Matthew D. Elias

Pulmonary outflow tract obstruction in fetuses with complex congenital heart disease: predicting the need for neonatal intervention

To identify prenatal echocardiographic markers that could predict the need for neonatal intervention in fetuses with right ventricular outflow tract obstruction.

Prevalence of electrocardiogram use in infants with apparent life-threatening events: a multicenter database study.

Objective An apparent life-threatening event (ALTE) is a common diagnosis in pediatrics, but there is no standardized method to evaluate these patients. We sought to determine the prevalence of electrocardiogram (ECG) use in patients presenting to children’s hospitals with an ALTE. Methods The data from the Pediatric Health Information System database from 43 children’s hospitals were collected during a 15-month period between October 2009 and December 2010. Patients were included if they were younger than 1 year at the time of presentation. Demographic data, including age, length of hospital stay, second ALTE, and survival, were recorded, along with the prevalence of ECGs and International Classification of Diseases, Ninth Revision, cardiac diagnoses. Results There were 2179 patients with an ALTE, with a mean age of 65.7 days old and length of stay of 3.4 days. A total of 947 (43%) of these patients received an ECG. The prevalence of ECG use and cardiac diagnoses were variable among the participating hospitals. Depending on the institution, 0% to 93% of patients had an ECG, and 4% to 39% of patients had an International Classification of Diseases, Ninth Revision, cardiac diagnosis. Conclusions Electrocardiograms are performed in fewer than half of patients with ALTE presenting to children’s hospitals. There is wide variation in the prevalence of ECG use as a diagnostic tool for infants presenting with an ALTE.

Long-Term Outcomes of Pediatric Cardiac Patients Supported by Extracorporeal Membrane Oxygenation

Objective: Extracorporeal membrane oxygenation is an important form of short-term mechanical support in children with cardiac disease, but information on long-term outcomes and quality of life is limited. The primary objective of this study was to determine the long-term outcomes of children previously supported by extracorporeal membrane oxygenation for cardiac etiologies. Design: A retrospective analysis was performed on patients with cardiac disease managed with extracorporeal membrane oxygenation between January 1, 1995, and December 31, 2012, at the Children’s Hospital of Philadelphia. Survivors completed patient- and parent-reported verbal and written surveys, and univariate analyses assessed risk factors for long-term outcomes. Setting: Tertiary-care children’s hospital. Patients: Patients with cardiac disease managed with extracorporeal membrane oxygenation. Interventions: None. Measurements and Main Results: Over 18 years, 396 patients were managed with extracorporeal membrane oxygenation with 43% survival to discharge. The median age at cannulation was 78 days. The majority had congenital heart disease (86%), surgery prior to extracorporeal membrane oxygenation (71%), and cardiopulmonary arrest as the primary extracorporeal membrane oxygenation indication (53%). With 6-year median follow-up, 66% are known to be deceased, including 38 deaths after hospital discharge. Among survivors at discharge, 65 (38%) completed the phone survey, and 33 (19%) completed the written survey. Negative clinical outcomes, defined as having at least significant physical limitations or “fair” or “poor” health, were present in 18% of patients. No patient- or extracorporeal membrane oxygenation–related variables were associated with negative outcomes in univariate analyses. There were significantly lower self-reported and parent-reported written Pediatric Quality of Life Inventory quality of life scores in children compared with healthy individual normative data but no differences in adolescents. Conclusions: In this series of pediatric cardiac patients supported by extracorporeal membrane oxygenation, mortality was 66% with 6-year median follow-up. The majority reported positive outcomes with respect to health and physical limitations, but children reported lower quality of life compared with healthy individuals.

Effects of Exercise Restriction on Patients With Anomalous Aortic Origin of a Coronary Artery.

