Acute kidney injury in hypoplastic left heart syndrome patients following the comprehensive stage two palliation.

Background: An alternative surgical approach for hypoplastic left heart syndrome is the Hybrid pathway, which delays the risk of acute kidney injury outside of the newborn period. We sought to determine the incidence, and associated morbidity, of acute kidney injury after the comprehensive stage 2 and the cumulative incidence after the first two operations in the Hybrid pathway.

Design: A single centre, retrospective study was conducted of hypoplastic left heart patients completing the second-stage palliation in the Hybrid pathway from 2009 to 2018.

Music Therapy Intervention to Reduce Caregiver Distress at End of Life: A Feasibility Study.

Background: Music therapy (MT) can relieve distressing end-of-life symptoms, but little is known regarding its effect on caregivers who are at risk for emotional distress as their loved ones approach death.

Measures: Quality of life (Linear Analogue Self-Assessment), depressive and anxiety symptoms (Patient Health Questionnaire for Depression and Anxiety), and stress (Role Overload Measure) pre-MT, post-MT and at 6-month follow-up, as well as a satisfaction survey post-MT.

Intervention: Single MT session for 20-45 minutes OUTCOMES: 15/20 completed MT intervention, 14 also completed pre-MT and post-MT assessments, and 9 completed assessments at all 3 timepoints.

Impact of Maternal-Fetal Environment on Outcomes Following the Hybrid Procedure in the Single Ventricle Population.

Treatment of infants with hypoplastic left heart syndrome (HLHS) remains challenging, and those affected remain with significant risks for mortality and morbidity throughout their lifetimes. The maternal-fetal environment (MFE) has been shown to affect outcomes for infants with HLHS after the Norwood procedure. The hybrid procedure, comprised of both catheterization and surgical components, is a less invasive option for initial intervention compared to the Norwood procedure.

Clinical approach to mechanical circulatory support in the transplant patient from the Pediatric Heart Transplant Society.

The use of mechanical circulatory support (MCS) for pediatric patients who have undergone heart transplant has grown rapidly in the past decade. This includes support in the immediate post-transplant period and "rescue" therapy for patient later in their transplant course. Extracorporeal membrane oxygenation (ECMO) remains a standard modality of support for intraoperative concerns and for acute decompensation in the immediate post-transplant period.

Worsening racial disparity in waitlist mortality for pediatric heart transplant candidates since the 2016 Pediatric Heart Allocation Policy revision.

Background: The US Pediatric Heart Allocation Policy (PHAP) was revised in March 2016, with the goal of reducing waitlist mortality. We evaluated the hypothesis that these changes, which increased status exceptions, have worsened racial disparities in waitlist outcomes.

Methods: Children in the Pediatric Heart Transplant Study database listed for first heart transplant from January 2012 - June 2020 were included and stratified by listing before (Era 1) or after (Era 2) the PHAP revision.

Data-driven computational models of ventricular-arterial hemodynamics in pediatric pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a complex disease involving increased resistance in the pulmonary arteries and subsequent right ventricular (RV) remodeling. Ventricular-arterial interactions are fundamental to PAH pathophysiology but are rarely captured in computational models. It is important to identify metrics that capture and quantify these interactions to inform our understanding of this disease as well as potentially facilitate patient stratification.

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.

Incidence and impact of acute kidney injury in patients with hypoplastic left heart syndrome following the hybrid stage 1 palliation.

Objective: Acute kidney injury leads to worse outcomes following paediatric cardiac surgery. There is a lack of literature focusing on acute kidney injury after the Hybrid stage 1 palliation for single ventricle physiology. Patients undergoing the Hybrid Stage 1, as a primary option, may have a lower incidence of kidney injury than previously reported.

Tricuspid Valve and Right Ventricular Function Throughout the Hybrid Palliation Strategy for Hypoplastic Left Heart Syndrome and Variants.

Background: Tricuspid valve (TV) and right ventricular (RV) function are major determinants of morbidity and mortality in patients with hypoplastic left heart syndrome (HLHS). We sought to retrospectively evaluate these parameters throughout the hybrid palliation strategy.

Methods: From 2002 to 2018, 203 patients with HLHS and variants presented for hybrid stage I (HS1).

Guidelines for Diuretic Utilization Reduce High Charge Medications.

Unlabelled: Decreasing practice variation and following evidence-based clinical guidelines improve patient outcomes and often reduce cost. Essentially all postsurgical cardiac patients require diuretics. The approach to diuresis in the pediatric cardiothoracic intensive care unit (CTICU) is not standardized.

Pediatric Fontan Associated Liver Disease: Non-invasive Evaluation with Serologic Markers and Acoustic Radiation Force Impulse (ARFI) Elastography.

