Fetal Surveillance in High-Risk Fetal Cardiac Disease: Frequency, Results and Relationship with Survival.

Objectives: We hypothesized that pregnancies with high-risk fetal heart disease (FHD) would benefit from frequent prenatal surveillance, abnormal fetal surveillance results would be associated with worse outcomes, and cardiovascular profile scoring (CVPS) could identify FHD cases at the highest risk of death.

Methods: A retrospective single-centre study of all actively treated pregnancies with high-risk FHD from 2006-2020. Frequency and results of fetal surveillance, survival, and mode of delivery were collected.

Size and Function of the Right Atrium in Healthy Children by Three-Dimensional Echocardiography.

Purpose: Right atrial volume (RAV) and function have proven prognostic value in the assessment of cardiac disease and may be more accurately assessed using three-dimensional echocardiography (3DE). Normal 3DE reference values for the right atrial (RA) volume and function in healthy children have not yet been published. Furthermore, current methods of indexing cardiac measurements to body surface area (BSA) alone may be insufficient.

Fetal Echocardiography From 10 to 15 Weeks of Gestation-Reliability, Genetic Associations, and Outcomes.

Introduction: There is increasing demand for accurate early fetal cardiac disease (FCD). We assessed the accuracy of early fetal echo (EFE) conducted in our high-volume fetal cardiac program and reviewed the spectrum of FCD, associated genetic anomalies, and outcomes encountered.

Methods: We identified all EFEs performed from 10 to 15 weeks of gestation from 2009 to 2021.

A Comparison of Perinatal Circulatory Transition in Critical Right and Left Heart Obstructive Lesions.

Background: During perinatal transition in hypoplastic left heart syndrome (HLHS), reduced systemic blood flow (Qs) and cerebral blood flow and increased pulmonary blood flow (Qp) are observed, contributing to hemodynamic instability. The aim of the present study was to explore whether similar or discordant perinatal changes occur in critical pulmonary outflow tract obstruction (POFO) compared with HLHS and healthy control subjects.

Methods: Echocardiography was prospectively performed at 36 to 39 gestational weeks and then serially from 6 to 96 hours after birth, before cardiac intervention.

Perinatal Cardiac Functional Adaptation in Hypoplastic Left Heart Syndrome: A Longitudinal Analysis.

Background: The perinatal transition is characterized by acute changes in cardiac loading. Compared with normal newborn combined cardiac output (CCO), single right ventricular (RV) output of neonates with hypoplastic left heart syndrome (HLHS) is markedly greater. The aim of this study was to examine the mechanisms of cardiac adaptation that facilitate this perinatal transition from late fetal to early neonatal life in HLHS.

Use of a Postoperative Care Management Pathway Reduces the Incidence of Chylothorax Post-Fontan Palliation.

Pleural effusions and chylothorax are challenging morbidities post-Fontan palliation. We sought to evaluate the efficacy of our Fontan Care Pathway (FCP) in reducing the incidence of post-operative chylothorax and Time to Chest Tube Removal (TTCTR), and to determine risk factors associated with longer TTCTR. Between 2016 and 2022 our institutional approach to post-Fontan care fell into three categories: Group 1 (n = 36): no standardized approach; Group 2 (n = 30): a prophylactic chylothorax diet (fat content < 5%); Group 3 (n = 57): the FCP (a chylothorax diet, fluid restriction, supplemental O2 and aggressive diuresis).

Socioeconomic Determinants of Health: Remoteness From Care.

Remoteness from care remains a major challenge to equitable provision of health services worldwide. Beyond the difficulties associated with geographically and climatically rugged terrain, there are also socioeconomic, cultural, and technological challenges associated with remote residence. The objective of this review is to examine the factors whereby remoteness can be associated with sociodemographic disadvantage in health care and describe some of the methodologies for measurement and analysis of remoteness, with examples from the literature, particularly focusing on Canada.

