Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty‐two US pediatric institutions participated in a 10‐year review (2008–2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management. Geocoding was used to determine a census tract‐based socioeconomic deprivation index. Descriptive statistics of patient characteristics and regression analysis of RHD classification, disease severity, and initial antibiotic prescription according to community deprivation were obtained. Data for 947 cases showed median age at diagnosis of 9 years; 51% and 56% identified as male and non‐White, respectively. Most (89%) had health insurance and were first diagnosed in the United States (82%). Only 13% reported travel to an endemic region before diagnosis. Although 96% of patients were prescribed secondary prophylaxis, only 58% were prescribed intramuscular benzathine penicillin G. Higher deprivation was associated with increasing disease severity (odds ratio, 1.25; 95% CI, 1.08–1.46). Conclusions The majority of recent US cases of ARF and RHD are endemic rather than the result of foreign exposure. Children who live in more deprived communities are at risk for more severe disease. This study demonstrates a need to improve guideline‐based treatment for ARF/RHD with respect to secondary prophylaxis and to increase research efforts to better understand ARF and RHD in the United States.

Objectives: Shock refractory to fluid and catecholamine therapy has significant morbidity and mortality in children. The use of methylene blue to treat refractory shock in children is not well described. We aim to collect and summarize the literature and define physicians’ practice patterns regarding the use of methylene blue to treat shock in children. Design: We conducted a systematic search of MEDLINE, Embase, PubMed, Web of Science, Cochrane for studies involving the use of methylene blue for catecholamine-refractory shock from database inception to 2019. Collected studies were analyzed qualitatively. To describe practice patterns of methylene blue use, we electronically distributed a survey to U.S.-based pediatric critical care physicians. We assessed physician knowledge and experience with methylene blue. Survey responses were quantitatively and qualitatively evaluated. Setting: Pediatric critical and cardiac care units. Patients or Subjects: Patients less than or equal to 25 years old with refractory shock treated with methylene blue. Interventions: None. Measurements and Main Results: One-thousand two-hundred ninety-three abstracts met search criteria, 139 articles underwent full-text review, and 24 studies were included. Studies investigated refractory shock induced by a variety of etiologies and found that methylene blue was generally safe and increased mean arterial blood pressure. There is overall lack of studies, low number of study patients, and low quality of studies identified. Our survey had a 22.5% response rate, representing 125 institutions. Similar proportions of physicians reported using (40%) or never even considering (43%) methylene blue for shock. The most common reasons for not using methylene blue were unfamiliarity with this drug, its proper dosing, and lack of evidentiary support. Conclusions: Methylene blue appears safe and may benefit children with refractory shock. There is a stark divide in familiarity and practice patterns regarding its use among physicians. Studies to formally assess safety and efficacy of methylene blue in treating pediatric shock are warranted.

Introduction: Rheumatic heart disease (RHD) tracks with other cardiovascular health inequities in the US; however, RHD mortality captures a fraction of those affected and lags behind diagnosis by decades. There is little contemporary data on children living with acute rheumatic fever (ARF) and RHD within the US. This study describes recent pediatric ARF/RHD in the US and examines the association with community deprivation. Methods: Sixty US institutions were invited to participate in a 10-year review (2008-2018). Geocoding was used to determine a census tract-based socioeconomic deprivation index (DI) ranging from 0-1 (1 being most deprived). Independent sample t-test was used to compare DI means. Odds ratios (OR) with 95% confidence intervals were obtained from multivariable logistic regression. The DI was scaled to provide ORs for a one standard deviation increase. Results: Data for 947 cases from 22 institutions showed median age at diagnosis of 9 years, with half identifying as male (51%) and non-white (52%), and three-quarters (74%) identifying as non-Hispanic. Most identified English as their primary language, had health insurance, and were first diagnosed in the US (84%, 89%, and 82%, respectively). Of the 157 (17%) with travel exposure, Pacific Islands (38%) and Africa (21%) were most commonly identified. Nearly three-quarters (73%) were diagnosed with ARF and most (98%) had an echocardiogram at diagnosis. While 96% of patients were prescribed secondary prophylaxis, only half (58%) were prescribed intramuscular Benzathine penicillin (BPG), the gold standard. The mean DI was 0.39±0.15. Higher deprivation was associated with RHD as opposed to ARF at diagnosis (DI 0.42 vs. 0.38, p<0.001). Additionally, higher deprivation was associated with increased risk of severe RHD (OR 1.34; 95% CI 1.11-1.62) and increased use of BPG vs enteral penicillin for secondary prevention (OR 0.67; 95% CI 0.56-0.8). Conclusions: Recent pediatric cases of ARF and RHD in the US are endemic, rather than from foreign exposure and children who live in more deprived communities are at risk for more severe disease. Additional research is needed into why intramuscular penicillin, which has superior efficacy compared to oral penicillin, is prescribed in only half of cases.

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We report a neonate with severe Marfan syndrome (MS), prenatally identified to have persistent atrial tachycardia, biventricular dysfunction, and an unusual structure within the atria. Detailed postnatal echocardiographic evaluation and cross‐sectional imaging confirmed congenital pseudoaneurysm of the mitral‐aortic intervalvular fibrosa. Emergent testing by next‐generation sequencing identified a FBN1 pathological variant, key to establishing goals of care. To our knowledge, this is the first reported case of a congenital pseudoaneurysm of the mitral‐aortic intervalvular fibrosa in MS.

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