Hepatic fibrosis is a significant complication in adult Fontan patients suggesting development as a function of time since the surgery. Children with Fontan circulation are not routinely assessed for development of liver disease. We aimed to evaluate the effectiveness of serologic biomarkers and acoustic radiation force impulse (ARFI) elastography to detect liver disease in pediatric Fontan patients.

Interstage outcomes in single ventricle patients undergoing hybrid stage 1 palliation.

Objective: Interstage readmissions are common in infants with single ventricle congenital heart disease undergoing staged surgical palliation. We retrospectively examined readmissions during the interstage period.

Design: Retrospective analysis.

Pediatric marginal donor hearts: Trends in US national use, 2005-2014.

Pediatric patients awaiting heart transplant face high mortality rates due to donor organ shortages, including non-use of marginal donor hearts. We examined national trends in pediatric marginal donor heart use over time. UNOS data were queried for heart donors <18 years from 2005 to 2014.

Building a comprehensive team for the longitudinal care of single ventricle heart defects: Building blocks and initial results.

Introduction: With increasing survival of children with HLHS and other single ventricle lesions, the complexity of medical care for these patients is substantial. Establishing and adhering to best practice models may improve outcome, but requires careful coordination and monitoring.

Methods: In 2013 our Heart Center began a process to build a comprehensive Single Ventricle Team designed to target these difficult issues.

Multidisciplinary Review of Code Events in a Heart Center.

Objective: To identify a cause for clinical deterioration, examine resuscitation efforts, and identify and correct system issues (thus improving outcomes) via a multidisciplinary code-review process soon after cardiopulmonary arrest.

Methods: Retrospective analysis of code events in a tertiary pediatric heart center from September 2010 to December 2013 and review of surgical-cardiac data from January 2010 to December 2013.

Results: A multidisciplinary team reviewed 47 code events, 16 of which (34%) were deemed potentially preventable.

Digoxin Use Is Associated With Reduced Interstage Mortality in Patients With No History of Arrhythmia After Stage I Palliation for Single Ventricle Heart Disease.

Background: Interstage mortality (IM) remains significant after stage 1 palliation (S1P) for single-ventricle heart disease (SVD), with many deaths sudden and unexpected. We sought to determine whether digoxin use post-S1P is associated with reduced IM, utilizing the multicenter database of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC).

Methods And Results: From June 2008 to July 2013, 816 infants discharged after S1P from 50 surgical sites completed the interstage to stage II palliation, transplant, or IM.

Management strategies after cardiac surgery in an infant with human rhinovirus.

This report presents management strategies after cardiopulmonary bypass surgery for an infant with community-acquired rhinovirus bronchiolitis. The case report emphasizes human rhinovirus as a lower respiratory pathogen, the difficulty treating the complications of human rhinovirus for an infant with cyanotic congenital heart disease after cardiac surgery, and the importance of preoperative evaluation for this respiratory infection in high-risk infants before cardiac surgery.

Acute kidney injury after cardiac surgery in infants and children: evaluation of the role of angiotensin-converting enzyme inhibitors.

Children with congenital heart disease who undergo cardiac surgery are vulnerable to acute kidney injury (AKI). This study sought to evaluate the role of angiotensin-converting enzyme (ACE) inhibitors and other nephrotoxic medications in the risk for the development of AKI in neonates and children undergoing cardiac surgery. A retrospective review of all patients younger than 2 years admitted to the cardiac intensive care unit after cardiac surgery from March 2007 to September 2008 was conducted.

Outcome of acute graft rejection associated with hemodynamic compromise in pediatric heart transplant recipients.

We sought to analyze the outcome of hemodynamically significant acute graft rejection in pediatric heart transplant recipients from a single-center experience. Acute graft rejection remains a major cause of morbidity and mortality for patients who undergo orthotopic heart transplantation and has been associated with the severity of the rejection episode. A retrospective review of all children experiencing a hemodynamically significant rejection episode after orthotopic heart transplantation was performed.

Outcome of extracorporeal membrane oxygenation for early primary graft failure after pediatric heart transplantation.

Objectives: We sought to analyze the indications and outcome of extracorporeal membrane oxygenation (ECMO) for early primary graft failure and determine its impact on long-term graft function and rejection risk.

Background: Early post-operative graft failure requiring ECMO can complicate heart transplantation.

Methods: A retrospective review of all children requiring ECMO in the early period after transplantation from 1990 to 2007 was undertaken.

A curious isolated cystic lesion of the membranous atrioventricular septum.

We present the case of an isolated cystic lesion of the atrioventricular component of the membranous septum of unclear aetiology, but responsible for cardiomegaly and benign disturbances of cardiac rhythm. As far as we are aware, this type of lesion has not previously been documented.