The International Society for Heart and Lung Transplantation Guidelines for the Management of Pediatric Heart Failure (Update From 2014).

Pediatric heart failure (HF) secondary to cardiomyopathies, acquired heart disease, and congenital heart disease is associated with significant morbidity and mortality. These guidelines represent an update from the International Society for Heart and Lung Transplantation guidelines for the management of pediatric HF that were published in 2014 and incorporate interval advancements in medical therapies and new approaches in the evaluation and management of children with HF.

Right Ventricular Dysfunction by Echocardiography Is an Early Marker of Evolving Cardiac Allograft Vasculopathy in Children After Heart Transplantation.

Background: Cardiac allograft vasculopathy (CAV) is the leading cause of late graft loss in children after pediatric heart transplantation (HTx). Coronary angiography, the reference standard for diagnosis, is invasive and carries a risk of complications. Noninvasive echocardiographic methods to reliably identify CAV in children have not yet been defined.

Beyond the consult: Outcomes following pediatric VAD referral.

Background: Little is known about patients who are referred for ventricular assist device (VAD) therapy but not implanted. The purpose of this study is to describe their outcomes at 1-year post initial consultation.

Methods: Retrospective analysis for patients referred to our VAD services between 01/2019 and 12/2023.

The Impact of Fontan Circulatory Failure on Heart Transplant Survival: A 20-Center Retrospective Cohort Study.

Background: Fontan circulatory failure (FCF) is a chronic state in palliated single ventricle heart disease with high morbidity and mortality rates, including heart failure, multisystem end-organ disease, and need for heart transplant. Specific FCF morbidities have not been rigorously defined, limiting study of how FCF morbidities affect pre-heart transplant and post-heart transplant outcomes. We hypothesized that FCF-related morbidities affect survival from heart transplant waitlisting through 1 year after heart transplant.

Contemporary outcomes of mechanical circulatory support using paracorporeal continuous flow pump in children: A report from the ACTION learning network.

Background: This study aims to evaluate the clinical outcomes of mechanical circulatory support (MCS) using paracorporeal continuous flow (pCF) pumps in children.

Methods: This multicenter retrospective study used the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) database (4/2018-7/2023). Children who were supported with a pCF pump were included.

Cardiac Medication Use in ACTION for Duchenne Muscular Dystrophy Cardiomyopathy.

This study seeks to understand cardiac medication use in a large cohort of males with Duchenne Muscular Dystrophy (DMD) followed prospectively with focus on current practices and adherence to consensus directed medical therapy (CDMT). DMD patients have been enrolled in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) Dystrophinopathy Registry since 2021. Cardiac medication use was analyzed at enrollment and most recent follow-up.

Evaluating the Prevalence of Optimal Neurodevelopmental Outcome at 2 Years in Children Previously on Ventricular Assist Device Support.

Background: Literature reporting neurodevelopmental outcomes for patients who undergo ventricular assist device (VAD) therapy is limited to posttransplant cohorts. This study aims to determine the prevalence of optimal neurodevelopmental outcome and factors associated with nonoptimal outcome in patients implanted with a VAD at ≤15 months of age.

Methods: Patients followed by the Complex Pediatric Therapies Follow-Up Program were included in a prospective-inception cohort study if born between January 2006 and December 2022 and implanted with a VAD at ≤15 months of age.

The approach to placement of a continuous-flow intracorporeal ventricular assist device in small left ventricle.

Background: The HeartMate 3 (HM3) has become among the most widely utilized durable left ventricular (LV) assist devices (LVAD) owing to reduced rates of pump thrombosis, bleeding, and stroke. One limitation of the HM3 is its large size, which poses a challenge for implantation in smaller LVs. Herein, we describe a novel technique for durable LVAD implantation in patients with a small LV.

Cardiac allograft vasculopathy and the endothelial glycocalyx: a missing link?

Cardiac allograft vasculopathy (CAV) is a significant contributor to graft loss following heart transplantation, with a linear cumulative incidence over time. Both immune and non-immune risk factors are associated with the development of CAV, however, a cohesive mechanistic link between them is yet to be established. Immune and non-immune risk factors may be linked to CAV via disturbance of the endothelial glycocalyx (EGX), a protective vascular structure whose functions appear to be impaired in the context of CAV progression.

Ventricular Assist Device Use in Pediatric Restrictive and Hypertrophic Cardiomyopathy: An ACTION Registry Analysis.

The use of ventricular assist devices (VADs) in children with restrictive (RCM) and hypertrophic cardiomyopathy (HCM) remains rare. We describe the outcomes of patients with RCM and HCM supported by VAD in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry from March 2012 to December 2024. Thirty-four patients were identified: 20 left-sided VAD (LVAD), 13 biventricular VAD (BiVAD), and 1 total artificial heart (TAH).

Rejection and graft vasculopathy secondary to effects of Crohn's disease in a heart-transplanted child.

Patients require immunosuppression after heart transplantation. Conditions such as Crohn's disease can impact tacrolimus absorption and pharmacokinetics. Subtherapeutic tacrolimus levels can lead to rejection and development of donor-specific antibodies (DSA), resulting in the development of cardiac allograft vasculopathy.

Concomitant Surgical Procedures and Aspirin Avoidance With Left Ventricular Assist Device Therapy.

