Heart Transplantation for Pediatric and Congenital Cardiac Disease: A Comparison of Two Eras over 23 Years and 188 Transplants at a Single Institution.

Background: To assess changes in patterns of practice and outcomes over time, we reviewed all patients who underwent heart transplantation (HTx) at our institution and compared two consecutive eras with significantly different immunosuppressive protocols (cohort 1 [80 HTx, June 1995-June 2006]; cohort 2 [108 HTx, July 2006-September 2018]).

Methods: Retrospective study of 180 patients undergoing 188 HTx (June 1995-September 2018; 176 first time HTx, 10 second HTx, and 2 third HTx). In 2006, we commenced pre-HTx desensitization for highly sensitized patients and started using tacrolimus as our primary postoperative immunosuppressive agent.

Development and impact of arrhythmias after the Norwood procedure: A report from the Pediatric Heart Network.

Objectives: The study objective was to determine the predictors of new-onset arrhythmia among infants with single-ventricle anomalies during the post-Norwood hospitalization and the association of those arrhythmias with postoperative outcomes (ventilator time and length of stay) and interstage mortality.

Methods: After excluding patients with preoperative arrhythmias, we used data from the Pediatric Heart Network Single Ventricle Reconstruction Trial to identify risk factors for tachyarrhythmias (atrial fibrillation, atrial flutter, supraventricular tachycardia, junctional ectopic tachycardia, and ventricular tachycardia) and atrioventricular block (second or third degree) among 544 eligible patients. We then determined the association of arrhythmia with outcomes during the post-Norwood hospitalization and interstage period, adjusting for identified risk factors and previously published factors.

Hospital Charges for Pediatric Heart Failure-Related Hospitalizations from 2000 to 2009.

Scarce data exist regarding costs of pediatric heart failure-related hospitalizations (HFRH) or how costs have changed over time. Pediatric HFRH costs, due to advances in management, will have increased significantly over time. A retrospective analysis of Healthcare Cost and Utilization Project Kids' Inpatient Database was performed on all pediatric HFRH.

Development of paediatric electrophysiology standards for Florida Children's Medical Services.

The Florida Children's Medical Services (CMS) has a long-standing history of ensuring that providers of multiple paediatric subspecialties abide by the highest standards. The cardiac sub-committee has written quality standard documents that participating programmes must meet or exceed. These standards oversee paediatric cardiology services including surgery, catheterisations, and outpatient services.

Arrhythmias in paediatric valvar disease.

Valvar heart disease can be complicated by hemodynamic derangements, depending on the degree of the abnormality. Stenosis causes pressure overload of the chamber draining through the valve and regurgitation results in volume overload. Many lesions have a component of both, resulting in both pressure and volume overload.

Efficacy of digoxin in comparison with propranolol for treatment of infant supraventricular tachycardia: analysis of a large, national database.

Introduction: Digoxin or propranolol are used as first-line enteral agents for treatment of infant supraventricular tachycardia. We used a large national database to determine whether enteral digoxin or propranolol was more efficacious as first-line infant supraventricular tachycardia therapy.

Materials And Methods: The Pediatric Health Information System database was queried over 10 years for infants with supraventricular tachycardia initiated on enteral digoxin or propranolol monotherapy.

Management of arrhythmias in patients with a tetralogy of Fallot.

Although arrhythmias are commonly found in patients with all different types of congenital heart disease, perhaps no patient is at higher risk of late development of arrhythmias that may result in sudden death than in the patient following tetralogy of Fallot repair. Despite major improvement in the surgical repair of this disease, a significant percentage of these patients continue to remain at risk for the late development of arrhythmias, and a small percentage will develop life-threatening arrhythmias and sudden death. Which patients remain at highest risk is still not clearly delineated.

Mortality and sudden death in pediatric left ventricular noncompaction in a tertiary referral center.

Background: Left ventricular noncompaction is a cardiomyopathy characterized by excessive trabeculation of the left ventricle, progressive myocardial dysfunction, and early mortality. Left ventricular noncompaction has a heterogeneous clinical presentation that includes arrhythmia and sudden cardiac death.

Methods And Results: We retrospectively reviewed all children diagnosed with left ventricular noncompaction at Texas Children's Hospital from January 1990 to January 2009.

Arrhythmia management in patients with a common arterial trunk and d-transposition of the great arteries.

Arrhythmias in patients with congenital heart disease present a challenge to the care of these patients and can result in significant morbidity and mortality. Transposition of the great arteries and common arterial trunk are no exceptions. It is important to identify risk factors for arrhythmia development in the peri-operative period.

Prevalence, morbidity, and mortality of heart failure-related hospitalizations in children in the United States: a population-based study.

