Mechanical circulatory support in failing single-ventricle physiology.

The use of ventricular assist device (VAD) therapy for advanced heart failure in infants and children with single-ventricle (SV) congenital heart disease (CHD) has expanded rapidly. However, issues pertaining to patient size, developmental hemostasis, device availability, complicated anatomy, and complex modes of hemodynamic/circulatory failure associated with each stage of SV palliation make VAD support particularly challenging in this population. Over the last 20 years, there has been significant improvement in support outcomes for this complex population; however, survival and rates of adverse events still lag behind children with other diagnoses.

Improving pediatric heart failure outcomes through collaboration and innovation: The current status of the Advanced Cardiac Therapies Improving Outcomes Network (ACTION).

The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) was founded in 2017 as a learning health care system to bring together all stakeholders with the aim of improving outcomes for pediatric and congenital heart disease patients with heart failure. From an initial focus on the ventricular assist device (VAD) population, work in the network has expanded to encompass the scope of heart failure in these patients. Through the collection and use of real-world data, the field has gained greater understanding of best practices in care of these patients, as well as improved access to devices with pediatric labeling of devices previously approved only for adults.

Impact of Anticoagulation Therapy After Bioprosthetic and Homograft Pulmonary Valve Replacement.

Background: The effect of anticoagulation on early postoperative outcomes after pulmonary valve replacement (PVR) with bioprosthetic valves and homografts is not yet defined. We hypothesized that short-term anticoagulation would be associated with improved valve durability.

Methods: Patients undergoing PVR or right ventricle-to-pulmonary artery conduits with a bioprosthetic or homograft valve >15 mm in diameter between January 2015 and April 2021 at Boston Children's Hospital were retrospectively compared by anticoagulation status.

Pediatric Extracorporeal Membrane Oxygenation in Restrictive and Hypertrophic Cardiomyopathy: A Single Center Experience.

Patients with restrictive cardiomyopathy (RCM) and hypertrophic cardiomyopathy (HCM) were previously considered poor candidates for mechanical circulatory support due to technical limitations related to restrictive ventricular physiology and small ventricular size, limiting the ability to provide adequate flows and decompress the heart. Literature examining use of extracorporeal membrane oxygenation (ECMO) in this population consists of a single case series reporting no survivors. We report our experience providing ECMO in children with RCM or HCM at a large pediatric quaternary cardiac center.

Improving Mechanical Circulatory Support Outcomes in Failing Bidirectional Glenn Physiology.

There remains high morbidity and mortality with mechanical circulatory support (MCS) in failing bidirectional Glenn (BDG) physiology. We performed a retrospective analysis of children with BDG physiology supported with MCS before and after 2018. Fourteen patients met inclusion criteria (median age 1.

The power of "it will be okay".

As caregivers for critically ill children and their families, there are moments when we find ourselves unsure of how best to offer support. The magnitude of the medical experience can cloud our communication with patients and their families. With years of counseling families through some of their most challenging and darkest hours, I aim to emphasize the profound impact of thoughtful and well-informed reassurance; how we can provide hope in hopeless situations.

Apixaban for Prevention of Thromboembolism in Pediatric Heart Disease.

Background: Children with heart disease frequently require anticoagulation for thromboprophylaxis. Current standard of care (SOC), vitamin K antagonists or low-molecular-weight heparin, has significant disadvantages.

Objectives: The authors sought to describe safety, pharmacokinetics (PK), pharmacodynamics, and efficacy of apixaban, an oral, direct factor Xa inhibitor, for prevention of thromboembolism in children with congenital or acquired heart disease.

Expanding use of the HeartMate 3 ventricular assist device in pediatric and adult patients within the Advanced Cardiac Therapies Improving Outcomes Network (ACTION).

Background: We report current outcomes in patients supported with the HeartMate 3 (HM3) ventricular assist device in a multicenter learning network.

Methods: The Advanced Cardiac Therapies Improving Outcomes Network database was queried for HM3 implants between 12/2017 and 5/2022. Clinical characteristics, postimplant course, and adverse events were collected.

Sixth Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report: The Society Of Thoracic Surgeons Pedimacs Annual 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, 2021, there were 1355 devices in 1109 patients (<19 years) from 42 North American Hospitals.

Results: Cardiomyopathy was the most common underlying cause (59%), followed by congenital heart disease (25%) and myocarditis (9%).

Pediatric heart transplant waiting times in the United States since the 2016 allocation policy change.

We describe waiting times for pediatric heart transplant (HT) candidates after the 2016 revision to the US allocation policy. The OPTN database was queried for pediatric HT candidates listed between 7/2016 and 4/2019. Of the 1789 included candidates, 65% underwent HT, 14% died/deteriorated, 8% were removed for improvement, and 13% were still waiting at the end of follow-up.

Fifth Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report.

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

Methods: From September 19, 2012, to December 31, 2020, 1229 devices in 1011 patients were reported to the registry from 47 North American Hospitals in patients aged younger than 19 years.

Results: Cardiomyopathy was the most common underlying etiology (58%), followed by congenital heart disease (CHD; 25%) and myocarditis (10%).

Acute Management of High-Risk and Intermediate-Risk Pulmonary Embolism in Children: A Review.

Severe forms of pulmonary embolism (PE) in children, althought rare, cause significant morbidity and mortality. We review the pathophysiologic features of severe (high-risk and intermediate-risk) PE and suggest novel pediatric-specific risk stratifications and an acute treatment algorithm to expedite emergent decision-making. We defined pediatric high-risk PE as causing cardiopulmonary arrest, sustained hypotension, or normotension with signs or symptoms of shock.

