Adverse Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology: From the Pediatric Heart Network Fontan Public Data Set.

Patients with Fontan physiology have reduced exercise performance compared to their peers as well as a higher incidence of bundle branch block (BBB). This study aims to investigate the association between BBB and exercise performance in the Fontan population through a retrospective review of the Pediatric Heart Network Fontan study public use dataset. "Low Performers" were defined as ≤ 25th percentile (for Fontan patients) for each exercise parameter at anaerobic threshold (AT) for gender and age and "Normal Performers" were all other patients.

Survival From Birth Until Young Adulthood Among Individuals With Congenital Heart Defects: CH STRONG.

Background: Limited population-based information is available on long-term survival of US individuals with congenital heart defects (CHDs). Therefore, we assessed patterns in survival from birth until young adulthood (ie, 35 years of age) and associated factors among a population-based sample of US individuals with CHDs.

Methods: Individuals born between 1980 and 1997 with CHDs identified in 3 US birth defect surveillance systems were linked to death records through 2015 to identify those deceased and the year of their death.

Congenital left atrial appendage ostial stenosis in an extremely premature infant diagnosed by transthoracic echocardiography.

Congenital left atrial appendage ostial stenosis is a very rare congenital cardiac condition. We present the case of an extremely premature infant with congenital left atrial appendage ostial stenosis diagnosed by transthoracic echocardiographic imaging.

Reproductive Health Counseling and Concerns Among Women with Congenital Heart Defects With and Without Disabilities.

Women with disabilities are less likely to receive reproductive health counseling than women without disabilities. Yet, little is known about reproductive health counseling and concerns among women with congenital heart defects (CHD) and disabilities. We used population-based survey data from 778 women aged 19 to 38 years with CHD to examine contraceptive and pregnancy counseling and pregnancy concerns and experiences by disability status, based on six validated questions on vision, hearing, mobility, cognition, self-care, and living independently.

Cardiology Care and Loss to Follow-Up Among Adults With Congenital Heart Defects in CH STRONG.

Many of the estimated 1.4 million adults with congenital heart defects (CHDs) in the United States are lost to follow-up (LTF) despite recommendations for ongoing cardiology care. Using 2016 to 2019 CH STRONG (Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG) data, we describe cardiac care among community-based adults with CHD, born in 1980 to 1997, identified through state birth defects registries.

Contemporary pregnancy outcomes for women with moderate and severe congenital heart disease.

Background: Women with congenital heart disease (CHD) are surviving into adulthood, with more undergoing pregnancy.

Methods: Retrospective review of the Vizient database from 2017-2019 for women 15-44 years old with moderate, severe or no CHD and vaginal delivery or caesarean section. Demographics, hospital outcomes and costs were compared.

Health Insurance Status and Access to Healthcare Among Young Adults with Congenital Heart Disease: from the Congenital Heart Survey To Recognize Outcomes, Needs and Well-beinG (CH STRONG), 2016-2019.

Having health insurance is associated with better access to healthcare and lower rates of comorbidity in the general population, but data are limited on insurance's impact on adults with congenital heart disease (ACHD). The Congenital Heart Survey To Recognize Outcomes, Needs and well-beinG (CH STRONG) was conducted among ACHD in three locations from 2016 to 2019. We performed multivariable logistic regression to determine the associations between health insurance and both access to healthcare and presence of comorbidities.

ECMO in adults with congenital heart disease - Analysis of a national discharge database.

Background: The number of adults with congenital heart disease (CHD) is increasing and long-term complications may lead to a need for extracorporeal membrane oxygenation (ECMO), but there are no large studies describing outcomes. We sought to describe the epidemiology for adult CHD patients who undergo ECMO and compare outcomes to adults without CHD (NO-CHD) requiring ECMO.

Methods: Query of hospital discharge data from Vizient from October 2014-December 2017 for patients ≥18 years old with an ICD-9 or ICD-10 code for CHD (745-747.

Association Between Sleep Disturbances With Neurodevelopmental Problems and Decreased Health-Related Quality of Life in Children With Fontan Circulation.

Background Children with Fontan circulation are known to be at increased risk for neurodevelopmental problems and decreased health-related quality of life (HRQOL), but many factors that may contribute to this risk are unknown. Sleep disturbances may be one previously unidentified factor that contributes to this risk. Methods and Results We analyzed data from the Pediatric Heart Network Fontan cross-sectional study to evaluate associations between a parent or child report of sleep disturbance with reported neurodevelopmental concerns and HRQOL in 558 children with Fontan circulation.

