Impact of maternal bariatric surgery on offspring perinatal cardiac function: A prospective study.

Objective: To assess perinatal cardiac function in offspring of women with previous bariatric surgery and examine its association with maternal glucose control.

Design: Prospective study.

Setting: Maternity unit, UK.

Management of prenatally detected vascular rings: a United Kingdom national survey.

Objective: To investigate UK variability in prenatal and postnatal management strategy of right aortic arch and double aortic arch (RAA/DAA).

Methods: Online surveys were sent to senior physicians (consultants) of the National Fetal Cardiology Working Group regarding prenatal diagnosis, counselling, and perinatal management of antenatally diagnosed RAA/DAA and to the British Congenital Cardiovascular Association regarding postnatal management strategies.

Results: There were 28 prenatal and 90 postnatal surveys completed.

Is Cardiomegaly an Indication of "Heart-Sparing Effect" in Small Fetuses?

Introduction: This study aimed to test the hypothesis that cardiac size is maintained in small fetuses presenting with cardiomegaly.

Materials And Methods: We identified singleton fetuses with estimated fetal weight <10th centile and with cardiomegaly without another more likely cardiac or extra-cardiac cause. We used Z-scores for cardiac and thoracic circumferences normalized for gestational age (GA), biparietal diameter (BPD), head circumference (HC), and femur length (FL), obtained from 188 normally grown fetuses.

Impact of gastrointestinal comorbidities in patients with right and left atrial isomerism.

Background And Aim: Heterotaxy syndrome, being right atrial isomerism (RAI) or left atrial isomerism (LAI), often presents with Congenital Heart Disease (CHD). Intestinal abnormalities, including malrotation are common. We assessed the spectrum of gut abnormalities and their impact on medium-term outcome in a cohort of patients with fetal and postnatal diagnoses of heterotaxy syndrome.

In-hospital interstage improves interstage survival after the Norwood stage 1 operation.

Objectives: The interstage mortality rate after a Norwood stage 1 operation remains 12-20% in current series. In-hospital interstage facilitates escalation of care, possibly improving outcome.

Methods: A retrospective study was designed for hypoplastic left heart syndrome (HLHS) and HLHS variants, offering an in-hospital stay after the Norwood operation until the completion of stage 2.

Evidence for uteroplacental malperfusion in fetuses with major congenital heart defects.

Aims: Fetuses affected by congenital heart defects (CHD) are considered to be at increased risk of fetal growth restriction and intrauterine demise. Whether these risks are a direct consequence of fetal CHD or a result of associated uteroplacental dysfunction is not evident from the data of recent studies. The aim of this study was to investigate the prevalence of uteroplacental dysfunction reflected by abnormal uterine artery Doppler indices and reduced fetal growth in CHD pregnancies.

Intervendor Discordance of Fetal and Neonatal Myocardial Tissue Doppler and Speckle-Tracking Measurements.

Background: Fetal and neonatal studies report a wide range of cardiac parameters derived by pulsed-wave Doppler tissue imaging (DTI) and two-dimensional speckle-tracking echocardiographic (STE) imaging. The use of different ultrasound systems and their vendor-specific software compromises the ability to compare echocardiographic findings among various studies. The aim of this study was to evaluate intervendor reproducibility as well as intra- and interobserver repeatability of DTI and STE measurements in normal-term fetuses and neonates.

Fetal dysrhythmias.

Fetal dysrhythmias are common abnormalities, usually manifesting as irregular rhythms. Although most irregularities are benign and caused by isolated atrial ectopics, in a few cases, rhythm irregularity may indicate partial atrioventricular block, which has different etiological and prognostic implications. We provide a flowchart for the initial management of irregular rhythm to help select cases requiring urgent specialist referral.

Risk factors for situs defects and congenital heart disease in primary ciliary dyskinesia.

Primary ciliary dyskinesia (PCD) is associated with abnormal organ positioning (situs) and congenital heart disease (CHD). This study investigated genotype-phenotype associations in PCD to facilitate risk predictions for cardiac and laterality defects. This retrospective cohort study of 389 UK patients with PCD found 51% had abnormal situs and 25% had CHD and/or laterality defects other than situs inversus totalis.

Perinatal Changes in Fetal Ventricular Geometry, Myocardial Performance, and Cardiac Function in Normal Term Pregnancies.

Background: The fetal heart at term is exposed to an increase in hemodynamic work as a consequence of fetal growth, increased circulating volume, and alteration in loading patterns due to maturational changes in fetoplacental circulation. The extent to which these cardiovascular changes influence human fetal and neonatal cardiac adaptation has not been fully elucidated. The aim of this study was to evaluate perinatal cardiovascular changes in ventricular geometry and myocardial performance in normal term fetuses.

The association between prenatal atrioventricular septal defects and chromosomal abnormalities.