Background: ARIES-HM3 (Antiplatelet Removal and Hemocompatibility Events With the HeartMate 3 Pump) demonstrated that aspirin avoidance with a fully magnetically levitated HeartMate 3 (HM3) left ventricular assist device (LVAD) reduces bleeding complications and does not increase thromboembolism. Whether a concomitant surgical procedure modifies the observed safety and benefits remains uncertain.

Objectives: This prespecified analysis of ARIES-HM3 studied clinical outcomes when concomitant surgical procedures are performed during LVAD implantation with excluding aspirin but maintaining a vitamin K antagonist.

Severe primary graft failure: Are there lasting impacts? Analysis from the PHTS Database.

Background: Primary graft failure (PGF) is a leading cause of early morbidity and mortality after heart transplantation (HTx). PGF is secondary to graft ischemia and ischemia-reperfusion injuries to the cardiomyocytes and vasculature of the donor heart after transplantation. Longer-term outcomes after PGF are not well studied.

Methylated Arginine Metabolites as Biomarkers for Clinical Status and Response to Type 5 Phosphodiesterase Inhibition in Patients With Fontan Circulation.

Background: There is significant interest in NO pathway modulators, specifically type 5 phosphodiesterase inhibitors (PDE5is), to treat patients with a Fontan circulation. Trials, however, have had mixed results. The relationship between the NO pathway and clinical status in patients with Fontan circulation is a significant knowledge gap.

Cardiac treatment for Duchenne muscular dystrophy: consensus recommendations from the ACTION muscular dystrophy committee.

Introduction: Duchenne muscular dystrophy is a devastating neuromuscular disorder characterized by the loss of dystrophin, inevitably leading to cardiomyopathy. Despite publications on prophylaxis and treatment with cardiac medications to mitigate cardiomyopathy progression, gaps remain in the specifics of medication initiation and optimization.

Method: This document is an expert opinion statement, addressing a critical gap in cardiac care for Duchenne muscular dystrophy.

The role of diagnostic modalities in differentiating hypertensive heart disease and hypertrophic cardiomyopathy: strategies in adults for potential application in paediatrics.

Hypertensive heart disease and hypertrophic cardiomyopathy both lead to left ventricular hypertrophy despite differing in aetiology. Elucidating the correct aetiology of the presenting hypertrophy can be a challenge for clinicians, especially in patients with overlapping risk factors. Furthermore, drugs typically used to combat hypertensive heart disease may be contraindicated for the treatment of hypertrophic cardiomyopathy, making the correct diagnosis imperative.

The ACTION VAD registry: A collective five-year experience.

Background: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) began in 2018 as a collaborative learning health system committed to improving outcomes in pediatric heart failure, including children and adults with congenital heart disease, supported with ventricular assist devices (VADs). This report describes patient and device characteristics, and outcomes through 1-year post-implant.

Methods: The ACTION VAD registry report was created from data submitted to the ACTION learning network from April 2018 to June 2023.

Modifiable Health Behaviours in Children with HCM: Lessons from the Heart Health Survey.

Obesity factors into hypertrophic cardiomyopathy (HCM)-related risk as a disease modifying environmental factor. Behaviours such as diet and sleep are seldom reported upon in children with HCM. It was our aim to report on these factors in this population.

Aspirin and Hemocompatibility After LVAD Implantation in Patients With Atherosclerotic Vascular Disease: A Secondary Analysis From the ARIES-HM3 Randomized Clinical Trial.

Importance: The Aspirin and Hemocompatibility Events With a Left Ventricular Assist Device in Advanced Heart Failure (ARIES-HM3) study demonstrated that aspirin may be safely eliminated from the antithrombotic regimen after HeartMate 3 (HM3 [Abbott Cardiovascular]) left ventricular assist device (LVAD) implantation. This prespecified analysis explored whether conditions requiring aspirin (prior percutaneous coronary intervention [PCI], coronary artery bypass grafting [CABG], stroke, or peripheral vascular disease [PVD]) would influence outcomes differentially with aspirin avoidance.

Objective: To analyze aspirin avoidance on hemocompatibility-related adverse events (HRAEs) at 1 year after implant in patients with a history of CABG, PCI, stroke, or PVD.

Subnormothermic Machine Perfusion of Neonatal and Small-Sized Pediatric Donor Hearts.

Donor heart machine perfusion enables avoidance of prolonged cold ischemia, however the optimal temperature is yet to be elucidated. Given that maintenance of temperature beyond ambient levels demands significant energy, we sought to determine the suitability of room-temperature perfusion preservation of neonatal/pediatric-sized (5-20 kg) piglet donor hearts. A custom device was fabricated suitable for this purpose, with continuous readout of perfusion pressure, flow rate, temperature, and oxygen saturation.

Impact of Remoteness of Residence and Socioeconomic Status on Outcomes Among Children With Heart Disease in Alberta.

Background: There is a paucity of data regarding the impact of remoteness of residence (RoR) and socioeconomic status (SES) on access to care and outcomes for children with congenital heart disease (CHD) or acquired heart disease (AHD) in a jurisdiction of universal health and centralized cardiac care.

Objectives: The primary objective was to examine whether RoR, SES, and their interaction impact access to health care and outcomes for children with heart disease in Alberta, Canada.

Methods: This was a population-based study of children with CHD or AHD born between January 1, 2005, and December 31, 2017, in Alberta, Canada.