End-of-Life Course and Subspecialty Palliative Care Involvement for Children on Mechanical Circulatory Support: Five-Center Retrospective Cohort Study from the United States, 2015-2020.

Objectives: To characterize end-of-life (EOL) care and subspecialty palliative care (SPC) involvement in children with heart disease supported on mechanical circulatory support (MCS), including ventricular assist devices (VADs) and extracorporeal membrane oxygenation (ECMO).

Design: A multicenter retrospective study describing clinical characteristics, SPC involvement and timing, and EOL circumstances.

Setting: Five U.

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.

Multi-institutional outcomes of Impella use in pediatric patients: A brief communication from the ACTION Network.

This retrospective cohort study includes 150 pediatric patients supported with the Impella between April 2018 and November 2024. Median weight was 62.9 kg (interquartile range [IQR] 51.

Ventricular assist device implantation in children with a mechanical valve: An ACTION registry analysis.

Background: Patients with congenital heart disease (CHD) frequently have had valve interventions, including replacement with a mechanical valve (mechV). The impact of a mechV on clinical outcomes in patients undergoing ventricular assist device (VAD) implantation is not well characterized.

Objectives: This study assessed VAD outcomes in patients with CHD and a mechV.

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.

Outcomes for Children With Congenital Heart Disease Undergoing Ventricular Assist Device Implantation: An ACTION Registry Analysis.

Background: There are no contemporary reports that highlight the national outcomes for children with congenital heart disease (CHD) undergoing ventricular assist device (VAD) implantation.

Objectives: This study sought to evaluate differences in VAD outcomes for children with CHD to those with non-CHD as well as those with univentricular CHD to those with biventricular CHD.

Methods: Data for CHD and non-CHD patients from the multicenter ACTION (Advanced Cardiac Therapies Improving Outcomes Network) undergoing VAD implantation from April 2018 to February 2023 were included.

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.

Histopathology, mRNA expression profile, and donor-derived cell-free DNA for assessment of rejection in pediatric heart transplantation.

Background: The relationship between histopathologic and molecular ("MMDx"®) assessments of endomyocardial biopsy (EMB) and serum donor-derived cell-free DNA (ddcfDNA) in acute rejection (AR) assessment following pediatric heart transplantation (HT) is unknown.

Methods: EMB sent for MMDx and histopathology from November 2021 to September 2022 were reviewed. MMDx and histopathology results were compared.

Impact of HeartWare ventricular assist device discontinuation on the pediatric population: An Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry analysis.

Background: The HeartWare ventricular assist device (HVAD) was discontinued in July 2021. The study aims to describe the impact the discontinuation the HVAD had on pediatric ventricular assist device (VAD) utilization and outcomes.

Methods: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry was queried for type of VAD utilization and outcomes/adverse event rates in patients with a body surface area of 0.

Seventh Annual Society of Thoracic Surgeons Pedimacs Report.

Background: The Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), supported by The Society of Thoracic Surgeons, provides detailed information on pediatric patients supported with ventricular assist devices (VADs).

Methods: From September 19, 2012, to December 31, 2022, 1463 devices in 1219 patients aged <19 years were reported to the registry from 40 North American hospitals.

Results: Cardiomyopathy remains the most common underlying etiology (59%), followed by congenital heart disease (26%) and myocarditis (8%).

Outcomes after initial heart failure consultation in Fontan patients.

Background: Patients with Fontan failure are high-risk candidates for heart transplantation and other advanced therapies. Understanding the outcomes following initial heart failure consultation can help define appropriate timing of referral for advanced heart failure care.

Methods: This is a survey study of heart failure providers seeing any Fontan patient for initial heart failure care.

Multicenter outcomes for ventricular assist device support for failed stage II palliation.

Background: Ventricular assist device (VAD) use for failed stage II palliation (S2P) is increasing with limited data on outcomes.

Methods: To address this knowledge gap, we conducted a multicenter retrospective review of the Advanced Cardiac Therapies Improving Outcomes Network registry. We leveraged the registry to analyze data on the clinical course, complications, and survival of systemic VADs (SVAD) after S2P.

Clinical Outcomes of SARS-CoV-2 Infection in Pediatric Patients on Ventricular Assist Device Support: An ACTION Registry Analysis.

