Prenatally diagnosed foramen ovale restriction in fetuses with hypoplastic left heart syndrome may be a predictor of longer hospitalization, but not of a need for an urgent rashkind procedure

Objectives: This prospective study aimed to assess the effectiveness of foramen ovale examination in classifying prenatal hypoplastic left heart syndrome (HLHS) in accordance with the new classification groupings for congenital heart defects. Material and methods: The analysis included 145 fetuses with HLHS, diagnosed and monitored between 2008 and 2015 in Prenatal Cardiology Department at Polish Mother’s Memorial Hospital Research Institute in Lodz. The main criteria for classifying our study population into three sub-groups was was the presence of a foramen ovale restriction, which we diagnosed by evaluating the diameter and blood flow through the foramen ovale. Of the total group, 73.8% (n = 107) were classified as severe planned, 24.1% (n = 35) as severe urgent, and 2.1% (n = 3) as the severest group. Results: Comparing the severe planned and the severe urgent HLHS groups showed: gestational age of delivery 38 vs 38 weeks respectively (p = 0.45); cesarean delivery 62% vs 79.2% (p = 0.15); neonatal birth weight 3110 g vs 2985 g (p = 0.2); Apgar score 9 vs 9 points; survival rate 65.8% vs 61.9% (p = 0.8); and hospitalization 38 vs 46.5 days (p = 0.059). Prenatal qualification for the group of severe urgent HLHS was characterized by 100% sensitivity, 80.6% specificity and a low posi- tive predictive value of 9.5%. Conclusions: 1. Prenatal qualification into the group of severe urgent CHD based on the features of foramen ovale was characterized by high sensitivity, a satisfying specificity and a low positive predictive value. 2. Prenatally diagnosed foramen ovale restriction may be a predictor of longer hospitalization, but not of a need for an urgent Rashkind procedure. 3. New classifications of CHDs allowed clinicians to determine prognoses and to plan optimal multi-specialized care which resulted in similar outcomes between the severe planned and severe urgent HLHS groups.

Ectopia cordis: prenatal diagnosis, perinatal outcomes, and postnatal follow-up of an international multicenter cohort case series

Objective: This study aimed to analyze prenatal diagnosis, perinatal outcomes, and postnatal follow-up in fetuses with ectopia cordis (EC). Methods: This retrospective analysis accessed 31 patients with EC who were either diagnosed or referred to a tertiary Fetal Medicine centers for EC diagnosis in Brazil, Germany, Italy, and Poland. We analyzed prenatal diagnosis, perinatal outcomes, and follow-up in these patients. Results: Our study included a cohort of 31 fetuses with EC, 4 and 27 of whom had partial and complete protrusion of the heart through a ventral defect in the thoracoabdominal wall, respectively. EC was diagnosed by fetal echocardiography at a mean gestational age of 20.3 ± 8.6 weeks (range, 8-35 weeks). Of the four cases, in which the karyotype was performed, all of them had a normal result (1 - 46,XX and 3 - 46,XY). Five patients showed conotruncal abnormalities and six ventricular septal defects. Termination of pregnancy (TOP) was performed in 15 cases (48%) and seven pregnant women had spontaneous fetal demise (22.5%). Of the seven fetuses that were born alive, four of them died, and three infants underwent surgery. Among these three infants, all of them survived, one was 5 months, 13 years old and 29 years old at the time of study completion. Conclusions: Ectopia cordis is associated with high mortality rates and intracardiac/extra-cardiac defects. Ventricular septal defects and conotruncal anomalies were the more common intracardiac defects associated with EC. However, in this cohort of fetuses with EC the incidence of PC was lower than reported in the literature

Large-scale preparation of the oligosaccharide phosphate fraction of Pichia holstii NRRL Y-2448 phosphomannan for use in the manufacture of PI-88

Mild acid-catalysed hydrolysis of the extracellular phosphomannan of the yeast Pichia holstii NRRL Y-2448 produces a high-molecular-weight phosphomannan core, a low-molecular-weight oligosaccharide phosphate fraction, and a neutral oligosaccharide fraction. A method was developed for the large-scale preparation of the oligosaccharide phosphate fraction, consisting predominantly of the pentasaccharide phosphate, 6-O-PO3H2-alpha -D-Man-(1 --> 3)-alpha -D-Man-(1 --> 3)-alpha -D-Man-(1 --> 3)-alpha -D-Man-(1 --> 2)-D-Man, for use in the manufacture of the promising new anti-cancer agent, PI-88. Further insights were also gained into the structure of the phosphomannan by HPLC analysis of the time course of the hydrolysis reaction. (C) 2001 Elsevier Science Ltd. All rights reserved