Formulation of Quality Improvement Methodology for Risk Mitigation in Congenital Cardiac Catheterization.

The C3PO collaborative, with a history of successful quality improvement (QI) initiatives, leveraged registry participants to develop a multi-center QI initiative to reduce adverse events (AEs) in congenital cardiac catheterization. A 32-person, interdisciplinary working group analyzed audited data for all congenital cardiac catheterization cases from 2014-2017. The primary outcome was the occurrence of any high-severity (level 3/4/5) AE.

Percutaneous Mechanical Aspiration for Infective Endocarditis: Proceedings From an Inaugural Multidisciplinary Summit and Comprehensive Review.

The clinical presentation and epidemiology of infective endocarditis (IE) have evolved over time. While the cornerstones of IE treatment remain antimicrobial therapy and surgery, percutaneous mechanical aspiration (PMA) has emerged as an option for carefully selected patients as a complementary modality, based on retrospective data, case series, and expert experience. In this comprehensive review, we summarize the proceedings from an inaugural summit dedicated to the discussion of PMA in the global management of IE, consisting of experts across multiple disciplines from diverse geographic regions and care environments.

Six-Month Outcomes in the Long-Term Outcomes After the Multisystem Inflammatory Syndrome in Children Study.

Importance: Multisystem inflammatory syndrome in children (MIS-C) is a life-threatening complication of COVID-19 infection. Data on midterm outcomes are limited.

Objective: To characterize the frequency and time course of cardiac dysfunction (left ventricular ejection fraction [LVEF] <55%), coronary artery aneurysms (z score ≥2.

Asian Pacific Society of Cardiology Position Statement on the Use of Transcatheter Aortic Valve Implantation in the Management of Aortic Stenosis.

Transcatheter aortic valve implantation (TAVI) has been established as an effective treatment modality in patients with severe aortic stenosis (AS) and the uptake of TAVI is rapidly growing in the Asia-Pacific region. However, there exist a heterogeneity in the management of aortic stenosis and the use of TAVI among countries in the region. Reasons for these differences include anatomic variations, disparity in healthcare resources and infrastructure, and the lack of consensus on the optimal management of AS in the Asia-Pacific region.

Extracorporeal Membrane Oxygenation in Children with Pulmonary Atresia and Intact Ventricular Septum: Mortality and Associated Outcomes.

Data on outcomes of extracorporeal membrane oxygenation (ECMO) are limited in patients with pulmonary atresia intact ventricular septum (PAIVS). The objective of this study was to describe the use of ECMO and the associated outcomes in patients with PAIVS. We retrospectively reviewed neonates with PAIVS who received ECMO between 2009 and 2019 in 19 US hospitals affiliated with the Collaborative Research for the Pediatric Cardiac Intensive Care Society (CoRe-PCICS).

Preoperative Computed Tomography Virtual Simulation for HeartMate 3 Implantation in Small Children.

An implantable ventricular assist device became smaller and has been used for small body size patients. However, it is still challenging to determine whether it is implantable for pediatric patients. The preoperative computed tomography virtual simulation provided spatial information among the pump, intracardiac structures, and extracardiac structures, which was very useful to assess the implantability for borderline children.

Mechanisms of exercise-related neurocardiogenic syncope and the relationship between resting and dynamic cardiac testing.

Objective: Syncope is common among pediatric patients and is rarely pathologic. The mechanisms for symptoms during exercise are less well understood than the resting mechanisms. Additionally, inert gas rebreathing analysis, a non-invasive examination of haemodynamics including cardiac output, has not previously been studied in youth with neurocardiogenic syncope.

Anomalous Left Coronary Artery from the Pulmonary Artery in Three Patients with MYRF-Associated Cardiac-Urogenital Syndrome.

Cardiac-Urogenital Syndrome (CUGS) is a recently identified genetic disease characterized by urogenital, diaphragmatic, ophthalmic, and cardiac abnormalities caused by heterozygous pathogenic variants in the Myelin Regulatory Factor (MYRF) gene. The complete spectrum of disease characteristics and prevalence is not yet defined. This report documents the first known cases of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in MYRF-associated Cardiac-Urogenital Syndrome (MYRF-CUGS).

Fate of Semilunar Valves From Discarded Hearts at the Time of Pediatric Heart Retransplantation.

Background: Partial heart transplantation (PHT) is a novel procedure for children in need of a growing valve replacement option. One challenge is identifying suitable donor valves. Semilunar heart valves from patients receiving a retransplant may be a source, however their functionality and growth potential especially at the time of retransplant are unknown.

Klotho mutation does not accelerate intervertebral disc aging in mice.

Aging is a risk factor for several chronic conditions, including intervertebral disc degeneration and associated back pain. Disc pathologies include loss of reticular-shaped nucleus pulposus cells, disorganization of annulus fibrosus lamellae, reduced disc height, and increased disc bulging. Sonic hedgehog, cytokeratin 19, and extracellular matrix proteins are markers of healthy disc.

Percutaneous photoacoustic debulking of infra-inguinal atherosclerotic disease- early European experience with a novel, solid-state, pulsed -wave, ultraviolet 355 nm laser.

The broad spectrum of clinical manifestations caused by peripheral arterial disease [PAD] and the morphologic heterogeneity of associated atherosclerotic lesions present a considerable management challenge. Endovascular interventions are recognized an effective treatment for PAD. Within this revascularization strategy the role of atherectomy debulking modalities continue to evolve.

