Seasons of Kawasaki Disease during the COVID-19 pandemic.

The incidence of Kawasaki Disease has a peak in the winter months with a trough in late summer/early fall. Environmental/exposure factors have been associated with a time-varying incidence. These factors were altered during the COVID-19 pandemic.

Factors Associated With Shock at Presentation in Kawasaki Disease Versus Multisystem Inflammatory Syndrome in Children Associated With Covid-19.

Background: Although clinical overlap between Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) has been evident, information regarding those presenting with shock has been limited. We sought to determine associations with shock within and between diagnosis groups.

Methods: The International KD Registry enrolled contemporaneous patients with either KD or MIS-C from 39 sites in 7 countries from January 1, 2020, to January 1, 2023.

Rationale and design of CHD PULSE: Congenital Heart Disease Project to Understand Lifelong Survivor Experience.

Background: With improved survival of adults with congenital heart disease (CHD) comes a need to understand the lifelong outcomes of this population. The aim of this paper is to describe the rationale and design of Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE), a study to determine long-term medical, neurocognitive, and psychosocial outcomes among adults with a history of intervention for CHD and to identify factors associated with those outcomes.

Methods: CHD PULSE is a cross-sectional survey conducted from September 2021 to April 2023 among adults aged 18 and older with a history of at least 1 intervention for CHD at 1 of 11 participating U.

Cardiac Biomarkers Aid in Differentiation of Kawasaki Disease from Multisystem Inflammatory Syndrome in Children Associated with COVID-19.

Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 show clinical overlap and both lack definitive diagnostic testing, making differentiation challenging. We sought to determine how cardiac biomarkers might differentiate KD from MIS-C. The International Kawasaki Disease Registry enrolled contemporaneous KD and MIS-C pediatric patients from 42 sites from January 2020 through June 2022.

Obesity and Outcomes of Kawasaki Disease and COVID-19-Related Multisystem Inflammatory Syndrome in Children.

Importance: Obesity may affect the clinical course of Kawasaki disease (KD) in children and multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19.

Objective: To compare the prevalence of obesity and associations with clinical outcomes in patients with KD or MIS-C.

Design, Setting, And Participants: In this cohort study, analysis of International Kawasaki Disease Registry (IKDR) data on contemporaneous patients was conducted between January 1, 2020, and July 31, 2022 (42 sites, 8 countries).

Toward a Roadmap for Best Practices in Pediatric Preventive Cardiology: A Science Advisory From the American Heart Association.

Cardiovascular disease risk factors are highly prevalent among youth in the United States and Canada. Pediatric preventive cardiology programs have independently developed and proliferated to address cardiovascular risk factors in youth, but there is a general lack of clarity on best practices to optimize and sustain desired outcomes. We conducted surveys of pediatric cardiology division directors and pediatric preventive cardiology clinicians across the United States and Canada to describe the current landscape and perspectives on future directions for the field.

Survival After Single-Stage Repair of Truncus Arteriosus and Associated Defects.

Background: The goal of this study was to describe in-hospital and long-term mortality after single-stage repair of truncus arteriosus communis (TAC) and explore factors associated with these outcomes.

Methods: This was a cohort study of consecutive patients undergoing single-stage TAC repair between 1982 and 2011 reported to the Pediatric Cardiac Care Consortium registry. In-hospital mortality was obtained for the entire cohort from registry records.

COVID-19 Positive Versus Negative Complete Kawasaki Disease: A Study from the International Kawasaki Disease Registry.

To determine clinical differences for children with complete Kawasaki disease (KD) with and without evidence of preceding SARS-CoV-2 infection. From January 2020, contemporaneous patients with complete KD criteria were classified as either SARS-CoV-2 positive (KDCOVID+; confirmed household exposure, positive PCR and/or serology) or SARS-CoV-2 negative (KDCOVID-; negative testing and no exposure) and compared. Of 744 patients in the International Kawasaki Disease Registry, 52 were KDCOVID- and 61 were KDCOVID+.

The influence of somatic maturity on the relationship between the triglyceride/high-density lipoprotein ratio and vascular health in children and adolescents with dyslipidemia.

Introduction: Both the triglyceride to HDL cholesterol (TG/HDL) ratio and timing of pubertal maturation have been identified as independent contributors to the development of atherosclerosis.

Objective: The purpose of our study was to determine the relationship between the TG/HDL ratio and measures of vascular health in children and adolescents with dyslipidemia stratified by somatic maturity. We hypothesized that somatic maturity would have a significant interaction with TG/HDL ratio and vascular health.

Association of Acute Anti-inflammatory Treatment With Medium-term Outcomes for Coronary Artery Aneurysms in Kawasaki Disease.

Background: The impact of adjunctive anti-inflammatory treatment on outcomes for patients with Kawasaki disease (KD) and coronary artery aneurysms (CAAs) is unknown.

