Utilizing Fetal Echocardiography to Risk Stratify and Predict Neonatal Outcomes in Fetuses Diagnosed with Congenital Heart Disease.

Objective:  Risk stratification of fetuses diagnosed with congenital heart disease (CHD) helps provide a delivery plan and prepare families and medical teams on expected course in the delivery room. Our aim was to assess the accuracy of echocardiographically determined risk-stratification assignments in predicting postnatal cardiac outcomes beyond the delivery room.

Study Design:  This was a retrospective study at a single center evaluating all fetuses with CHD who were risk-stratified by echocardiographically determined level of care (LOC) assignment, ranging from 1a (lowest risk) to 4 (highest risk).

Prenatal Diagnosis of Congenital Heart Disease: The Crucial Role of Perinatal and Delivery Planning.

Although most congenital heart defects (CHDs) are asymptomatic at birth, certain CHD lesions are at significant risk of severe hemodynamic instability and death if emergent cardiac interventions are not performed in a timely fashion. Therefore, accurate identification of at-risk fetuses and appropriate delivery resource planning according to the degree of anticipated hemodynamic instability is crucial. Fetal echocardiography has increased prenatal CHD detection in recent years due to advancements in ultrasound techniques and improved obstetrical cardiac screening protocols, enabling the prediction of newborns' hemodynamic status.

Anatomical and physiological diagnostic discrepancies in fetuses with single-ventricle congenital heart disease in a contemporary cohort.

Objective: Image quality of fetal echocardiography (FE) has improved in the recent era, but few recent studies have reported the accuracy of FE, specifically in single ventricle (SV) congenital heart disease (CHD). This study aimed to assess the ability of FE to correctly predict SV-CHD postnatal anatomy and physiology in a contemporary cohort.

Methods: The contemporary clinical reports of patients with SV-CHD, in which FE was performed between July 2017 and July 2021, were compared with postnatal echocardiograms from a formal quality assurance program.

Prevalence of pulmonary hypertension in obstructive sleep apnea and its relation to disease severity.

Study Objectives: The purpose of this study is to examine the prevalence of pulmonary hypertension (PHTN) in children with obstructive sleep apnea (OSA) using echocardiographic (ECHO) parameters and to examine ECHO findings as they relate to severity of OSA.

Methods: A retrospective cohort study of patients with OSA undergoing polysomnogram and ECHO within 30 days of each other, between January 1, 2015, and December 31, 2020, was performed, excluding cardiac disease. ECHO evidence of PHTN was defined as ≥ 2 of the following: tricuspid regurgitation velocity > 3.

Contemporary Outcomes in Fetuses Diagnosed with Vascular Rings.

Vascular rings are increasingly identified on fetal echocardiography. The purpose of this study is to analyze clinical outcomes and patterns of diagnostic testing in fetuses with vascular rings diagnosed by echocardiography. A retrospective cohort study was performed of fetuses with postnatally confirmed vascular rings from 2017 to 2022.

Congenitally Corrected Transposition of the Great Arteries: Fetal Diagnosis, Associations, and Postnatal Outcome: A Fetal Heart Society Research Collaborative Study.

Background Fetal diagnosis of congenitally corrected transposition of the great arteries (ccTGA) has been increasingly reported; however, predictors of clinical outcomes remain underexplored. We undertook a multicenter, retrospective study to investigate natural history, associated anomalies, and outcomes of fetal ccTGA. Methods and Results Fetuses with ccTGA diagnosed from January 2004 to July 2020 within 20 North American programs were included.

Evaluation of Left Ventricular Outflow Gradients During Staged Exercise Stress Echocardiography Helps Differentiate Pediatric Patients With Hypertrophic Cardiomyopathy From Athletes and Normal Subjects.

Background: Elevated left ventricular outflow tract (LVOT) gradients during exercise can occur in patients with hypertrophic cardiomyopathy (HCM) as well as in athletes and normal controls. The authors' staged exercise protocol calls for imaging at rest and during each stage of exercise to evaluate the mechanism of LVOT obstruction at each stage. They investigated whether this staged approach helps differentiate HCM from athletes and normal controls.

Fetal Diagnosis in a Unique Case of Vascular and Cardiac Interdependence in Omphaloischiopagus Conjoined Twins.

• Cardiac interdependence in conjoined twins is a rare phenomenon. • Presence of congenital heart disease may be an important prognostic factor. • There is high risk of high-output heart failure and pulmonary hypertension in the donor twin.

Impact of Socioeconomic Status, Race and Ethnicity, and Geography on Prenatal Detection of Hypoplastic Left Heart Syndrome and Transposition of the Great Arteries.

Background: Prenatal detection (PND) has benefits for infants with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but associations between sociodemographic and geographic factors with PND have not been sufficiently explored. This study evaluated whether socioeconomic quartile (SEQ), public insurance, race and ethnicity, rural residence, and distance of residence (distance and driving time from a cardiac surgical center) are associated with the PND or timing of PND, with a secondary aim to analyze differences between the United States and Canada.

Methods: In this retrospective cohort study, fetuses and infants <2 months of age with HLHS or TGA admitted between 2012 and 2016 to participating Fetal Heart Society Research Collaborative institutions in the United States and Canada were included.

Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study.

Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia.