Background: Few data exist on prevalence, morbidity, and mortality of pediatric heart failure hospitalizations. We tested the hypotheses that pediatric heart failure-related hospitalizations increased over time but that mortality decreased. Factors associated with mortality and length of stay were also assessed.

Magnetic versus manual catheter navigation for ablation of free wall accessory pathways in children.

Background: Transcatheter ablation of accessory pathway (AP)-mediated tachycardia is routinely performed in children. Little data exist regarding the use of magnetic navigation (MN) and its potential benefits for ablation of AP-mediated tachycardia in this population.

Methods And Results: We performed a retrospective review of prospectively gathered data in children undergoing radiofrequency ablation at our institution since the installation of MN (Stereotaxis Inc, St.

Hospital charges for pediatric heart transplant hospitalizations in the United States from 1997 to 2006.

Background: Heart transplantation remains a resource-intensive therapy for children. However, data regarding change in costs over time are scarce. We tested the hypothesis that hospital charges for pediatric heart transplant hospitalizations would increase from 1997 to 2006 and assessed factors associated with hospital charges.

Restrictive physiology is associated with poor outcomes in children with hypertrophic cardiomyopathy.

Pediatric patients with hypertrophic cardiomyopathy (HCM) and restrictive physiology (RP) with poor outcomes have been identified, but data on their course are limited. Our goal was to delineate the clinical features and course of children with HCM and RP. An institutional review of 119 patients identified between 1985 and 2010 with the diagnosis of HCM was performed.

Effectiveness of mechanical circulatory support in children with acute fulminant and persistent myocarditis.

Background: Acute fulminant myocarditis is a life-threatening disease in children. A limited number of reports suggest that mechanical circulatory support (MCS) may be used to successfully bridge children with acute fulminant myocarditis to recovery or transplantation. We evaluated the effectiveness of MCS in children with myocarditis and identified risk factors associated with adverse outcomes.

Ultrasound dilution: an accurate means of determining cardiac output in children.

Background: Cardiac output is a useful measure of myocardial performance. Cardiac output monitoring is frequently performed in critically ill adults to guide physicians' treatment strategies. However, standard methods of determining cardiac output in children are not without risk and can be problematic secondary to their invasive nature and other technical problems.

Impact of the American Heart Association scientific statement on screening electrocardiograms and stimulant medications.

Objective: To evaluate the impact of the American Heart Association (AHA) scientific statement regarding electrocardiograms (ECGs) and stimulant medications on the practice of community pediatricians.

Design: Retrospective evaluation and survey analysis.

Setting: Academic tertiary care center.

Electrocardiographic abnormalities and arrhythmias are strongly associated with the development of cardiomyopathy in muscular dystrophy.

Background: Dilated cardiomyopathy (DCM) is a well-recognized sequela of muscular dystrophy (MD). Early identification of cardiac involvement with timely therapy can favorably impact outcome. We hypothesize that electrocardiography (ECG) can be a useful adjunct in the identification of DCM in MD.

Risk factors and mode of death in isolated hypertrophic cardiomyopathy in children.

Objectives: This study was designed to review outcomes of pediatric isolated hypertrophic cardiomyopathy (HCM) managed uniformly at a single institution and assess whether reported adult risk factors for sudden death are predictive in pediatric HCM.

Background: Cardiac death in HCM occurs suddenly (SCD) or may be nonsudden (non-SCD). Little data exists on non-SCD in children.

Assessment of the Cylex ImmuKnow cell function assay in pediatric heart transplant patients.

Background: The Cylex ImmuKnow (Cylex, Columbia, MD) cell function assay (CICFA) is a commercially available test of immune response that purportedly identifies solid organ transplant patients at risk for either acute rejection (AR) or infection. Data on the utility of this test in pediatric heart transplant patients are very limited. This study tested the hypothesis that CICFA is a clinically useful test in this transplant population.

B-type natriuretic peptide is a sensitive screening test for acute rejection in pediatric heart transplant patients.

Background: The utility of B-type natriuretic peptide (BNP) for detecting acute rejection (AR) is unclear. The purpose of our study was to evaluate BNP as a screening test for AR in pediatric heart transplant patients.

Methods: All endomyocardial biopsies (EMBs) with concurrent BNP levels from February 2004 through March 2007 at the study institution were reviewed and the association between BNP levels and acute rejection was assessed.

Effect of body mass index on outcome in pediatric heart transplant patients.

Background: Obesity and cachexia are risk factors for adverse outcomes in adult transplant patients. However, little is known about the effects of body mass index (BMI) on outcomes in pediatric heart transplant patients.

Methods: Patients > 2 years of age undergoing heart transplantation from 1985 to 2004 at our institution were included in this study.