention-to-treat position. Two neonates died due to tamponade and cardiac arrest following balloon septostomy and one neonate from sepsis before surgery. Extracardiac anomalies occurred in three fetuses, and karyotype anomalies in seven fetuses (18.9 %). In 4 of 5 babies born with additional extracradiac or karyotype anomalies, parents opted for compassionate care. The first had trisomy 13, the second had trisomy 18, the third neonate presented with spina bifida, and the fourth presented with hydronephrosis and pulmonary atresia. Termination of pregnancy took place in 17 cases (16.1 %). CONCLUSION Thorough antenatal evaluation should include karyotyping, detailed extracardiac and intracardiac assessment to accurately predict the risks of surgery. Prenatal counseling might be modified after the exclusion of additional anomalies. These data provide up-to-date information for parental counseling.

Fetal pulmonary venous flow and restrictive foramen ovale in hypoplastic left heart.

PURPOSE Hypoplastic left heart (HLH) with intact or restrictive interatrial communication (HLH-IAS/RAS) is associated with high mortality rates. The object was to correlate pulmonary venous (PV) Doppler spectra and direct foramen ovale (FO) assessment with the neonatal need for early atrial septostomy (EAS) and neonatal outcome. MATERIALS AND METHODS We reviewed all prenatal echocardiograms and outcomes of 51 fetuses with HLH and information about the interatrial communication between 1994 - 2011.  IAS/RAS was defined as a small/absent interatrial shunt on 2-dimensional imaging. Three PV Doppler spectra were observed: type A: continuous forward flow with a small a wave reversal; type B: continuous forward flow with increased a-wave reversal; type C: brief to-and-fro flow. RESULTS Three of 51 neonates with type C pulmonary venous flow pattern and suspicion of IAS/RAS on 2-dimensional (2-DE) evaluation required EAS.  In one fetus pulmonary venous flow changed from type B to type C spectra throughout gestation. Fetuses with type C spectra showed a 71.4 % survival after 30 days compared to 92.3 % in fetuses with type A spectra. Short term survival after EAS was 33 %. CONCLUSION Prenatal PV flow pattern and 2-DE of the FO size help in identifying the fetus at risk for neonatal EAS and patient selection for fetal cardiac intervention. Most late second trimester values will not change over time.

Evaluation of right ventricular function in fetuses with hypoplastic left heart syndrome using tissue Doppler techniques.

The outcome of patients with hypoplastic left heart syndrome (HLHS) is influenced by right ventricular function. This study aimed to investigate whether differences in right ventricular function of fetuses with HLHS are present during gestation.

Prenatal Assessment of Ventriculocoronary Connections and Ventricular Endocardial Fibroelastosis in Hypoplastic Left Heart

OBJECTIVE The outlook for newborns with hypoplastic left heart (HLH) has substantially improved over the last decade. However, differences in outcome among various anatomical subgroups have been described. We aimed to describe the incidence of ventriculocoronary communications and endocardial fibroelastosis in HLH and the possible implication on hospital survival (30 d). METHODS We retrospectively reviewed our medical records, still frames and video loops of 72 fetuses with HLH and critical aortic valve stenosis and evolving HLH from 2008 - 2013. The presence of VCAC and EFE were systematically assessed. Outcome parameters were incidence of VCAC and EFE among different anatomical subgroups of HLH and hospital survival (30 d). RESULTS 72 fetuses were included in this series. The incidence of VCAC was 11.1 % (8 cases) and EFE occurred in 33.3 % (24 cases). 5 fetuses with VCAC occurred in the subgroup of mitral valve stenosis/aortic valve atresia (MS/AA, 62.5 %) and 2 fetuses with VCAC occurred in the group of mitral atresia/aortic valve atresia (MA/AA, 25 %). Further classification was not possible in one case with VCAC (12.5 %). EFE predominantly occurred in the subgroup of MS/AA, MA/AA and in those cases with aortic valve stenosis and evolving HLH. The overall hospital survival on an intention-to-treat basis was 91.2 % (52/57 newborns). Hospital survival was 91 % for the subgroup of cases with MS/AA and for all other anatomical subgroups. CONCLUSION The presence of VCAC in HLH can be diagnosed by fetal echocardiography predominantly occurring in cases with obstructed outflow and to some extent patent mitral valve. EFE is a frequent coexisting finding. Hospital survival was comparable among different anatomical subgroups and in cases with VCAC. The presence of VCAC in HLH did not limit the results of surgical palliation within the observation period of 30 days.

First experience with three-dimensional speckle tracking (3D wall motion tracking) in fetal echocardiography.

OBJECTIVES Fetal cardiac function can be quantified by different methods. This is the first approach of real three-dimensional(3 D)-based speckle tracking echocardiography in the fetus to assess different cardiac strain parameters. METHODS We present preliminary results of fetal global myocardial strain analyses. For fetal echocardiography a Toshiba Artida system was used. Based on an apical or basal four-chamber view of the fetal heart, raw data volumes with a high temporal resolution were acquired and digitally stored. RESULTS 8 individual healthy fetuses with an echocardiogram performed between 21 and 37 weeks of gestation were included. The mean temporal resolution was 31.2 ± 4.3 volumes per second (vps). Basic parameters such as longitudinal and circumferential strain as well as advanced 3 D myocardial motion patterns such as area strain, rotation, twist and torsion were assessed. CONCLUSION Currently the assessment of fetal myocardial deformation parameters by 3 D speckle tracking seems to be technically feasible only in individual cases. In the future further development of this technique is necessary to improve its application in fetal echocardiography.

Pulmonary Vasoreactivity to Materno-Fetal Hyperoxygenation Testing in Fetuses with Hypoplastic Left Heart.

PURPOSE The aim of the study was to describe the response of fetal lung vasculature to maternal hyperoxygenation (MH) in the case of prenatally diagnosed hypoplastic left heart (HLH) with intact or restrictive (IAS/RAS) and without restriction of the atrial septum. Furthermore, the ability of MH to differentiate between newborns with HLH who do not require immediate atrial septostomy and newborns who will undergo immediate left atrial septoplasty after birth was evaluated. MATERIALS AND METHODS Cross-sectional prospective study of fetuses ≥ 26 weeks of gestation with prenatally diagnosed HLH. Lung perfusion (LP) was qualitatively assessed by color Doppler interrogation and LP was quantitatively measured using the pulsatility index for veins (PIV). Measurements were performed both with the mother breathing room air (LPRA) and after receiving 100% oxygen for 10 minutes (LPMH). The oxygen test was defined as positive if MH led to an increase in lung perfusion and as negative if MH did not lead to an increase. RESULTS A total number of 22 pregnancies with hypoplasia of the left heart structures were included. 6/20 cases presented with an intact or restrictive atrial septum (IAS/RAS). All of these fetuses presented with a reduced LPRA. MH led to an increase in LP in 2/6 cases. The overall 30-day-survival rate was 83.3% (5/6). In 14/20 fetuses an open septum was detected. 11 cases had a normal LPRA, and the LPRA was reduced in 3/14 fetuses. The overall 30-day-survival rate was 92.9% (13/14). CONCLUSION MH might be a useful adjunct in the assessment of pulmonary vasculopathy in fetuses with HLH.