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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. The primary outcome was mortality. Survival was modeled by the Kaplan-Meier method. Univariable and multivariable Cox proportional hazard models were created to identify prognostic factors for survival.Results: Our 188 HTx included 18 neonates, 85 infants, 83 children, and 2 adults (>18 years). Median age was 260.0 days (range: 5 days-23.8 years). Median weight was 7.5 kg (range: 2.2-113 kg). Patients in cohort 1 were less likely to have been immunosensitized preoperatively (12.5% vs 28.7%, P= .017). Nevertheless, Kaplan-Meier analysis suggested superior survival in cohort 2 (P= .0045). Patients in cohort 2 were more likely to be alive one year, five years, and ten years after HTx. Multivariable analysis identified the earlier era (hazard ratio [HR] [95% confidence interval] for recent era = 0.32 [0.14-0.73]), transplantation after prior Norwood operation (HR = 4.44 [1.46-13.46]), and number of prior cardiac operations (HR = 1.33 [1.03-1.71]) as risk factors for mortality.Conclusions: Our analysis of 23 years of pediatric and congenital HTx reveals superior survival in the most recent 12-year era, despite the higher proportion of patients with elevated panel reactive antibody in the most recent era. This improvement was temporally associated with changes in our immunosuppressive strategy.

Background: This article reviews all patients who underwent heart transplantation (HTx) within a single institution (172 patients underwent 179 HTx [167 first-time HTxs, 10 second HTxs, 2 third HTxs]) to describe diagnostic characteristics, management protocols, and risk factors for mortality.Methods: Descriptive analysis was performed for the entire cohort using mean, standard deviation, median, interquartile range, and overall range, as appropriate. Univariable and multivariable Cox proportional hazards models were performed to identify prognostic factors for outcomes over time. The primary outcome of interest was mortality, which was modeled by Kaplan-Meier analysis.Results: Median age at HTx was 263 days (range, 5 days to 24 years; mean = 4.63 ± 5.95 years; 18 neonates, 79 infants). Median weight at HTx was 7.5 kg (range, 2.2-113 kg; mean = 19.36 ± 23.54). Diagnostic categories were cardiomyopathy (n = 62), primary transplantation for hypoplastic left heart syndrome (HLHS) or HLHS-related malformation (n = 33), transplantation after cardiac surgery for HLHS or HLHS-related malformation (n = 17), non-HLHS congenital heart disease (n = 55), and retransplant (n = 12). Operative mortality was 10.1% (18 patients). Cumulative total follow-up is 1,355 years. Late mortality was 18.4% (33 patients). Overall Kaplan-Meier five-year survival was 76.2%. One hundred twenty-one patients are alive with a mean follow-up of 7.61 ± 6.46 years. No survival differences were seen among the five diagnostic subgroups (P= .064) or between immunosensitized patients (n = 31) and nonimmunosensitized patients (n = 141; P= .422).Conclusions: Excellent results are expected for children undergoing HTx with comparable results among diagnostic groups. Pretransplant mechanical circulatory support and posttransplant mechanical circulatory support are risk factors for decreased survival. Survival after transplantation for HLHS or HLHS-related malformation is better with primary HTx in comparison to HTx after prior cardiac surgery.

AbstractIntroduction: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 effciacious 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. Patients were excluded for Wolff–Parkinson–White, intravenous antiarrhythmics (other than adenosine), or death. Success was considered as discharge on the initiated monotherapy. Risk factors for successful monotherapy and risk factors for readmission for supraventricular tachycardia for patients discharged on monotherapy were determined. Results:A total of 374 patients (59.6% male) met the study criteria. Median length of stay was 7 days (interquartile range of 3–16 days). Patients had CHD (n=199, 53.2%) and underwent cardiac surgery (n=123, 32.9%), ICU admission (n=238, 63.6%), mechanical ventilation (n=146, 39.0%), and extracorporeal membrane oxygenation (n=3, 0.8%). Pharmacotherapy initiation was at median 37 days of life (interquartile range of 12–127 days) and 47.3% were initiated on digoxin. Success was similar between digoxin (73.1%) and propranolol (73.5%). Initial therapy with digoxin was not associated with success (odds ratio 1.01, 95% CI 0.64–1.61, p=0.93). Multivariable analysis demonstrated hospital length of stay (odds ratio 0.98, 95% CI 0.98–1.00) and involvement of a paediatric cardiologist (odds ratio 0.46, 95% CI 0.29–0.75) associated with monotherapy failure, and male gender (odds ratio 1.66, 95% CI 1.03–2.67) associated with monotherapy success. No variables were significant for readmission on multivariable analysis. Discussion:Digoxin or propranolol may be equally efficacious for inpatient treatment of infant supraventricular tachycardia.