Disability Among Young Adults With Congenital Heart Defects: Congenital Heart Survey to Recognize Outcomes, Needs, and Well-Being 2016-2019.

Background Disabilities have implications for health, well-being, and health care, yet limited information is available on the percentage of adults with congenital heart defects (CHD) living with disabilities. We evaluated the prevalence of disability and associated characteristics among the 2016-2019 CH STRONG (Congenital Heart Survey to Recognize Outcomes, Needs, and Well-Being) population-based sample of 19- to 38-year-olds with CHD from 3 US locations. Methods and Results Prevalence of disability types (hearing, vision, cognition, mobility, self-care, living independently) were compared with similarly aged adults from the general population as estimated by the American Community Survey and standardized to the CH STRONG eligible population to reduce nonresponse bias and confounding.

Worse Hospital Outcomes for Children and Adults with COVID-19 and Congenital Heart Disease.

The aim of the current study is to investigate hospitalization outcomes of COVID-19 positive children and adults with moderate or severe congenital heart disease to children and adults without congenital heart disease. Retrospective review using the Vizient Clinical Data Base for admissions of patients with an ICD-10 code for COVID-19 from April 2020 to March 2021. Admissions with COVID-19 and with and without moderate or severe congenital heart disease (CHD) were stratified into pediatric (< 18 years) and adult (≥ 18 years) and hospital outcomes were compared.

Advance Care Directives Among a Population-Based Sample of Young Adults with Congenital Heart Defects, CH STRONG, 2016-2019.

Little is known about advance care planning among young adults with congenital heart defects (CHD). Congenital Heart Survey to Recognize Outcomes, Needs, and well-beinG (CH STRONG) participants were born with CHD between 1980 and 1997, identified using active, population-based birth defects surveillance systems in Arkansas, Arizona and Atlanta, and Georgia, and surveyed during 2016-2019. We estimated the percent having an advance care directive standardized to the site, year of birth, sex, maternal race, and CHD severity of the 9312 CH STRONG-eligible individuals.

COVID-19 in Adults With Congenital Heart Disease.

Background: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications.

Objectives: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes.

Methods: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide.

Exercise Performance in Adolescents With Fontan Physiology (from the Pediatric Heart Network Fontan Public Data Set).

In the pediatric population, exercise capacity differs between females and males and the gap widens through adolescence. However, specific age- and sex-based changes in adolescents with congenital heart disease and Fontan palliation have not been reported. The purpose of the current study is to identify age- and sex-specific changes in exercise performance at peak and ventilatory anaerobic threshold (AT) for adolescents with Fontan physiology.

Improvement in Pulmonary Vascular Resistance After Relief of Fontan Circuit Obstruction.

Objectives: Patients with single-ventricle congenital heart disease undergo staged surgical palliations leading to a final Fontan procedure. After Fontan, cardiac index (CI) is primarily determined by pulmonary vascular resistance (PVR). Lower Fontan pressure has been achieved after relieving obstruction within the Fontan circuit, but to date the effect on PVR has not been quantified.

Effect of comorbid neuropsychiatric disorders on children and adolescents undergoing surgery for moderate and severe congenital heart disease.

Background: Children and adolescents with congenital heart disease (CHD) are at an increased risk of neuropsychiatric disorders (NPDs). The purpose of this study is to determine how a comorbid NPD affects hospital outcomes and costs for CHD patients undergoing cardiac surgery.

Methods: Retrospective review of the 2000-2012 Healthcare Cost and Utilization Project Kids' Inpatient Databases for admissions 10 to 21 years old with an ICD-9 code for moderate or severe CHD and a procedure code for cardiopulmonary bypass as a marker for cardiac surgery; admissions with syndromes that could be associated with NPD were excluded.

Early bioprosthetic tricuspid valve stenosis due to size mismatch in Ebstein anomaly-Successful transcatheter treatment.

Patients with congenital heart disease are surviving well into adulthood thanks to advances in medical and clinical care. We present a patient with Ebstein anomaly who underwent surgical tricuspid valve replacement and suffered early valve stenosis due to her unique anatomy. This case highlights the importance of the "unnatural" anatomy that can be encounter in this challenging patient population.

Structured Neonatal Point-of-Care Ultrasound Training Program.

Objective: Point-of-care ultrasound (POC US) has been increasingly used by intensive care physicians. Growing use of POC US necessitates defining distinct clinical indications for its application, as well as structured POC US training programs. Homogeneous approach to POC US education combined with rigorous quality assurance should further enable POC US to become standard-of-care clinical tool.