Multisystem Inflammatory Syndrome in Children in February 2020 and Implications of Genomic Sequencing for SARS-CoV-2.

Multisystem inflammatory syndrome in children (MIS-C) is a newly recognized disease process that can complicate severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. We present what we believe to be the earliest case of MIS-C, occurring in February 2020. Our patient's SARS-CoV-2 infection was caused by an emerging lineage with the D614G variant in the spike protein. This lineage would subsequently become the predominant cause of SARS-CoV-2 outbreaks in Europe and the United States where MIS-C was first described.

Newborn Screening Pulse Oximetry to Detect Critical Congenital Heart Disease: A Follow-Up Survey of Current Practice at Army, Navy and Air Force Hospitals.

INTRODUCTION: The purpose of this study was to assess the evolution of newborn pulse oximetry screening (+POx) among Army, Air Force, and Naval military hospitals (MH), including prevalence, protocol use, quality assurance processes, access to echocardiography, and use of telemedicine. This is a follow-up from a prior study published in 2011. MATERIALS AND METHODS: An Internet-based questionnaire was forwarded to the chief pediatrician at MH worldwide which support newborn deliveries. Descriptive data were reported using percentages. Grouped responses, as applicable, were further compared using the chi-square test. A p-value < 0.05 was considered statistically significant. RESULTS: Seventy-eight percent (36/46) of MH supporting deliveries worldwide responded to the survey (17 Army hospitals, 11 Navy Hospitals, 8 Air Force hospitals). All responding hospitals utilize +POx, of which 94% endorsed protocol compliance with the American Academy of Pediatrics guidelines. Nine (25%) hospitals were located outside of the United States. Delivery volumes (infants per month) range between 1-49 (36%), 50-99 (28%), 100-199 (19%), and 200-300 (17%). Eleven hospitals reported regular review of +POx data, with most reviewing them monthly. Four MH share findings with state institutions. Ten hospitals either have a staff pediatric cardiologist or use tele-echocardiography for on-site evaluations. Ten hospitals are located greater than 60 miles from the nearest center with echocardiography capabilities. Of the five hospitals using tele-echocardiography, four confirmed critical congenital heart disease (CCHD) using this practice, and all five reported averting transfer of an infant using this technology. Of the 22 hospitals lacking the ability to obtain on-site echocardiography, 12 (55%) are interested in implementing a tele-echocardiography protocol. CONCLUSIONS: All responding MH use +POx, representing significant increase from the 30% of MH reporting use of +POx seven years ago. The majority of MH follow AAP +POx guidelines, and though most have providers review results prior to discharge, only one-third report periodic chart review for quality assurance. Most MH transfer infants with positive +POx results for evaluation due to a lack of on-site echocardiography. Tele-echocardiography was reported as a potential solution to diagnose or rule out CCHD. Over half of remaining hospitals without cardiologists are interested in using this technology to evaluate stable infants with positive CCHD screening.

Abnormal myocardial blood flow in children with mild/moderate aortic stenosis.

OBJECTIVE: To quantify myocardial blood flow in infants and children with mild or moderate aortic stenosis using adenosine-infusion cardiac magnetic resonance. BACKGROUND: It is unclear whether asymptomatic children with mild/moderate aortic stenosis have myocardial abnormalities. In addition, cardiac magnetic resonance-determined normative myocardial blood flow data in children have not been reported. METHODS: We studied 31 infants and children with either haemodynamically normal hearts (n=20, controls) or mild/moderate aortic stenosis (n=11). The left ventricular myocardium was divided into six segments, and the change in average segmental signal intensity during contrast transit was used to quantify absolute flow (ml/g/minute) at rest and during adenosine infusion by deconvolution of the tissue curves with the arterial input of contrast. RESULTS: In all the cases, adenosine was well tolerated without complications. The mean pressure gradient between the left ventricle and the ascending aorta was higher in the aortic stenosis group compared with controls (24 versus 3 mmHg, p<0.001). Left ventricular wall mass was slightly higher in the aortic stenosis group compared with controls (65 versus 50 g/m², p<0.05). After adenosine treatment, both the absolute increase in myocardial blood flow (p<0.0001) and the hyperaemic flow significantly decreased (p<0.001) in children with mild/moderate aortic stenosis compared with controls. CONCLUSION: Abnormal myocardial blood flow in children with mild/moderate aortic stenosis may be an important therapeutic target.

Dofetilide for atrial arrhythmias in congenital heart disease: a multicenter study.

BACKGROUND: Very little is known about use of the class III antiarrhythmic dofetilide in patients with congenital heart disease (CHD). METHODS: A multicenter retrospective review of experience with dofetilide in CHD patients was undertaken. RESULTS: Twenty adults with CHD and refractory atrial arrhythmias were treated with dofetilide at four institutions over a 7-year period. Three (15%) experienced adverse effects during in-hospital initiation of dofetilide (two with torsade de pointes, one with excessive QTc prolongation) and were not continued on this therapy. The remaining 17 were discharged taking dofetilide, with either resolved or improved arrhythmia. One was lost to follow-up. Five subsequently discontinued dofetilide due to waning effectiveness, manifest by recurrence of their arrhythmias. Eleven (55%) remained on dofetilide at most recent visit, with a median follow-up of nearly 1 year. Seven of these 11, or 35% of the CHD patients originally started on dofetilide, experienced a complete resolution of their arrhythmia. The remaining four had breakthrough episodes of atrial arrhythmia, but remained on dofetilide. No patient experienced torsade de pointes after the in-hospital initiation period. CONCLUSIONS: Used appropriately, dofetilide appears to be a viable adjunct to catheter-based ablation and alternative pharmacological approaches for the treatment of atrial arrhythmias in adult patients with congenital heart disease.