Objective: Atrioventricular septal defect is associated with a high risk of a chromosomal abnormality, particularly trisomy 21. The aim of this study is to assess the rate of trisomy 21 in fetuses diagnosed with an atrioventricular septal defect and to examine the influence of prior screening on the rate of trisomy 21.

Methods: Electronic ultrasound database was searched to identify fetuses diagnoses with an atrioventricular septal defect from 2002 to 2014.

Screening and Treatment of Intestinal Rotational Abnormalities in Heterotaxy: A Systematic Review and Meta-Analysis.

Objective: To assess the role of screening and prophylactic surgery for intestinal rotational abnormalities (IRAs) in asymptomatic patients with heterotaxy.

Study Design: PubMed, Embase, and Cinahl were searched electronically to determine the overall incidence of IRAs in heterotaxy; the detection rate of IRAs associated with screening; the incidence of midgut volvulus in patients without screening; and the incidence of morbidity and mortality after prophylactic and emergency Ladd procedures. Relevant data were computed with a meta-analysis of proportions.

Variants of the scimitar syndrome.

Unlabelled: Introduction The scimitar syndrome comprises hypoplastic right pulmonary artery and lung, anomalous right pulmonary venous drainage to the inferior caval vein, aortopulmonary collateral(s) to the right lung, and bronchial anomalies. Aim The aim of this study was to describe the morphological and clinical spectrum of variants from the classical scimitar syndrome in a single institution over 22 years.

Results: In total, 10 patients were recognised.

Interrupted inferior vena cava in fetuses with omphalocele. Case series of fetuses referred for fetal echocardiography and review of the literature.

Objectives: Congenital heart disease is reported in 15-45% of omphalocele cases. Associated abnormalities of systemic veins have occasionally been reported in children and rarely documented in the fetus. We report a case series of interrupted inferior vena cava (Int-IVC) in association with omphalocele and review the literature.

Early fetal echocardiography and anomaly scan in fetuses with increased nuchal translucency.

Nuchal translucency (NT) identified at the time of the 11-14 week scan has been established as one of the best tools for screening for Down syndrome. It has also proven to be of value in screening for other conditions such as cardiac defects and extra-cardiac structural abnormalities. In this article, we present data from our fetal medicine unit that highlight the importance of using NT measurement as a stratifying tool when referring for early scans.

Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients.

Background: Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome.

Methods And Results: We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000-2007) retrospectively in a multinational, multicenter setting.

Comparison of transplacental treatment of fetal supraventricular tachyarrhythmias with digoxin, flecainide, and sotalol: results of a nonrandomized multicenter study.

Background: Fetal tachyarrhythmia may result in low cardiac output and death. Consequently, antiarrhythmic treatment is offered in most affected pregnancies. We compared 3 drugs commonly used to control supraventricular tachycardia (SVT) and atrial flutter (AF).

Prenatal identification of the pulmonary arterial supply in tetralogy of Fallot with pulmonary atresia.

Objective: To define the patterns of flow of blood to the lungs in fetuses with tetralogy of Fallot and pulmonary atresia.

Background: In this condition, supply of blood to the lungs is provided via an arterial duct or systemic-to-pulmonary collateral arteries, or very rarely through other conduits such as coronary arterial fistulas or an aortopulmonary window. The intrapericardial pulmonary arteries vary in size, and may be absent.

Morphologic and functional predictors of eventual circulation in the fetus with pulmonary atresia or critical pulmonary stenosis with intact septum.

Objectives: The purpose of this study was to determine the morphologic and physiological predictors of post-natal surgical pathway in a longitudinal series of fetuses with pulmonary atresia with intact ventricular septum (PAIVS) and/or critical pulmonary stenosis with reversal of ductal flow (CPS) using statistical modeling.

Background: Pulmonary atresia with intact ventricular septum is rarely associated with chromosomal or extra cardiac malformations, so decisions about continuing a pregnancy are strongly influenced by the prediction of univentricular (UV) or biventricular (BV) circulation.

Methods: Predictive scores were derived, using a combination of z-scores of fetal cardiac measurements (for femoral length) and tricuspid/mitral valve (TV/MV) ratios, to facilitate early prediction of UV or BV circulation in 21 fetuses with PAIVS (18 fetuses) or CPS (3 fetuses) between 1998 and 2004.

Recurrent intrauterine fetal loss due to near absence of HERG: clinical and functional characterization of a homozygous nonsense HERG Q1070X mutation.

Background: Inherited arrhythmias may underlie intrauterine and neonatal arrhythmias. Resolving the molecular genetic nature of these rare cases provides significant insight into the role of the affected proteins in arrhythmogenesis and (extra-) cardiac development.

Objective: The purpose of this study was to perform clinical, molecular, and functional studies of a consanguineous Arabian family with repeated early miscarriages and two intrauterine fetal losses in the early part of the third trimester of pregnancy due to persistent arrhythmias.