AbstractBackground:Children with decompensated heart failure are at high risk for arrhythmias, and ventricular assist device placement is becoming a more common treatment strategy. The impact of ventricular assist devices on arrhythmias and how arrhythmias affect the clinical course of this population are not well described. Methods and results:A single-centre retrospective analysis of children receiving a ventricular assist device between 1998 and 2011 was performed. In all, 45 patients received 56 ventricular assist devices. The median age at initial placement was 13 years (interquartile range 6–15). The median duration of support was 10 days (range 2–260). The aetiology of heart failure included cardiomyopathy, transplant rejection, myocarditis, and congenital heart disease. In all, 32 patients (71%) had an arrhythmia; 19 patients (42%) had an arrhythmia before ventricular assist device and eight patients (18%) developed new arrhythmias on ventricular assist device. Ventricular tachycardia was most common (25/32, 78%). There was no correlation between arrhythmia and risk of death or transplantation (p=0.14). Of the 15 patients who weaned from ventricular assist device, post-ventricular assist device arrhythmias occurred in nine (60%), with five (33%) having their first arrhythmia after weaning. Patients with ventricular dysfunction after ventricular assist device were more likely to have arrhythmias (p<0.02). Conclusions:Arrhythmias, especially ventricular, are common in children requiring ventricular assist device. They frequently persist for those able to wean from ventricular assist device.

AbstractValvar 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. Increased wall stress causes myocardial stretching and fibrosis, resulting in scarring; a nidus for arrhythmia development. Arrhythmias can complicate the clinical picture and increase the morbidity and mortality in patients with both congenital and acquired valvar disease. In adults with congenital heart disease, arrhythmias are the most common cause of sudden death, followed by heart failure. Valvar stenosis and insufficiency certainly contribute to this. This article highlights the need for arrhythmia surveillance for high-risk valvar lesions.

AbstractThe 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. On April, 2012, the cardiac sub-committee decided to develop similar standards in paediatric electrophysiology. A task force was created and began this process. These standards include a catalogue of required and optional equipment, as well as staff and physician credentials. We sought to establish expectations of procedural numbers by practitioner and facility. The task force surveyed the members of the Pediatric and Congenital Electrophysiology Society. Finding no consensus, the task force is committed to generate the data by requiring that the CMS participating programmes enrol and submit data to the Multicenter Pediatric and Adult Congenital EP Quality (MAP-IT™) Initiative. This manuscript details the work of the Florida CMS Paediatric Electrophysiology Task Force.

AbstractAlthough 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. Diligent arrhythmia surveillance and aggressive treatment strategies are necessary to minimise this risk. This article highlights important strategies to manage arrhythmia development and prevention in this patient population.

AbstractArrhythmias 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. The peri-operative arrhythmia burden may relate to the underlying congenital heart disease, haemodynamic perturbations, operative events, and potential residual lesions. In addition, these patients are at risk for developing arrhythmias later in life, and non-invasive and potentially invasive arrhythmia surveillance should be a routine part of the care of these patients. This article highlights important strategies to manage arrhythmia development and prevention in this patient population.

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.

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. The COstatus system (Transonic Systems, NY), which is based on ultrasound dilution technology, works off in situcatheters and uses an innocuous indicator to allow for routine measurements of cardiac output and blood volumes in pediatric patients. The purpose of this study was to validate cardiac output measured by the COstatus system with those obtained by the clinical standard technique of pulmonary artery thermodilution.

AbstractImproved survival in children with hypoplastic left heart syndrome has created a sub-population of children and young adults who are living with functionally univentricular physiology. Routine surveillance with comprehensive screening for structural cardiac disease, functional cardiac disease, arrhythmias, thromboembolic disease, and associated dysfunction of end organs is important. Future directives will better define the plans of care for routine surveillance in patients with hypoplastic left heart syndrome.

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. PARTICIPANTS Patients with ECGs referred to our institution by pediatricians with an indication of stimulant medication screening in the year after the AHA statement. INTERVENTION We compared the ECG ordering practices of community pediatricians and the outcomes of further evaluation and estimated the associated cost before and after the AHA scientific statement. MAIN OUTCOME MEASURES Abnormal ECG findings, further workup, and change in clinical practice. RESULTS In the year after publication of the 2008 AHA scientific statement, 372 ECGs were ordered with an indication of stimulant medication screening. Before publication of this statement, a mean (SD) of 6.9 (3.2) ECGs per month were referred for this indication. Despite continuing controversy, this number increased 4-fold to 31.2 (9.5) ECGs per month in the subsequent year. Twenty-four ECGs (6.4%) had abnormal findings. Eighteen patients were referred for further evaluation, and, at last follow-up, none had been found to have definitive disease. Six of 24 patients with abnormal ECG findings (25.0%) had a perceived significant delay in therapy because of the process. In responding pediatricians, 34.6% reported that the scientific statement had clearly affected their practice. CONCLUSIONS The clinical practice of community pediatricians in regard to screening ECGs and stimulant medications has been affected by the recent AHA scientific statement. The yield of performing ECGs with an indication of stimulant medication screening is very low.Arch Pediatr Adolesc Med. 2011;165(2):166-170--

Several letters to the editor are presented about adolescence, reading poetry, and colleges.