Multisystem Inflammatory Syndrome in Children: Survey of Protocols for Early Hospital Evaluation and Management.

OBJECTIVE: To describe the similarities and differences in the evaluation and treatment of multisystem inflammatory syndrome in children (MIS-C) at hospitals in the US. STUDY DESIGN: We conducted a cross-sectional survey from June 16 to July 16, 2020, of US children's hospitals regarding protocols for management of patients with MIS-C. Elements included characteristics of the hospital, clinical definition of MIS-C, evaluation, treatment, and follow-up. We summarized key findings and compared results from centers in which >5 patients had been treated vs those in which ≤5 patients had been treated. RESULTS: In all, 40 centers of varying size and experience with MIS-C participated in this protocol survey. Overall, 21 of 40 centers required only 1 day of fever for MIS-C to be considered. In the evaluation of patients, there was often a tiered approach. Intravenous immunoglobulin was the most widely recommended medication to treat MIS-C (98% of centers). Corticosteroids were listed in 93% of protocols primarily for moderate or severe cases. Aspirin was commonly recommended for mild cases, whereas heparin or low molecular weight heparin were to be used primarily in severe cases. In severe cases, anakinra and vasopressors frequently were recommended; 39 of 40 centers recommended follow-up with cardiology. There were similar findings between centers in which >5 patients vs ≤5 patients had been managed. Supplemental materials containing hospital protocols are provided. CONCLUSIONS: There are many similarities yet key differences between hospital protocols for MIS-C. These findings can help healthcare providers learn from others regarding options for managing MIS-C.

Coronavirus Disease 2019 Infection in Children with Pre-Existing Heart Disease.

We present 7 children with congenital heart disease and coronavirus disease 2019. Of these, 5 were younger than 1 year of age and 3 had atrioventricular canal defect and trisomy 21. All 7 developed acute decompensation, with 1 death in an 18-year-old with hypertrophic cardiomyopathy and other comorbidities.

Neonatal Nurse Practitioner Use of Ultrasonography to Verify Umbilical Venous Catheter Placement in the Neonatal Intensive Care Unit.

BACKGROUND: Literature dating back to 1982 supports the use of ultrasound to confirm UVC position as a more reliable method compared to x-ray. The use of ultrasound to confirm UVC position remains infrequent in the NICU. PURPOSE: To determine the feasibility of training an NNP in the acquisition and interpretation of a parasternal short-axis (PSAX) image of the heart to confirm UVC position. METHODS: A retrospective review of 48 charts evaluated descriptive data using standard technique of an x-ray to confirm UVC placement. An NNP was trained to acquire and interpret a parasternal short-axis (PSAX) cardiac view by a pediatric cardiologist. The NNP independently and prospectively evaluated 21 neonates and determined whether the UVC was in satisfactory or unsatisfactory position. The same image was blindly over-read by the pediatric cardiologist. Agreement between the NNP and pediatric cardiologist interpretations as well as the pediatric cardiologist and radiologist interpretations was evaluated using the κ-test. RESULTS: Initial pediatric cardiology interpretation of UVC position showed the UVC to be malpositioned in 66% of neonates (n = 14). NNP and pediatric cardiology interpretations in UVC position had 86% agreement (κ-0.667). Pediatric cardiology (ultrasound) and radiology interpretations (x-ray) in UVC position had 86% agreement (κ-0.69). IMPLICATIONS FOR PRACTICE: It is feasible to train NNPs to perform and accurately interpret a single PSAX ultrasound image to determine satisfactory UVC position. IMPLICATIONS FOR RESEARCH: Increasing the use of ultrasound to determine satisfactory position of UVCs in the NICU can reduce unnecessary exposure to ionizing radiation in neonates.

Fetal diagnosis of left-ventricular noncompaction cardiomyopathy in identical twins with discordant congenital heart disease.

Left-ventricular noncompaction (LVNC) is characterized by prominent myocardial trabeculae with deep intratrabecular recesses. Associated with ventricular dysfunction, LVNC can present in isolation or in conjunction with structural heart defects (i.e., "nonisolated" LVNC). Although it was once considered to be difficult and unreliable, successful detection of this condition by way of fetal echocardiography has been well documented. We present what is to our knowledge the first prenatal diagnosis of LVNC in monochorionic-monozygotic twins. Nonisolated LVNC was identified in twins A and B at 23 weeks' gestation. A noncompaction-to-compaction ratio >2 was documented in both cases. Discordance for congenital heart disease was observed in the twins: Twin A presented with pulmonary atresia with an intact ventricular septum, critical tricuspid stenosis, and severe right-ventricular hypoplasia, whereas twin B presented with mild mitral valve dysplasia. Despite an initially complicated postnatal course for twin A, this case shows that it is feasible to achieve good outcomes with accurate prenatal detection and aggressive postnatal management.

