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.

Hypertrophic cardiomyopathy in Duchenne muscular dystrophy: a case series.

Duchenne muscular dystrophy is characterised by fibrofatty replacement of muscle, resulting in dilated cardiomyopathy. Hypertrophic cardiomyopathy affects 1:200-1:500 people and is characterised by asymmetric ventricular septal hypertrophy. To date, there have been two separately reported cases describing the combined pathology of these disorders.

Evolocumab treatment reduces carotid intima-media thickness in paediatric patients with heterozygous familial hypercholesterolaemia.

Aim: Children with heterozygous familial hypercholesterolaemia (HeFH) show greater carotid intima-media thickness (cIMT). Evolocumab, a proprotein convertase subtilisin/kexin type 9 inhibitor monoclonal antibody, substantially reduced low-density lipoprotein cholesterol (LDL-C) and modestly reduced lipoprotein(a) in children with HeFH. We investigated evolocumab's effect on cIMT progression.

Desmoplakin Cardiomyopathy in Pediatric Patients: A Distinct, Underrecognized Cohort of Arrhythmogenic Cardiomyopathy.

Background: cardiomyopathy is a distinct subset of arrhythmogenic cardiomyopathy, reported primarily in adults, that has predominantly left ventricular involvement and features of myocarditis. Clinical characteristics, risk stratification, and management of pediatric patients with variants are not well known. We sought to identify phenotypic features and prognosis of pediatric patients with pathogenic or likely pathogenic variants.

Animal Models for Mechanical Circulatory Support: A Research Review.

Heart failure is a clinical syndrome that has become a leading public health problem worldwide. Globally, nearly 64 million individuals are currently affected by heart failure, causing considerable medical, financial, and social challenges. One therapeutic option for patients with advanced heart failure is mechanical circulatory support (MCS) which is widely used for short-term or long-term management.

Use of exclusive human milk diet in a neonate with single ventricle physiology supported on a ventricular assist device as a bridge to heart transplantation: case report.

Malnutrition is common among paediatric heart failure patients, with nutritional rehabilitation critical for survival and optimal health outcomes. Ventricular assist devices have been associated with improved growth, though additional nutritional support may be needed. Here, we report the use of human milk-based fortifiers to avoid severe malnutrition in a neonate supported on a ventricular assist device until transplantation.

Abnormal blood pressure among individuals evaluated for tuberculosis infection in a U.S. public health tuberculosis clinic.

Tuberculosis infection (TBI) has been associated with increased cardiovascular risks. We aimed to characterize abnormal blood pressure (BP) readings in individuals with TBI. We conducted a retrospective study of adults with TBI presenting for their initial medical visit at a large midwestern U.

Another-regulin regulates cardiomyocyte calcium handling via integration of neuroendocrine signaling with SERCA2a activity.

Calcium (Ca) dysregulation is a hallmark feature of cardiovascular disease. Intracellular Ca regulation is essential for proper heart function and is controlled by the sarco/endoplasmic reticulum Ca ATPase (SERCA2a). Another-regulin (ALN) is a newly discovered cardiomyocyte-expressed SERCA2a inhibitor, suggesting cardiomyocyte Ca-handling is more complex than previously appreciated.

Palliative Care Referral Patterns and Implications for Standardization in Cardiac ICU.

Evidence suggests that pediatric palliative care involvement (PPC) is beneficial to medically complex patients. Historically, PPC involvement has been overlooked or delayed and varies by institution but PPC awareness has increased in cardiovascular ICUs (CVICU) and so we investigated frequency and timeliness of PPC referral for patients dying in ICU. Retrospective study of pediatric cardiac patients experiencing death in ICU to review PPC presence and timing of initial PPC, most recent PPC, and interventions, therapies, CPR, and presence of do-not-resuscitate DNR discussion.