Coagulation profiles and viscoelastic testing in multisystem inflammatory syndrome in children.

Objective: To characterize viscoelastic testing profiles of children with multisystem inflammatory syndrome in children (MIS-C).

Methods: This single-center retrospective review included 30 patients diagnosed with MIS-C from March 1 to September 1, 2020. Thromboelastography (TEG) with platelet mapping was performed in 19 (63%) patients and compared to age- and sex-matched controls prior to cardiac surgery.

Difference in the prevalence of intracardiac thrombus on the first presentation of atrial fibrillation versus flutter in the pediatric and congenital heart disease population.

Introduction: Guidelines recommend trans-esophageal echocardiography (TEE) for patients with atrial fibrillation (AF) or atrial flutter (AFL) for >48 h, due to risk of intracardiac thrombus formation. With growing evidence that AFL in adults with structurally normal hearts has less thrombogenic potential compared to AF, and the need for TEE questioned, we compared prevalence of intracardiac thrombus detected by TEE in pediatric and congenital heart disease (CHD) patients presenting in AF and AFL.

Methods/results: Single-center, cross-sectional analysis for unique first-time presentations of patients for either AF, AFL, or intra-atrial reentrant tachycardia (IART) between 2000 and 2019.

Cardiac manifestations in SARS-CoV-2-associated multisystem inflammatory syndrome in children: a comprehensive review and proposed clinical approach.

Initial reports on COVID-19 described children as largely spared from severe manifestations, with only 2-6% of children requiring intensive care treatment. However, since mid-April 2020, clusters of pediatric cases of severe systemic hyperinflammation and shock epidemiologically linked with COVID-19 have been reported. This condition was named as SARS-Cov-2-associated multisystem inflammatory syndrome in children and showed similarities to Kawasaki disease.

Utilization and Outcomes of Children Treated with Direct Thrombin Inhibitors on Paracorporeal Ventricular Assist Device Support.

Thrombotic and bleeding complications have historically been major causes of morbidity and mortality in pediatric ventricular assist device (VAD) support. Standard anticoagulation with unfractionated heparin is fraught with problems related to its heterogeneous biochemical composition and unpredictable pharmacokinetics. We sought to describe the utilization and outcomes in children with paracorporeal VAD support who are treated with direct thrombin inhibitors (DTIs) antithrombosis therapy.

The Creation of a Pediatric Health Care Learning Network: The ACTION Quality Improvement Collaborative.

Improving the outcomes of pediatric patients with congenital heart disease with end-stage heart failure depends on the collaboration of all stakeholders; this includes providers, patients and families, and industry representatives. Because of the rarity of this condition and the heterogeneity of heart failure etiologies that occur at pediatric centers, learnings must be shared between institutions and all disciplines to move the field forward. To foster collaboration, excel discovery, and bring data to the bedside, a new, collaborative quality improvement science network-ACTION (Advanced Cardiac Therapies Improving Outcomes Network)-was developed to meet the needs of the field.

Apixaban for treatment of intracardiac thrombosis in children with congenital heart disease.

Apixaban is well studied in adults; paediatric data are extremely limited. We describe 3 children (age 2-6 years, weight 13-17 kg) with congenital heart disease who developed intracardiac thrombosis, and in whom typical treatments had failed (in 1) or were considered impractical (in 2). All were treated with apixaban 2.

Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: A multicenter registry analysis.

Background: The HeartMate 3 ventricular assist device (VAD) is a newer centrifugal continuous-flow VAD used for bridge-to-transplant and destination therapy in adults. However, there is limited experience regarding its use in children and adults with complex congenital heart disease (CHD).

Methods: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a multicenter learning network comprised of pediatric hospitals implanting VADs in children and adults with complex CHD.

Collaboration and new data in ACTION: a learning health care system to improve pediatric heart failure and ventricular assist device outcomes.

Outcomes in pediatric heart failure and ventricular assist devices (VADs) remain suboptimal. Given the complexity and limited numbers, centers cannot be expected to acquire mastery and generalizable knowledge independently. Recognizing this problem, the community has formed Advanced Cardiac Therapies Improving Outcomes Network (ACTION), a collaborative learning health care system committed to unrelenting collaboration and continuous learning to improve critical outcomes in pediatric heart failure.

Outcomes of children supported with an intracorporeal continuous-flow left ventricular assist system.

Background: Since 2012, there has been growing use of the HeartWare (Medtronic, Mounds View, MN) intracorporeal continuous flow (CF) ventricular assist device (VAD) in children, despite it not being labeled for use in pediatric patients. We sought to describe the use and outcomes of children with HeartWare VADs.

Methods: We identified all patients aged < 19 years and young adults aged 19 to 30 years supported with HeartWare who were entered into the pediatric portion (Pedimacs) of the Interagency Registry for Mechanically Assisted Circulatory Support (Intermacs) and the Intermacs registries, respectively, between September 2012 and June 2017.

Antithrombotic therapies in children on durable Ventricular Assist Devices: A literature review.

Introduction: Ventricular Assist Devices (VADs) are increasingly utilised in children with end-stage heart failure, and experience high bleeding and clotting rates. In particular, pediatric VAD patients are more challenging than adults to anticoagulate due to developmental hemostasis, lack of suitable drug preparations, and difficult anticoagulation monitoring often due to poor vascular access; in addition to difficulties of VAD design in smaller children. This review aims to summarize the current evidence related to antithrombotic therapy in pediatric VAD patients.