Management of pulmonary vascular disease associated with congenital left-to-right shunts: A single-center experience.

Objective: The study objective was to describe the course and outcomes of children under 18 years of age, with left-to-right shunts and pulmonary arterial hypertension undergoing 1 of 2 management approaches: pulmonary arterial hypertension treatment before left-to-right shunt repair (Treat First) and left-to-right shunt repair first with or without subsequent pulmonary arterial hypertension treatment (Repair First).

Methods: We performed a retrospective single-center study, conducted from September 2015 to September 2021, of children with left-to-right shunts and pulmonary arterial hypertension (defined as indexed pulmonary vascular resistance ≥ 4 Wood units [WU]∗m) but without Eisenmenger physiology. Patient characteristics, longitudinal hemodynamics data, pulmonary arterial hypertension management, left-to-right shunt repair, and outcomes were reviewed.

A single institution anesthetic experience with catheterization of pediatric pulmonary hypertension patients.

Cardiac catheterization remains the gold standard for the diagnosis and management of pediatric pulmonary hypertension (PH). There is lack of consensus regarding optimal anesthetic and airway regimen. This retrospective study describes the anesthetic/airway experience of our single center cohort of pediatric PH patients undergoing catheterization, in which obtaining hemodynamic data during spontaneous breathing is preferential.

Differences in Quality of Life in Children Across the Spectrum of Congenital Heart Disease.

Objective: To create complexity groups based upon a patient's cardiac medical history and to test for group differences in health-related quality of life (HRQOL).

Methods: Patients 8-18 years with congenital heart disease (CHD) and parent-proxies from the Pediatric Cardiac Quality of Life Inventory (PCQLI) Testing Study were included. Outcome variables included PCQLI Total, Disease Impact, and Psychosocial Impact scores.

Persistence of persistent pulmonary hypertension of the newborn: A case of de novo TBX4 variant.

We present a case of a late preterm infant placed on extracorporeal life support in the first day of life for persistent pulmonary hypertension of the newborn. Developmental arrest, pulmonary vascular hypertensive changes, and pulmonary interstitial glycogenosis were present on lung biopsy at 7 weeks of age. Pulmonary hypertension has persisted through childhood.

Pulmonary artery acceleration time in young children is determined by heart rate and transpulmonary gradient but not by pulmonary blood flow: A simultaneous echocardiography-cardiac catheterization study.

Introduction: Pulmonary artery acceleration time (PAAT) is considered useful for the non-invasive evaluation of pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR). PAAT is dependent on PAP, PVR, pulmonary artery compliance, stroke volume, and heart rate. Its relative dependency on these determinants may differ between young and older children, raising uncertainty regarding its utility in young children.

Genetics dictating therapeutic decisions in pediatric pulmonary hypertension? A case report suggesting we are getting closer.

Despite therapeutic advances over the past decades, pulmonary arterial hypertension (PAH) and related pulmonary vascular diseases continue to cause significant morbidity and mortality in neonates, infants, and children. Unfortunately, an adequate understanding of underlying biology is lacking. There has been a growing interest in the role that genetic factors influence pulmonary vascular disease, with the hope that genetic information may aid in identifying disease etiologies, guide therapeutic decisions, and ultimately identify novel therapeutic targets.

Doppler Echocardiographic Features of Pulmonary Vein Stenosis in Ex-Preterm Children.

Background: Echocardiography is used to screen for the presence of pulmonary vein stenosis (PVS) in ex-preterm infants and children. However, there are no standard accepted criteria for screening or diagnosis of PVS by echocardiography. The aim of this study was to identify Doppler waveform features and Doppler systolic and diastolic velocity cutoff values associated with a diagnosis of PVS by cardiac catheterization.

Clinical Utility of Echocardiography in Former Preterm Infants with Bronchopulmonary Dysplasia.

Background: The clinical utility of echocardiography for the diagnosis of pulmonary vascular disease (PVD) in former preterm infants with bronchopulmonary dysplasia (BPD) is not established. Elevated pulmonary vascular resistance (PVR) rather than pulmonary artery pressure (PAP) is the hallmark of PVD. We evaluated the utility of echocardiography in infants with BPD in diagnosing pulmonary hypertension and PVD (PVR >3 Wood units × m) assessed by cardiac catheterization.

Development and Utility of Quality Metrics for Ambulatory Pediatric Cardiology in Kawasaki Disease.

The Adult Congenital and Pediatric Cardiology (ACPC) Section of the American College of Cardiology sought to develop quality indicators/metrics for ambulatory pediatric cardiology practice. The objective of this study was to report the creation of metrics for patients with Kawasaki disease. Over a period of 5 months, 12 pediatric cardiologists developed 24 quality metrics based on the most relevant statements, guidelines, and research studies pertaining to Kawasaki disease.

Mortality in infants with bronchopulmonary dysplasia: Data from cardiac catheterization.

Rationale: Pulmonary hypertension (PH) is relatively common in infants with severe bronchopulmonary dysplasia (BPD), however, hemodynamic data and factors associated with mortality in this patient group are sparsely described in the literature.

Objectives: To characterize the hemodynamics of former preterm infants with BPD and PH, as measured at cardiac catheterization, and to identify respiratory and cardiovascular predictors of mortality.

Methods: Single-center, retrospective cohort study, including, 30 patients born at less than 32-week gestational age (GA), who had an oxygen requirement at 36 weeks postmenstrual age and underwent cardiac catheterization between July 2014 and December 2017.

