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

Measurement of the Great Vessels in the Mediastinum Could Help Distinguish True From False-Positive Coarctation of the Aorta in the Third Trimester

Objective. We investigated the utility of analyzing prenatal mediastinal measures of the great arteries in distinguishing true coarctation of the aorta (CoA) from false-positive CoA. Methods. All fetuses in this study had suspicion of CoA based on the presence of right-left heart disproportion. We defined 3 study groups: group 1, true fetal CoA; group 2, false-positive fetal CoA with a narrow aortic arch; and group 3, false-positive fetal CoA without a narrow aortic arch. Results. In group 1, the mean mediastinal pulmonary artery (PA) to ascending aorta (Ao) diameter ratio ± SD was 2.03 ± 0.48, and in group 2, the ratio was 1.60 ± 0.23. The difference was statistically significant (P = .0018, t test). In group 3, the mean PA:Ao ratio was 1.35 ± 0.14. The difference between groups 1 and 3 was statistically significant (P = .0002, t test). In our study group, for a PA:Ao ratio of 1.60, sensitivity was 83.0%; specificity, 85.0%; positive predictive value, 62.5%; and negative predictive value, 94.0%. Conclusions. In the third trimester, the main PA:Ao ratio as measured in the fetal mediastinum can be a helpful tool in distinguishing true CoA requiring neonatal cardiac surgery from false-positive CoA and simple disproportion requiring medical attention but no surgery in the first month of postnatal life. Key words: coarctation of the aorta; great vessels; mediastinum; third trimester. ntenatal diagnosis of coarctation of the aorta (CoA) is a challenge, although it is critically important for early treatment of the neonate. 1-3 Echocardiography allows for identification of groups at high risk but does not predict with certainty the presence of a CoA after birth. Echocardiographic markers suggestive of CoA in the fetus are many and include a visual disproportion in size between the ventricles, with the right heart larger than the left, visualization of a narrow aortic isthmus or aortic shelf, the presence of a left superior vena cava, and a bicuspid aortic valve. In the third trimester of pregnancy in an otherwise structurally normal heart, substantial right-left heart size disproportion raises the possibility of the diagnosis of CoA with the need for delivery at an appropriately equipped center for performance of a cardiovascular evaluation and postnatal echocardiography. However

Absent pulmonary valve syndrome - diagnosis, associations and outcome in 71 prenatally diagnosed cases

To analyze the spectrum of prenatally diagnosed absent pulmonary valve syndrome (APVS) and the outcome from diagnosis onwards. Fetuses with APVS and tetralogy of Fallot (TOF/APVS) and with APVS and intact ventricular septum (APVS/IVS) were included.status: publishe