Background: Management of young patients with anomalous aortic origin of a coronary artery (AAOCA) may involve exercise restriction. We sought to identify the association of exercise restriction with changes over time in body mass index (BMI) and exercise capacity in this cohort. Methods: We performed a retrospective review of patients with AAOCA seen at The Children’s Hospital of Philadelphia between January 1, 1998, and August 31, 2014. Linear mixed model repeated-measures analysis assessed changes in BMI and exercise capacity. Results: We included 72 patients with a median age at presentation of 12.6 years (interquartile range: 10.1-15.8) and mean follow-up of 3.6 ± 3.0 years. The majority had an anomalous right coronary artery (71%) and interarterial ± intramural coronary course (90%). Surgery was performed in 54%, more often in those with interarterial/intramural course (P < .001) and symptoms (P = .003). Most patients (82%) were exercise-restricted on presentation, and restricted patients were older than those who were not restricted (P = .01). There was no significant difference between restricted and nonrestricted patients in initial BMI z scores, percentage of patients with BMI over 85th percentile (26%) or exercise capacity variables. In univariable analysis, exercise restriction over time was not associated with change in BMI z score (P = .25) or change in exercise variables. Restriction was not associated with significant change in these variables in multivariable analysis. Conclusions: Although further investigation is warranted to determine the degree of adherence to exercise restriction, the recommendation of restriction alone is not associated with increasing BMI or decreasing exercise performance in the short-term.

Acute disseminated encephalomyelitis following meningoencephalitis: case report and literature review.

Abstract Meningoencephalitis and acute disseminated encephalomyelitis (ADEM) are both neurological disease processes, but there have been few cases of meningoencephalitis progressing to ADEM in the pediatric population. A case of a 4-year-old girl with an initial diagnosis of meningoencephalitis is presented here, whose initial presentation was manifested by prolonged fever, gray matter signal abnormality on brain magnetic resonance imaging, cerebrospinal fluid pleocytosis, and a markedly irritable mental status. As her neurological examination changed with focal abnormalities, a repeat magnetic resonance imaging demonstrated new areas of both gray and white matter signal abnormality, consistent with ADEM. Her symptoms and imaging findings completely resolved with a course of methylprednisolone. Based on the literature and this current case, it is our recommendation to consider ADEM as a diagnosis if meningoencephalitis is not improving.

Exercise restriction is not associated with increasing body mass index over time in patients with anomalous aortic origin of the coronary arteries

Anomalous aortic origin of the coronary arteries is associated with exercise-induced ischaemia, leading some physicians to restrict exercise in patients with this condition. We sought to determine whether exercise restriction was associated with increasing body mass index over time. From 1998 to 2015, 440 patients ⩽30 years old were enrolled into an inception cohort. Exercise-restriction status was documented in 143 patients. Using linear mixed model repeated-measures regression, factors associated with increasing body mass index z-score over time, including exercise restriction and surgical intervention as time-varying covariates, were investigated. The 143 patients attended 558 clinic visits for which exercise-restriction status was recorded. The mean number of clinic visits per patient was 4, and the median duration of follow-up was 1.7 years (interquartile range (IQR) 0.5-4.4). The median age at first clinic visit was 10.3 years (IQR 7.1-13.9), and 71% (101/143) were males. All patients were alive at their most recent follow-up. At the first clinic visit, 54% (78/143) were exercise restricted, and restriction status changed in 34% (48/143) during follow-up. The median baseline body mass index z-score was 0.2 (IQR 0.3-0.9). In repeated-measures analysis, neither time-related exercise restriction nor its interaction with time was associated with increasing body mass index z-score. Surgical intervention and its interaction with time were associated with decreasing body mass index z-score. Although exercise restriction was not associated with increasing body mass index over time, surgical intervention was associated with decreasing body mass index z-score over time in patients with anomalous aortic origin of the coronary arteries.

RISK FACTORS FOR MORTALITY IN PEDIATRIC CARDIAC INTENSIVE CARE UNIT PATIENTS MANAGED WITH EXTRACORPOREAL MEMBRANE OXYGENATION IN A CONTEMPORARY COHORT

Extracorporeal membrane oxygenation (ECMO) utilization for children with cardiac disease has increased over the past 20 years, yet mortality remains high. In a previous study (1995-2001), we reported 39% survival to discharge. This study aims to report mortality for pediatric cardiac patients