"There's no acknowledgement of what this does to people": A qualitative exploration of mental health among parents of children with critical congenital heart defects.

Aims And Objectives: The purpose of this study was to explore the psychological impact of parenting a child with a critical congenital heart defect and the feasibility and acceptability of integrating psychological services into paediatric cardiology care.

Background: Children with critical congenital heart defect are at an increased risk for long-term behavioural, social and emotional difficulties. Data suggest that this risk is partially attributable to parental mental health, which is a stronger predictor of long-term behavioural problems in congenital heart defect children than disease-specific and surgical factors.

Pushing the envelope: a treat and repair strategy for patients with advanced pulmonary hypertension associated with congenital heart disease.

Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease as a consequence of altered pulmonary hemodynamics with increased pulmonary blood flow and pressure. The development of pulmonary vascular disease (PVD) in this patient population is an important concern in determining operative strategy. Early, definitive surgical repair, when possible, is the best therapy to prevent and treat PVD.

Development of Quality Metrics in Ambulatory Pediatric Cardiology.

The American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Section had attempted to create quality metrics (QM) for ambulatory pediatric practice, but limited evidence made the process difficult. The ACPC sought to develop QMs for ambulatory pediatric cardiology practice. Five areas of interest were identified, and QMs were developed in a 2-step review process.

Mental Health Among Parents of Children With Critical Congenital Heart Defects: A Systematic Review.

Background: Parents of children with critical congenital heart defects (PCCHDs) may be at high risk for mental health morbidity; however, the literature is not well characterized. Given that compromised parental mental health can lead to long-term cognitive, health-related, and behavioral problems in children, a systematic review of this literature could provide informed recommendations for continued research and enhance the care of families of children living with critical congenital heart defects.

Methods And Results: We conducted a systematic review using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines that resulted in 30 studies on the mental health of PCCHDs.

Recognition of Undiagnosed Neonatal Heart Disease.

Heart defects are the most common congenital malformation. Approximately 8000 infants per year in the United States require diagnosis in the newborn period to avoid severe injury or death. It is incumbent on the neonatologist and pediatrician to expeditiously detect the presence of symptomatic heart disease so that infants can be stabilized before cardiovascular decompensation.

Outcomes of a modified approach to transcatheter closure of perimembranous ventricular septal defects.

Objectives: To describe the immediate and midterm results of a modified method for transcatheter closure of perimembranous ventricular septal defects (pmVSDs).

Background: Transcatheter closure of pmVSDs has been associated with development of heart block due to impingement on the ventricular conduction system. Ventricular septal aneurysms (VSAs) are common; the VSA tissue can serve as a target for the device without necessitating direct contact with the conduction system.

Use and performance of premounted stents compared to nonpremounted stents in pediatric and adult congenital cardiac catheterization.

Objectives: We sought to characterize the use, efficacy, and performance characteristics of premounted stents relative to nonpremounted stents when used during congenital cardiac catheterization.

Background: Endovascular stent implantation is an effective means of relieving vascular obstruction in patients with congenital heart disease. However, stent implantation is technically challenging and important complications occur.

Reporting adverse events in a surgical trial for complex congenital heart disease: the Pediatric Heart Network experience.

Objective: The purpose of this analysis was to evaluate a novel strategy for reporting adverse events in the Pediatric Heart Network's randomized surgical trial of systemic-pulmonary artery shunt versus right ventricle-pulmonary artery conduit in infants with hypoplastic left heart syndrome. The strategy was developed to align the reporting process with the needs of a surgical trial while maintaining participant safety.

Methods: Adverse event reporting was analyzed for 2 groups of study subjects: those randomized to a trial arm during a period in which a standard adverse event reporting system was used (period 1) and those randomized after institution of a system that focused serious adverse event reporting on 6 sentinel events (period 2).

Detection rates of congenital heart disease in Guatemala.

Objectives: In developing countries, congenital heart disease is often unrecognised, leading to serious morbidity and mortality. Guatemala is one of the few developing countries where expert paediatric cardiac treatment is available and affordable, and therefore early detection could significantly improve outcome. We assessed regional congenital heart disease detection rates in Guatemala, and determined whether they correlated with the regional human development index.

Anatomic and technical predictors of stent malposition during implantation for vascular obstruction in patients with congenital and acquired heart disease.

Objectives: We evaluated the anatomic and technical factors predicting stent malposition and embolization in patients undergoing endovascular stent implantation for relief of noncoronary vascular obstruction.

Background: Endovascular stent implantation provides a highly effective, minimally invasive solution to vascular obstruction in patients with structural heart disease. However, stent implantation is technically challenging and stent embolization occurs in up to 5.

Peripheral arterial function in infants and young children with one-ventricle physiology and hypoxemia.

Patients with 1-ventricle (1V) physiology may be at risk for peripheral arterial dysfunction at a young age. To determine whether infants and young children with 1V physiology and hypoxemia have peripheral arterial dysfunction before undergoing the Fontan operation, we measured (1) flow-mediated vasodilation (FMD) in the brachial artery, (2) serum levels of vasoactive mediators endothelin-1 (ET-1) and metabolites of nitric oxide, and (3) arterial stiffness with pulse-wave velocity (PWV) in the aorta. Eighteen patients with 1V physiology before the Fontan procedure and hypoxemia and 19 patients with normoxemia and 2-ventricle (2V) physiology were studied.