Background— There is a lack of clear diagnostic and management guidelines for acute myocarditis in the pediatric population. We used a multi-institutional database to characterize demographics, practice variability, and outcomes in this population. Methods and Results— Patients with acute myocarditis (n=514) were identified from April 2006 to March 2011 using the Pediatric Health Information System database, and regional variations in management and outcomes were analyzed. Ninety-seven patients (18.9%) received extracorporeal membrane oxygenation, 22 (4.3%) received ventricular assist device, 21 (4.1%) received heart transplantation, and 37 (7.2%) died. Of the 104 patients who received extracorporeal membrane oxygenation or ventricular assist device, 17 (16.3%) had heart transplantation, 25 (24%) died, and 62 (59.6%) showed recovery of myocardial function. There was a decrease in the use of endomyocardial biopsy ( P =0.03) and an increase in the use of magnetic resonance imaging ( P <0.01) over the study period. Although the use of medications and procedures varied between different regions, the occurrence of death or heart transplantation showed no significant regional associations. The use of extracorporeal membrane oxygenation (odds ratio, 5.8; 95% confidence interval, 2.9–11.4; P <0.01), ventricular assist device (odds ratio, 8.2; 95% confidence interval, 2.7–24.9; P <0.01), and vasoactive medications (odds ratio, 5.7; 95% confidence interval, 1.2–26.1; P =0.03) was independently associated with death/transplantation. Conclusions— There is significant temporal and regional variation in the diagnostic modalities and management used for pediatric myocarditis, which continues to have high morbidity and mortality. Extracorporeal membrane oxygenation, ventricular assist device, and vasoactive medications are independently associated with increased mortality/transplantation.

Background Cardiac disease is a major cause of death in muscular dystrophies. The use of feasible and reproducible echocardiographic measures of cardiac function is critical to advance the field of therapeutics for dystrophic cardiomyopathy. Methods Participants aged 8 to 18 years with genetically confirmed Duchenne (DMD), Becker (BMD) or limb girdle (LGMD) muscular dystrophies were enrolled at five centers and a standardized echocardiogram (echo) was performed. Measures of systolic and diastolic function and speckle tracking echo (STE) derived cardiac strain were reviewed independently by two central readers. Furthermore, echo measures from DMD participants were compared to retrospective aged matched controls from a single site to assess measures of myocardial function. Results 48 participants (mean age of 13.3±2.7 years) were enrolled. Shortening fraction (SF%) had a greater inter-observer correlation (intra-class correlation coefficient; ICC=0.63) compared to ejection fraction (EF%; ICC=0.49). One reader could only measure EF% in 53% of participants. Myocardial performance index (MPI) measured by pulse wave Doppler and tissue Doppler imaging showed similar ICCs (0.55 and 0.54). STE showed a high ICC (0.96). Focusing on DMD participants (n=33), significantly increased mitral A wave velocities, lower E/A ratios and lower TDI mitral lateral E’ velocities were observed compared to age matched controls. STE demonstrated subclinical myocardial dysfunction with decreased average circumferential and longitudinal strain in 3 distinct subgroups: DMD participants with normal SF%; DMD participants with age <13 years; and DMD participants with MPI<0.40 compared to controls. Conclusions In a muscular dystrophy cohort, assessment of cardiac function is feasible and reproducible using SF%, diastolic measures and MPI. Cardiac strain measures identified early myocardial disease in DMD.

Glomerular hyperfiltration is common in children with malignancies at diagnosis and during initial cycles of chemotherapy. It is particularly prevalent in patients with central nervous tumors, which are frequently smaller in volume. Therefore, the pathophysiological mechanism of hyperfiltration cannot be explained solely on the basis of large tumor volume and subsequent cell breakdown. We hypothesize that host hypermetabolic state plays an important role in pathophysiology of hyperfiltration.

This study presents age-stratified radiation dose values for 6 common congenital heart interventional catheterization procedures. Fluoroscopy time alone is not an adequate measure for monitoring radiation exposure. These values will be used as baseline for measuring the effectiveness of future quality improvement activities by the Congenital Cardiac Catheterization Project on Outcomes collaborative.

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In comparison to Fontan patients with a dominant left ventricle, those with a dominant RV have higher fiber stress, a higher rate of ventricular dilatation, lower circumferential fiber shortening, and similar longitudinal shortening. RV morphology, ventricular dilation, and worse circumferential strain are associated with death or heart transplantation. The difference in myofiber architecture may contribute to suboptimal adaptation of the RV as a systemic ventricle.

Figure. A, Prenatal echocardiogram (Echo).Top, The aorta (Ao) and the main pulmonary artery (MPA) in the long-axis view with a large defect between them. Middle, Short-axis image showing again that the aorta and MPA are normally related to each other and that there is a large communication between the 2 great vessels. The left pulmonary artery (LPA) arises normally from the MPA, but the right pulmonary artery (RPA) arises from the aorta. Bottom, The MPA gives rise to the ductal arch and the LPA underneath. B, Postnatal echocardiogram images confirm the fetal diagnosis. Images are arranged side-by-side and in an orientation similar to that of the prenatal images. C, Part of the surgical repair included closure of the aortopulmonary window (APW) to permit communication between the MPA and RPA (longer dashed line) and aortic root enlargement with a patch (short dashed line). D, Postsurgery discharge echocardiogram. Top, Absence of residual aortopulmonary communication and mild RPA stenosis. Bottom, Unobstructed aortic arch (Arch). Duct indicates ductus arteriosus; and SVC, superior vena cava.

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