Impact of Neonatal Intervention on Left Ventricular Performance in Ebstein's Anomaly and Tricuspid Valve Dysplasia.

Background: Severe neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are associated with high perinatal morbidity and mortality. The authors recently demonstrated left ventricular (LV) dysfunction and dyssynchrony to be prevalent in affected newborns and to contribute to poor outcomes. The aim of this study was to investigate the impact of patent ductus arteriosus (PDA) closure, spontaneous or surgical ligation, or right ventricular exclusion (Starnes procedure) on LV performance in neonatal EA and TVD.

Lower Socioeconomic Status is Associated with an Increased Incidence and Spectrum of Major Congenital Heart Disease and Associated Extracardiac Pathology.

Several studies have suggested an inverse relationship between lower socioeconomic status (SES) and the incidence of congenital heart disease (CHD) among live births. We sought to examine this relationship further in a Canada-wide population study, exploring CHD subtypes, trends, and associated noncardiac abnormalities. Infants born in Canada (less Quebec) from 2008 to 2018 with CHD requiring intervention in the first year were identified using ICD-10 codes through the Canadian Institute for Health Information Discharge Abstract Database.

Perinatal iron restriction is associated with changes in neonatal cardiac function and structure in a sex-dependent manner.

Iron deficiency (ID) is common during gestation and in early infancy and can alter developmental trajectories with lasting consequences on cardiovascular health. While the effects of ID and anemia on the mature heart are well documented, comparatively little is known about their effects and mechanisms on offspring cardiac development and function in the neonatal period. Female Sprague-Dawley rats were fed an iron-restricted or iron-replete diet before and during pregnancy.

Impact of Socioeconomic Status and Residence Distance on Infant Heart Disease Outcomes in Canada.

Background Socioeconomic status (SES) impacts clinical outcomes associated with severe congenital heart disease (sCHD). We examined the impact of SES and remoteness of residence (RoR) on congenital heart disease (CHD) outcomes in Canada, a jurisdiction with universal health insurance. Methods and Results All infants born in Canada (excluding Quebec) from 2008 to 2018 and hospitalized with CHD requiring intervention in the first year were identified.

Comparing a knowledge-based 3D reconstruction algorithm to TomTec 3D echocardiogram algorithm in measuring left cardiac chamber volumes in the pediatric population.

Background: Three-dimensional echocardiography (3DE) is an emerging method for volumetric cardiac measurements; however, few vendor-neutral analysis packages exist. Ventripoint Medical System Plus (VMS3.0+) proprietary software utilizes a validated Magnetic resonance imaging (MRI) database of normal ventricular and atrial morphologies to calculate chamber volumes.

Trends in the Prenatal Detection of Major Congenital Heart Disease in Alberta From 2008-2018.

Objective: The effect of expanded obstetrical ultrasound cardiac views on the diagnosis of fetal congenital heart disease (CHD) has not been fully examined at a population level. We hypothesized there has been a significant increase in the prenatal detection of CHD in Alberta, particularly for CHD associated with cardiac outflow tract and 3-vessel view abnormalities.

Methods: Using provincial databases, we retrospectively identified all fetuses and infants diagnosed between 2008 and 2018 in Alberta with major CHD requiring surgical intervention within the first postnatal year.

Accuracy of Fetal Echocardiography in Defining Anatomic Details: A Single-Institution Experience over a 12-Year Period.

Background: Fetal echocardiography has evolved over four decades, now permitting the prenatal diagnoses of most major congenital heart disease (CHD). To identify areas for targeted improvement, the authors explored the diagnostic accuracy of fetal echocardiography in defining major fetal CHD.

Methods: All fetuses with major fetal CHD (11 subtypes) at a single institution between 2007 and 2018 were identified (n = 827).

Left Ventricular Dysfunction in Neonatal Ebstein's Anomaly and Tricuspid Valve Dysplasia.