Non-invasive pressure-volume loop derived temporal elastance, contractility, and efficiency indices for assessing Duchenne muscular dystrophy patients.

Ejection fraction is commonly used to assess Duchenne muscular dystrophy-associated cardiomyopathy (DMDAC), but it may remain normal (wrongly) despite significant myocardial dysfunction in patients. Therefore, better indicators of myocardial dysfunction are needed for longitudinal (with time) assessment and treatment of DMDAC patients. This study evaluates non-invasive LV PV loop-derived elastance, contractility and efficiency in relation to EF for patients developing DMDAC.

Dilated cardiomyopathy variant R14del increases phospholamban pentamer stability, blunting dynamic regulation of calcium.

The sarco(endo)plasmic reticulum Ca ATPase (SERCA) is a membrane transporter that creates and maintains intracellular Ca stores. In the heart, SERCA is regulated by an inhibitory interaction with the monomeric form of the transmembrane micropeptide phospholamban (PLB). PLB also forms avid homo-pentamers, and dynamic exchange of PLB between pentamers and SERCA is an important determinant of cardiac responsiveness to exercise.

Covered Stent Correction for Sinus Venosus Atrial Septal Defects, an Emerging Alternative to Surgical Repair: Results of an International Registry.

Background: Covered stent correction for a sinus venosus atrial septal defect (SVASD) was first performed in 2009. This innovative approach was initially viewed as experimental and was reserved for highly selected patients with unusual anatomic variants. In 2016, increasing numbers of procedures began to be performed, and in several centers, it is now offered as a standard of care option alongside surgical repair.

Can we diagnose aortic arch obstruction in a fetus with an atrioventricular septal defect?

Background: Aortic arch obstruction and/or coarctation of aorta is uncommon (5-20%) in balanced atrioventricular septal defects. Although technically challenging, prenatal diagnosis of aortic arch obstruction in atrioventricular septal defect is critical for delivery planning and improves prenatal counselling regarding the timing of cardiac surgery. We sought to identify prenatal predictors of coarctation of aorta in atrioventricular septal defect.

Current state of neurodevelopmental and psychosocial care practices for paediatric patients with ventricular assist devices: an Advanced Cardiac Therapies Improving Outcomes Network and Cardiac Neurodevelopmental Outcome Collaborative collaborative survey.

Purpose: Paediatric patients with heart failure requiring ventricular assist devices are at heightened risk of neurologic injury and psychosocial adjustment challenges, resulting in a need for neurodevelopmental and psychosocial support following device placement. Through a descriptive survey developed in collaboration by the Advanced Cardiac Therapies Improving Outcomes Network and the Cardiac Neurodevelopmental Outcome Collaborative, the present study aimed to characterise current neurodevelopmental and psychosocial care practices for paediatric patients with ventricular assist devices.

Method: Members of both learning networks developed a 25-item electronic survey assessing neurodevelopmental and psychosocial care practices specific to paediatric ventricular assist device patients.

SOX17-Associated Pulmonary Hypertension in Children: A Distinct Developmental and Clinical Syndrome.

Objective: To characterize clinical, hemodynamic, imaging, and pathologic findings in children with pulmonary arterial hypertension (PAH) and variants in SRY-box transcription factor 17 (SOX17), a novel risk gene linked to heritable and congenital heart disease-associated PAH.

Study Design: We assembled a multi-institutional cohort of children with PAH and SOX17 variants enrolled in the Pediatric Pulmonary Hypertension Network (PPHNet) and other registries. Subjects were identified through exome and PAH gene panel sequencing.

Elevating diversity, inclusion, and health equity in Pediatric Heart Network Scholars grant funding: unique opportunities and lessons learned.

There is a growing awareness that diversity, health equity, and inclusion play a significant role in improving patient outcomes and advancing knowledge. The Pediatric Heart Network launched an initiative to incorporate diversity, health equity, and inclusion into its 2021 Scholar Award Funding Opportunity Announcement. This manuscript describes the process of incorporating diversity, health equity, and inclusion into the Pediatric Heart Network Scholar Award and the lessons learned.

Hemostatic derangements associated with cardiopulmonary bypass predict outcomes in pediatric patients undergoing corrective heart surgery.

Background: Understanding of the hemostatic and complement alterations associated with cardiopulmonary bypass (CPB) in pediatric patients and the impact of these alterations on outcome is limited.

Objectives: The present study prospectively characterized these alterations and their association with postoperative outcomes in pediatric CPB.

Methods: All patients aged <21 years undergoing CPB at the authors' institution between 2020 and 2021 who weighed >3 kg, were >36 weeks gestational age, and had no known prothrombotic or hemorrhagic disorders were eligible.

A Single-Center Review of Clinical Outcomes After Transcatheter Relief of Superior Vena Cava Stenosis in a Pediatric and Young Adult Population.

Background: The suspected etiology of superior vena cava (SVC) obstruction is predominantly iatrogenic. Transcatheter interventions relieve stenosis with good long-term results, although there is a paucity of published data in pediatrics. We aim to describe patient characteristics and clinical outcomes after transcatheter SVC intervention at a single-center pediatric quaternary care center.