Test-retest variability for quantitative two-dimensional and Doppler measurements in the fetus.

Objectives: Echocardiography is used to quantitatively characterize cardiovascular function in fetuses with cardiac abnormalities and inform decisions for fetal or perinatal interventions. It is clinically important to understand the reproducibility of these measures, particularly between testers. While studies have reported intra-observer variability and inter-observer variability, little is known about test-retest variability for these measures.

Preoperative Aortic Arch Size and Late Outcome After Coarctation Repair by Lateral Thoracotomy.

Background: The approach (lateral thoracotomy versus median sternotomy) to repair coarctation of the aorta is frequently based on arch dimensions from the preoperative echocardiogram. Few studies have assessed the relationship between preoperative arch dimensions and late postoperative outcome. This study aimed to define how preoperative arch dimensions relate to late outcomes and identify long-term predictors of a successful operation.

Factors Influencing Pediatric Outpatient Transthoracic Echocardiography Utilization Before Appropriate Use Criteria Release: A Multicenter Study.

Background: Although pediatric appropriate use criteria (AUC) for outpatient transthoracic echocardiography (TTE) are available, little is known about TTE utilization patterns before their release. The aims of this study were to determine the relation between AUC and TTE utilization and to identify patient and physician factors associated with discordance between the AUC and clinical practice.

Methods: A retrospective review of 3,000 initial outpatient pediatric cardiology encounters at six centers was performed.

Impaired cerebral autoregulation in preoperative newborn infants with congenital heart disease.

Objectives: To characterize cerebral autoregulation (CA) in preoperative newborn infants with congenital heart disease (CHD).

Methods: This was a prospective, pilot study of term newborns with CHD who required intensive care. Continuous mean arterial blood pressure (MAP), cerebral tissue oxygen saturation (SO) via near-infrared spectroscopy, and arterial oxygen saturation (SaO) were collected.

Utility of Echocardiography in the Assessment of Left Ventricular Diastolic Function and Restrictive Physiology in Children and Young Adults with Restrictive Cardiomyopathy: A Comparative Echocardiography-Catheterization Study.

The aim of the study is to determine the utility of echocardiography in the assessment of diastolic function in children and young adults with restrictive cardiomyopathy (RCM). RCM is a rare disease with high mortality requiring frequent surveillance. Accurate, noninvasive echocardiographic measures of diastolic function may reduce the need for invasive catheterization.

Evaluation and Management of Fetal Cardiac Function and Heart Failure.

Two-dimensional (2D) and Doppler echocardiography has become both an essential component in the assessment of anatomic anomalies of the fetal heart and an important tool in the assessment of fetal cardiac function. Various techniques for assessing fetal cardiovascular function exist and can be used to characterize systolic and diastolic ventricular function and estimate fetal cardiac output. These techniques can be utilized to assess fetuses with cardiovascular compromise due to a number of cardiac and non-cardiac conditions, including twin-twin transfusion syndrome, high-cardiac output lesions such as fetal tumors and vascular malformations, and primary fetal cardiomyopathy.

Critical care for patients with congenital abnormalities of the coronary arteries.

Congenital abnormalities of the coronary arteries in the absence of structural heart disease account for a small but interesting percentage of cardiac lesions in children. Their presentation may vary from incidental identification to aborted/sudden cardiac death. Patients with aborted sudden death episodes will require significant support if they develop extensive ischaemic myocardial injury.

Changes in Ventricular Geometry Predict Severity of Right Ventricular Hypertension.

Changes in ventricular geometry are often seen in patients with right ventricular hypertension secondary to pulmonary hypertension (PH). Progressive systolic bowing of the inter-ventricular septum occurs with increasing right ventricular pressure (RVp) and can be quantified with the left ventricular end-systolic eccentricity index (LVEI). Only limited data exist in children to evaluate the relationship between the LVEI and invasive RVp.

Intrauterine fetal demise after prenatal diagnosis of congenital heart disease: assessment of risk.

Objectives: Elective deliveries in fetal congenital heart disease (CHD) attempt to balance fetal and neonatal risk with the goal of optimizing overall outcome. However, the magnitude of the risk for intrauterine fetal demise (IUFD) is unclear. This study aimed to (1) determine the rate of IUFD and (2) identify fetal risk factors associated with IUFD.

Using Improvement Methodology to Optimize Echocardiographic Imaging of Coronary Arteries in Children.

Background: According to the American Society of Echocardiography, coronary artery (CA) imaging is recommended in pediatric examinations to identify CA anomalies. A review of the authors' center's echocardiographic studies revealed that CA images were often nondiagnostic. The aim of this study was to utilize quality improvement methodology to increase the percentage of first-time pediatric studies with definitive CA identification from a baseline of 45% to a goal of at least 75% in 9 months.

Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.

Background: Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era.

Diastolic dysfunction and cerebrovascular redistribution precede overt recipient twin cardiomyopathy in early-stage twin-twin transfusion syndrome.

Background: Indications for intervention in early-stage (Quintero I and II) twin-twin transfusion syndrome (TTTS) are not standardized. Fetal echocardiography can be used to guide the management of early-stage patients. The aim of this study was to identify early cardiovascular findings that may precede progression to overt recipient twin (RT) cardiomyopathy in early-stage TTTS.