Pericardiectomy for Successful Treatment of Constrictive Pericarditis in a Pediatric Patient.

A 15-year-old girl with history of asthma and obesity presented with recurrent anasarca without systolic heart failure or significant renal disease. She was diagnosed with constrictive pericarditis and successfully underwent pericardiectomy with pericardial stripping and a waffle procedure. (Level of Difficulty: Advanced.).

Persistent great artery dilatation in Beals syndrome: A novel finding.

We report a unique case of dilated aortic root and pulmonary artery in an infant with clinical features consistent with Beals syndrome confirmed to have fibrillin-2 mutation. This case highlights a novel finding of main pulmonary artery dilatation that has not been previously reported with Beals syndrome or fibrillin-2 mutation. In addition, the importance of serial echocardiography and consideration of medical management is discussed.

Left Ventricular Hypertrophy in Pediatric Hypertension: A Mini Review.

Adults with arterial hypertension (HTN) have stroke, myocardial infarction, end-stage renal disease (ESRD), or die at higher rates than those without. In children, HTN leads to target organ damage, which includes kidney, brain, eye, blood vessels, and heart, which precedes "hard outcomes" observed in adults. Left ventricular hypertrophy (LVH) or an anatomic and pathologic increase in left ventricular mass (LVM) in response to the HTN is a pediatric surrogate marker for HTN-induced morbidity and mortality in adults. This mini review discusses current definitions, clinically relevant methods of LVM measurements and normalization methods, its epidemiology, management, and issue of reversibility in children with HTN. Pediatric definition of LVH and abnormal LVM is not uniformed. With multiple definitions, prevalence of pediatric HTN-induced LVH is difficult to ascertain. In addition while in adults cardiac magnetic resonance imaging is considered "the gold standard" for LVM and LVH determination, pediatric data are limited to "special populations": ESRD, transplant, and obese children. We summarize available data on pediatric LVH treatment and reversibility and offer future directions in addressing LVH in children with HTN.

Assessment of Left Ventricular Mass and Hypertrophy by Cardiovascular Magnetic Resonance Imaging in Pediatric Hypertension.

Cardiovascular magnetic resonance (CMR) imaging in adults is considered the gold standard for assessment of left ventricular mass (LVM) and left ventricular hypertrophy (LVH). The authors aimed to evaluate agreement of LVM measurements and LVH determination between echocardiography (ECHO) and CMR imaging in children with hypertension (HTN) confirmed by 24-hour ambulatory blood pressure monitoring (ABPM). The children (n=22) underwent contemporaneous ECHO, CMR imaging, and ABPM. Patients had a mean body mass index of 30.9±7.5 (kg/m2 ), and 81.8% had severe HTN. LVM measured by ECHO was 189.6±62.1 g and by CMR imaging was 164.6±44.7 g (P<.0001). Bland-Altman analysis revealed significant variability between ECHO and CMR imaging in the measurement of LVM. Interobserver error was higher with ECHO than with CMR imaging. ECHO had high sensitivity and low specificity in LVH determination. In conclusion, ECHO overestimates LVM and is less accurate in measuring LVM as compared with CMR imaging in children with HTN. Further prospective study using CMR imaging to assess LVM in children is warranted.

Prevalence and outcomes of coronary artery ectasia associated with isolated congenital coronary artery fistula.

Isolated congenital coronary artery fistula (CAF) is rare and varies with respect to size and hemodynamic significance. The prevalence of coronary artery ectasia in association with isolated congenital CAF, regardless of size, and after closure of large fistulae has not been systematically evaluated in the literature. This study aimed to characterize the demographic and echocardiographic differences between patients with large and small fistulae and to describe outcomes with respect to coronary ectasia in those who underwent closure. This is a retrospective review of an echocardiographic database that identified patients coded for CAF (1995 to 2012) and excluded those associated with complex cardiac disease and/or coronary anomalies and cardiomyopathy. Small fistulae were noted to arise mostly from the left anterior descending artery, drain into the pulmonary artery, and have a very low incidence of ectasia (n = 3 of 92), with a mean coronary artery diameter z score in these 3 patients of 3.45 ± 1.15. Larger fistulae had a female predominance, with most originating from the right coronary artery and draining into the right atrium; among the 12 patients who underwent procedural closure of large CAF, all feeding coronary arteries remained ectatic after closure, with a mean coronary artery diameter z score of 9.54 ± 5.66 after a total mean follow-up time of 3.95 ± 4.07 years. In conclusion, the occurrence of coronary dilatation justifies long-term follow-up irrespective of fistula size and successful closure of large CAF.