Adult patients on left ventricular assist device (LVAD) support have increased morbidity and mortality after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. There are no reported clinical data describing outcomes among pediatric patients on ventricular assist device (VAD) support infected with SARS-CoV-2. We conducted a retrospective study using the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry to evaluate patient characteristics and clinical outcomes after SARS-CoV-2 infection.

Prenatally Diagnosed Interventricular Septal Aneurysm with Associated Ventricular Dysfunction.

Congenital interventricular septal aneurysms (IVSA) of the muscular septum are rare and can be associated with other familial abnormalities of the ventricular septum, arrhythmias, additional congenital heart disease, and chromosomal abnormalities. IVSA is also linked to ventricular dysfunction and non-compaction, although there are limited reports of this association presenting in utero. We describe a case of fetal ventricular septal aneurysm associated with ventricular dysfunction and pericardial effusion.

Contemporary Care and Outcomes of Critically-ill Children With Clinically Diagnosed Myocarditis.

Purpose: To describe contemporary management and outcomes in children with myocarditis who are admitted to a cardiac intensive care unit (CICU) and to identify the characteristics associated with mortality.

Methods: All patients in the Pediatric Cardiac Critical Care Consortium (PC) registry between August 2014 and June 2021 who were diagnosed with myocarditis were included. Univariable analyses and multivariable logistic regression evaluated the factors associated with in-hospital mortality.

Interventricular Septal Hematoma Complicating Ventricular Assist Device Placement in an Infant: Case Report and Review of the Literature.

Interventricular septal hematoma is a rare and life-threatening complication of pediatric cardiac surgery. Commonly seen following ventricular septal defect repair, it has also been associated with ventricular assist device (VAD) placement. Although conservative management is usually successful, operative drainage of interventricular septal hematoma occurring in pediatric patients undergoing VAD implantation should be considered.

Stroke Diagnosis Protocol for Children With Ventricular Assist Devices.

Ventricular assist devices (VADs) are increasingly used for end-stage heart failure in children. VAD-associated neurologic dysfunction, including stroke and intracranial hemorrhage, occurs in more than 20% of patients. Starting in 2019, we implemented a protocol to diagnose stroke in relation to VAD to facilitate treatment.

Ventricular Assist Device Outcomes in Infants and Children With Stage 1 Single Ventricle Palliation.

Data on ventricular assist device (VAD) outcomes in infants with stage 1 single ventricle (SV) palliation are limited. We examined the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry for outcomes of pre/poststage 1 SV patients undergoing VAD implantation between March 2018 and October 2020. Data are collected from 32 centers and major adverse events undergo central adjudication.

Waitlist and post-transplant outcomes for children with myocarditis listed for heart transplantation over 3 decades.

Background: There is limited and conflicting information on waitlist and transplant outcomes for children with myocarditis.

Methods: Retrospective review included children with myocarditis and dilated cardiomyopathy (DCM) listed for HT from January 01, 1993 to December 31, 2019 in the Pediatric Heart Transplant Society database. Clinical characteristics, waitlist and post-HT outcomes (graft loss, rejection, cardiac allograft vasculopathy, infection and malignancy) for children listed from early (1993-2008) and current era (2009-2019) with myocarditis were evaluated and compared to those with DCM.

Outcomes of infants with pulmonary atresia with intact ventricular septum listed for heart transplantation: A multi-institutional study.

Background: Management of infants with pulmonary atresia/intact ventricular septum (PA/IVS) is variable. Because of higher mortality in more severe forms, heart transplant (HT) is an acceptable approach, but waitlist and post-transplant outcomes are unclear. This study compared outcomes of infants with PA/IVS vs.

Biventricular Assist Device Support for Intractable Arrhythmias From Histiocytoid Cardiomyopathy.

Histiocytoid cardiomyopathy (HICMP) is a rare mitochondrial cardiomyopathy associated with recurrent life-threatening arrhythmias and variable degrees of systolic dysfunction. Successful heart transplantation for HICMP has been described, but there has been no published experience with biventricular assist device (BiVAD) support for intractable arrhythmias in HICMP. We report a 13 month old girl with left ventricular noncompaction and preserved systolic function who presented in cardiogenic shock secondary to incessant ventricular arrhythmias.