Background: The mechanisms and prognostic importance of left ventricular (LV) dysfunction in neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are not well understood. The authors recently demonstrated reduced cardiac output and dyssynchrony to be common in fetal EA/TVD and therefore hypothesized that LV dysfunction may be associated with worse outcomes in neonatal EA/TVD.

Methods: A multicenter retrospective case-control study was conducted among neonatal patients with EA/TVD (n = 32) and a healthy control cohort (n = 17) encountered from 2004 to 2019.

Extra-cardiac diagnoses and postnatal outcomes of fetal tetralogy of fallot.

Objective: Past studies of fetal tetralogy of fallot (ToF) have reported extra-cardiac anomalies (ECAs) in 17%-45%, genetic syndromes in as low as 20% and poor postnatal outcomes. This study sought to examine these factors in a contemporary cohort.

Methods: A retrospective review examining 83 fetuses with ToF diagnosed 2012-2019.

The Diagnostic Yield of Fetal Echocardiography Indications in the Current Era.

Background: The aim of this study was to examine the diagnostic yield of current fetal echocardiography (FE) indications representing a recent era.

Methods: FE reports of all pregnancies referred to two provincial FE programs from 2009 to 2018 were examined, identifying the indication for FE (14 categories), gestational age at referral, and whether there was no fetal heart disease (FHD), mild or possible FHD (e.g.

Influence of extreme prematurity and bronchopulmonary dysplasia on cardiac function.

Objective: Prematurity and bronchopulmonary dysplasia (BPD) are associated with poorly understood abnormalities of ventricular function. We therefore comprehensively compared biventricular function in infants with and without BPD.

Methods: Prospective observational study in extremely preterm infants with (n = 20) and without (n = 38) BPD using conventional and advanced echocardiography at 28 days (T1) and near-term (T2).

The Perinatal Transition and Early Neonatal Period in Hypoplastic Left Heart Syndrome Is Associated With Reduced Systemic and Cerebral Perfusion.

Background: The impact of the striking perinatal circulatory changes on blood flow distribution have not to date been well examined in hypoplastic left heart syndrome (HLHS). This study aimed to document perinatal redistribution of cardiac output in HLHS compared with healthy control subjects, to further understand the impact of the perinatal transition on cerebral and systemic blood flow.

Methods: Prospectively recruited HLHS case subjects (n = 31) and healthy control subjects (n = 19) underwent serial echocardiography from late fetal stages to 96 hours after birth.

Fetal double outlet right ventricle without heterotaxy syndrome: Diagnostic spectrum, associated extracardiac pathology and clinical outcomes.

Objectives: To document the clinical spectrum and outcomes of fetal double outlet right ventricle (DORV) without heterotaxy in a recent diagnostic era.

Methods: Prenatal cases of DORV consecutively diagnosed from 2007 to 2018 were retrospectively identified. Clinical records, including details regarding genetic testing and pre and postnatal imaging were reviewed.

Fetal Diagnosis is Associated with Improved Perioperative Condition of Neonates Requiring Surgical Intervention for Coarctation.

To define the relative importance of fetal diagnosis and comorbidities in severity of preoperative compromise, outcomes and hospitalization in neonatal coarctation of the aorta (CoA). Retrospective comparison of preoperative condition and postoperative course of neonates prenatally (PreDx n = 48) or postnatally diagnosed (PostDx n = 67) with CoA. Congenital and non-congenital comorbidities were adjusted for.

Persistent Aortic Stiffness and Left Ventricular Hypertrophy in Children of Diabetic Mothers.

Background: Fetuses of diabetic mothers develop left ventricular (LV) hypertrophy and are at increased long-term risk of cardiovascular disease. In our previous longitudinal study from midgestation to late infancy we showed persistence of LV hypertrophy and increased aortic stiffness compared with infants of healthy mothers, the latter of which correlated with third trimester maternal hemoglobin A1c. In the present study, we reexamined the same cohort in early childhood to determine if these cardiovascular abnormalities persisted.