Case Ascertainment in Pediatric Heart Failure Using International Classification of Disease Clinical Modification (ICD-CM) Codes.

Most epidemiological studies in pediatric heart failure (HF) use administrative database sources, defining patient cohorts by presence of a single HF ICD code. However, the ability of ICD codes to identify true HF patients is unknown in pediatrics. Here we describe the accuracy of HF ICD-10-CM code search algorithms, in identifying pediatric patients with HF from electronic data sources.

Case Ascertainment in Pediatric Heart Failure Using International Classification of Disease Clinical Modification (ICD-CM) Codes.

Background: Most epidemiological studies in pediatric heart failure (HF) use administrative database sources, defining patient cohorts by presence of a single HF ICD code. However, the ability of ICD codes to identify true HF patients is unknown in pediatrics. Here we describe the accuracy of HF ICD-10-CM code search algorithms, in identifying pediatric patients with HF from electronic data sources.

Patient and parent-reported outcomes in paediatric ventricular assist device support: a multi-center ACTION learning network feasibility and pilot experience.

Background: Patient- and proxy-reported outcomes (PROs) are an important indicator of healthcare quality and can be used to inform treatment. Despite the widescale use of PROs in adult cardiology, they are underutilised in paediatric cardiac care. This study describes a six-center feasibility and pilot experience implementing PROs in the paediatric and young adult ventricular assist device population.

Initial multicenter experience with ventricular assist devices in children and young adults with muscular dystrophy: An ACTION registry analysis.

Purpose: Cardiac disease results in significant morbidity and mortality in patients with muscular dystrophy (MD). Single centers have reported their ventricular assist device (VAD) experience in specific MDs and in limited numbers. This study sought to describe the outcomes associated with VAD therapy in an unselected population across multiple centers.

Attitudes & practices surrounding pregnancy post heart transplant among pediatric providers.

Background: Many pediatric heart transplant (HT) recipients reach adulthood and may be interested in family planning; there is little data regarding safety of pregnancy post HT and clinicians' opinions differ. Pediatric HT clinicians are instrumental in early counseling. Thus, a better understanding of pediatric HT clinicians' practices regarding family planning and how well aligned these practices are with adult transplant centers is essential.

Hemodynamic Predictors of Renal Function After Pediatric Left Ventricular Assist Device Implantation.

Although renal function often improves after pediatric left ventricular assist device (LVAD) implantation, recovery is inconsistent. We aimed to identify hemodynamic parameters associated with improved renal function after pediatric LVAD placement. A single-center retrospective cohort study was conducted in patients less than 21 years who underwent LVAD placement between June 2004 and December 2015.

Berlin Heart EXCOR and ACTION post-approval surveillance study report.

Background: The Berlin Heart EXCOR Pediatric (EXCOR) ventricular assist device (VAD) was introduced in North America nearly 2 decades ago. The EXCOR was approved under Humanitarian Device Exemption status in 2011 and received post-market approval (PMA) in 2017 from Food and Drug Administration. Since the initial approval, the field of pediatric mechanical circulatory support has changed, specifically with regard to available devices, anticoagulation strategies, and the types of patients supported.

Implications of Left Ventricular Dysfunction at Presentation for Infants with Coarctation of the Aorta.

Infants with aortic coarctation may present with left ventricular (LV) dysfunction which may complicate the postoperative course and lead to increased healthcare costs. We aimed to define the prevalence of moderate to severe left ventricular (LV) systolic dysfunction, evaluate time to recovery, and compare health care costs. Single-center retrospective cohort study at a tertiary care hospital was conducted.

Time in Therapeutic Range for Bivalirudin Among Pediatric Ventricular Assist Device Recipients.

Given the adverse event rates involving bleeding and thrombosis among children on ventricular assist devices (VADs), anticoagulant management has become a focal point for quality improvement and innovation. There may be advantages to using direct thrombin inhibitors, such as bivalirudin, though this has not been fully explored. As the percent time in therapeutic range (%TTR) for anticoagulants is classically associated with improved clinical outcomes, we evaluated the %TTR for bivalirudin among pediatric VAD recipients.

Cardiac transplantation in children with Noonan syndrome.

NS and related RAS/MAPK pathway (RASopathy) disorders are the leading genetic cause of HCM presenting in infancy. HCM is a major cause of morbidity and mortality in children with Noonan spectrum disorders, especially in the first year of life. Previously, there have been only isolated reports of heart transplantation as a treatment for heart failure in NS.

Marked Practice Variation in Antithrombotic Care with the Berlin Heart EXCOR Pediatric Ventricular Assist Device.

Management of antithrombotic therapy (ATT) for pediatric ventricular assist devices is challenging, and the Berlin EXCOR remains the only Food and Drug Administration (FDA)-approved option. Among those on the EXCOR, 28% have neurologic complications and major bleeding occurs in 50%. The Edmonton Protocol was developed to guide ATT, but the adverse event rate remains high, leading most centers to make modifications.

Haemodynamic profiles of children with end-stage heart failure.

