Prolonged Elevations of Factor VIII and von Willebrand Factor Antigen After Multisystem Inflammatory Syndrome in Children.

Multisystem Inflammatory Syndrome in Children (MIS-C) is a late systemic inflammatory response to a recent mild or asymptomatic coronavirus disease of 2019 infection. The pathophysiology is incompletely understood but it often features significant coagulopathy along with cardiac and endothelial dysfunction. Endothelial inflammation has been primarily described in acute coronavirus disease of 2019 infection, with less characterization in MIS-C. Here we describe novel findings of nearly universal severe and prolonged factor VIII (FVIII) and von Willebrand factor antigen elevations in an institutional cohort of patients with MIS-C ages younger than or 21 years old (N=31). All patients had elevated acute phase reactants and D-dimer at presentation and met published criteria for MIS-C. FVIII was high at presentation in 97% of patients but continued to rise during the ensuing weeks of treatment to a mean 429%, peaking on median day 17 of illness as an outpatient. FVIII levels were >600% in multiple patients. von Willebrand factor antigen was measured less frequently but showed similar trends. These escalations occurred amidst resolving cardiac dysfunction and acute phase reactant normalization and despite patients receiving multimodal anti-inflammatory treatments and aspirin and enoxaparin thromboprophylaxis. No thrombotic events occurred. Endothelial dysfunction represented by very elevated FVIII levels may persist longer than other acute phase reactants may reflect.

Anatomic Approach and Outcomes in Children Undergoing Percutaneous Pericardiocentesis.

Pericardiocentesis is traditionally performed using a subxiphoid approach. Hepatomegaly or loculated and noncircumferential effusions warrant nonstandard approaches to drain effusions; echocardiographic guidance has made these less traditional, non-subxiphoid approaches feasible. The study is aimed at comparing clinical outcomes of the subxiphoid and non-subxiphoid approaches to percutaneous pericardiocentesis in a pediatric population. This is a retrospective chart review of all children undergoing percutaneous pericardiocentesis from August 2008 to December 2019 at a single-center. A total of 104 patients underwent echocardiography-guided pericardiocentesis during the timeframe. Additionally, fluoroscopy was also used in 80 patients. Hematopoietic stem cell transplantation was the most common underlying diagnosis (n = 53, 50.9%). A non-subxiphoid approach was used in 58.6% (n = 61) of patients. The fifth and sixth intercostal spaces were the most commonly used (n = 17 each). The non-subxiphoid group tended to be older (95.9 vs. 21.7 months, p = 0.006) and weighed more (23.6 vs. 11.2 kgs, p = 0.013) as compared to the subxiphoid group. Non-subxiphoid approach was associated with shorter procedure times (21 vs. 37 min, p = 0.005). No major complications were seen. Five minor complications occurred and were equally distributed in the two groups. Complications were more likely in younger patients (p = 0.047). The technique and anatomic approach to pericardiocentesis, and the location or size of effusion did not influence the risk of complications. Echocardiography-guided percutaneous pericardiocentesis in children was associated with low complication rates in this single-center pediatric experience. The use of a non-traditional, non-subxiphoid approach was associated with shorter procedure times and did not significantly affect complication rates.

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.

Outcomes of Surgical Repair of Vascular Rings and Slings in Children: A Word for the Asymptomatic.

Vascular rings (VRs) are rare aortic arch anomalies that may present with a wide variety of symptoms related to esophageal and/or airway compression. We reviewed our surgical experience in both symptomatic and asymptomatic children. All children (n = 58) who underwent surgical repair of VRs or slings (mean age 27.4 ± 45.60 months; 36 males [62%]) between March 2000 and April 2020 were included. The most common anatomic variant was a right aortic arch (RAA) with aberrant left subclavian artery (ALSCA) (n = 29; 50%). Kommerell's diverticulum was present in 23 of these patients (79%). The second most common variant was a double aortic arch (n = 22; 38%), followed by pulmonary artery sling (n = 4; 6%), RAA with mirror image branching and left ligamentum arteriosum (n = 3; 5.2%), and left aortic arch (LAA) with aberrant right subclavian artery (n = 1; 1.7%). One patient had a double ring with pulmonary artery sling and RAA with ALSCA. Symptoms were present in 42 patients (72%). Left lateral thoracotomy was the approach in 50 patients (86%), while sternotomy was used in 8 (14%). Symptomatic improvement occurred in the majority of symptomatic patients (93%). There was one perioperative mortality (1.7%) in the symptomatic group which was non-VR related. Morbidities included recurrent laryngeal nerve injury in three patients (5.2%) and transient chylothorax in two (3.4%). Persistence/recurrence of symptoms resulted in one early and one late reoperation. The mean follow-up was 3 ± 5 years. In the current era, VR repair in children including asymptomatic ones can be performed with excellent results. We recommend complete repair of RAA with aberrant LSCA by resection of Kommerell's diverticulum and translocation of the ALSCA to avoid recurrence.

Implementation of Developmental Screening Guidelines for Children with Congenital Heart Disease.

OBJECTIVES: To assess awareness and implementation among pediatric primary care providers of the 2012 American Heart Association (AHA) guidelines for the evaluation and management of developmental abnormalities in children with congenital heart disease (CHD). We hypothesized that children with CHD are not being provided neurodevelopmental screening and support according to the AHA guidelines. STUDY DESIGN: An online survey was administered to licensed pediatric primary care providers in Minnesota (pediatricians = 530, family physicians = 1469) to evaluate awareness of the AHA guidelines, current screening practices, and barriers to implementation of these guidelines. RESULTS: A total of 326 providers (17% of 1911 successful e-mails) responded to the survey, which included 148 pediatricians (29% of 518 successful e-mails) and 178 family physicians (13% of 1393 successful e-mails). Overall, 202 providers (62%) reported caring for children with CHD. Among those caring for children with CHD, the most commonly reported reasons for neurodevelopmental referral were nonspecific to CHD. Presence of risks specific to children with CHD, such as history of cyanotic heart disease or open heart surgery as an infant, accounted for only 25% and 22% of the referrals, respectively. Only 21% of providers were aware of the guidelines, and only 7% received guidance from a pediatric cardiologist regarding neurodevelopmental screening in children with CHD. CONCLUSION: There is need for further education of primary care providers on the developmental risks associated with CHD as well as increased involvement by the pediatric cardiology community to enhance the developmental outcomes of children with CHD.