Methods: Using data from the International KD Registry in patients with ≥ medium CAA we evaluate associations of treatment with outcomes and major adverse cardiac events (MACE).

Results: Medium or large CAA was present in 527 (32%) patients.

Outcomes Following Aortic Valve Replacement in Children With Conotruncal Anomalies.

Objective: Conotruncal anomalies can develop aortopathy and/or aortic valve (AV) disease and AV replacement (AVR) is occasionally needed. We report long-term results and examine factors affecting survival following AVR in this group.

Methods: We queried the Pediatric Cardiac Care Consortium (PCCC, US database for interventions for congenital heart diseases) to identify patients with repaired conotruncal anomalies and AVR.

Multicentre comparative analysis of long-term outcomes after aortic valve replacement in children.

Objective: The ideal valve substitute for surgical intervention of congenital aortic valve disease in children remains unclear. Data on outcomes beyond 10-15 years after valve replacement are limited but important for evaluating substitute longevity. We aimed to describe up to 25-year death/cardiac transplant by type of valve substitute and assess the potential impact of treatment centre.

Variation in Pharmacologic Management of Patients with Kawasaki Disease with Coronary Artery Aneurysms.

Objective: To evaluate practice variation in pharmacologic management in the International Kawasaki Disease Registry (IKDR).

Study Design: Practice variation in intravenous immunoglobulin (IVIG) therapy, anti-inflammatory agents, statins, beta-blockers, antiplatelet therapy, and anticoagulation was described.

Results: We included 1627 patients from 30 IKDR centers with maximum coronary artery aneurysm (CAA) z scores 2.

Brain Abscess and the Nonfenestrated Fontan Circulation.

Brain abscess is a rare but life-threatening condition. Intracardiac or extracardiac right-to-left shunting in patients with unrepaired cyanotic congenital heart disease, pulmonary arteriovenous malformations, or venovenous collaterals allows microbes unfiltered access to the brain. Brain abscess must be considered when cyanotic patients present with headache.

Impact of Major Residual Lesions on Outcomes After Surgery for Congenital Heart Disease.

Background: Many factors affect outcomes after congenital cardiac surgery.

Objectives: The RLS (Residual Lesion Score) study explored the impact of severity of residual lesions on post-operative outcomes across operations of varying complexity.

Methods: In a prospective, multicenter, observational study, 17 sites enrolled 1,149 infants undergoing 5 common operations: tetralogy of Fallot repair (n = 250), complete atrioventricular septal defect repair (n = 249), arterial switch operation (n = 251), coarctation or interrupted arch with ventricular septal defect (VSD) repair (n = 150), and Norwood operation (n = 249).

Are Mechanical Prostheses Valid Alternatives to the Ross Procedure in Young Children Under 6 Years Old?

Background: Aortic valve replacement in young children is associated with technical difficulties and potential morbidity. In contrast to the versatile Ross operation, mechanical prostheses (MP) are uncommonly used.

Methods: We examined transplant-free survival and cardiac reoperation among 124 young children (aged 1-6 years) who underwent the Ross operation (n = 84) or MP (n = 40) for congenital disease (1982-2003) using the Pediatric Cardiac Care Consortium database.

Management of Multisystem Inflammatory Syndrome in Children Associated With COVID-19: A Survey From the International Kawasaki Disease Registry.

Background: Since April 2020, there have been numerous reports of children presenting with systemic inflammation, often in critical condition, and with evidence of recent infection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This condition, since defined as the multisystem inflammatory syndrome in children (MIS-C), is assumed to be a delayed immune response to coronavirus disease 2019 (COVID-19), and there are frequently cardiac manifestations of ventricular dysfunction and/or coronary artery dilation.

Methods: We surveyed the inpatient MIS-C management approaches of the members of the International Kawasaki Disease Registry across 38 institutions and 11 countries.

Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From the International Kawasaki Disease Registry.

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD.

Cardiorespiratory Fitness in Youth: An Important Marker of Health: A Scientific Statement From the American Heart Association.

Cardiorespiratory fitness (CRF) refers to the capacity of the circulatory and respiratory systems to supply oxygen to skeletal muscle mitochondria for energy production needed during physical activity. CRF is an important marker of physical and mental health and academic achievement in youth. However, only 40% of US youth are currently believed to have healthy CRF.

Low-Molecular-Weight Heparin vs Warfarin for Thromboprophylaxis in Children With Coronary Artery Aneurysms After Kawasaki Disease: A Pragmatic Registry Trial.

Background: The substantial risk of thrombosis in large coronary artery aneurysms (CAAs) (maximum z-score ≥ 10) after Kawasaki disease (KD) mandates effective thromboprophylaxis. We sought to determine the effectiveness of anticoagulation (low-molecular-weight heparin [LMWH] or warfarin) for thromboprophylaxis in large CAAs.

Methods: Data from 383 patients enrolled in the International KD Registry (IKDR) were used.