Aims: To evaluate associations between haemodynamic profiles and symptoms, end-organ function and outcome in children listed for heart transplantation.

Methods And Results: Children <18 years listed for heart transplant between 1993 and 2013 with cardiac catheterization data [pulmonary capillary wedge pressure (PCWP), right atrial pressure (RAP), and cardiac index (CI)] in the Pediatric Heart Transplant Study database were included. Outcomes were New York Heart Association (NYHA)/Ross classification, renal and hepatic dysfunction, and death or clinical deterioration while on waitlist.

Recovery From Acute Kidney Injury and CKD Following Heart Transplantation in Children, Adolescents, and Young Adults: A Retrospective Cohort Study.

Background: Acute kidney injury (AKI) is common in children following surgery for congenital heart disease and has been associated with poor long-term kidney outcomes. Children undergoing heart transplantation may be at increased risk for the development of both AKI and chronic kidney disease (CKD). This study examines AKI rates in children, adolescents, and young adults after heart transplantation and analyzes the relationship between AKI and CKD in this population.

Electrocardiographic repolarization abnormalities and increased risk of life-threatening arrhythmias in children with dilated cardiomyopathy.

Background: Life-threatening arrhythmia events (LTEs) occur in ~5% of children with dilated cardiomyopathy (DCM). While prolonged QRS duration has been shown to be associated with LTEs, electrocardiographic (ECG) repolarization findings have not been examined.

Objective: We sought to determine the associations between ECG repolarization abnormalities and LTEs in children with DCM.

Outpatient Outcomes of Pediatric Patients with Left Ventricular Assist Devices.

Outpatient experience of children supported with continuous-flow ventricular assist devices (CF-VAD) is limited. We reviewed our experience with children discharged with CF-VAD support. All pediatric patients <18 years old with CF-VADs implanted at our institution were included.

Impact of ventricular assist device placement on longitudinal renal function in children with end-stage heart failure.

Background: Although ventricular assist devices (VADs) restore hemodynamics in those with heart failure, reversibility of end-organ dysfunction with VAD support is not well characterized. Renal function often improves in adults after VAD placement, but this has not been comprehensively explored in children.

Methods: Sixty-three children on VAD support were studied.

Group visits in the pediatric heart transplant outpatient clinic.

The "GVM" has emerged as an alternative to traditional individualized appointments in the ambulatory care setting. We hypothesized that group visits could successfully be utilized in a PHtx clinic. Seven patients, ages 1-18 yr old, and their families participated in a total of 11 group visits in lieu of individualized appointments.

A novel pediatric treatment intensity score: development and feasibility in heart failure patients with ventricular assist devices.

Background: The evolution of pharmacologic therapies and mechanical support including ventricular assist devices (VADs) has broadened the scope of care available to children with advanced heart failure. At the present time, there are only limited means of quantifying disease severity or the concomitant morbidity for this population. This study describes the development of a novel pediatric treatment intensity score (TIS), designed to quantify the burden of illness and clinical trajectory in children on VAD support.

Reliability of echocardiographic measurements of left ventricular systolic function in potential pediatric heart transplant donors.

Background: Echocardiogram reports, but not images, are usually available for the evaluation of potential donor hearts. To assess the reliability of local reports of potential pediatric heart donors, we compared echocardiographic measurements of left ventricular (LV) systolic function between local hospitals and a central echocardiography laboratory.

Methods: We identified all potential donors aged <18 years managed by the California Transplant Donor Network from 2009 to 2013.

QTc Prolongation in Acute Pediatric Migraine.

Objectives: Migraine headache is common in pediatrics and is frequently assessed in emergency departments. Altered cardiac conduction, including prolongation of the QTc interval on electrocardiogram, has been observed in adults during migraine headache and resolves interictally. Prolonged QTc is associated with life-threatening arrhythmia, and many acute and prophylactic therapies for migraine can further prolong the QTc interval.

HLA desensitization with bortezomib in a highly sensitized pediatric patient.

The proteasome inhibitor bortezomib has been used with variable success in the treatment of AMR following heart transplant. There is limited experience with this agent as a pretransplant desensitizing therapy. We report a case of successful HLA desensitization with a bortezomib-based protocol prior to successful heart transplantation.

Optimizing care of adults with congenital heart disease in a pediatric cardiovascular ICU using electronic clinical decision support*.

Objectives: The optimal location for postoperative cardiac care of adults with congenital heart disease is controversial. Some congenital heart surgeons operate on these adults in children's hospitals with postoperative care provided by pediatric critical care teams who may be unfamiliar with adult national performance measures. This study tested the hypothesis that Clinical Decision Support tools integrated into the clinical workflow would facilitate improved compliance with The Joint Commission Surgical Care Improvement Project performance measures in adults recovering from cardiac surgery in a children's hospital.

Supine cycling in pediatric exercise testing: disparity in performance measures.

Supine cycling may be an important alternative modality for exercise testing. Subtle differences in supine and upright exercise physiology have been suggested but not fully explored in the pediatric population. The aim of this study was to compare peak and submaximal metabolic data in the upright and supine exercise positions.