Rheumatic Heart Disease in the United States: Forgotten But Not Gone: Results of a 10 Year Multicenter Review.

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.

Optimized Computed Tomography Angiography Protocol for the Evaluation of Thrombus in Patients with Fontan Anatomy.

The Fontan procedure is the final stage in the palliative surgical approach to patients with single-ventricle physiology. These patients have an increased risk for thromboembolic disease in the Fontan circuit, which can be evaluated by chest computed tomography angiography (CTA) in acute settings. However, false-positive results are common secondary to unusual streaming patterns in the Fontan circuit. A biphasic CTA protocol was evaluated for the capability to clearly identify structures of the Fontan circuit that are critical for the evaluation of thromboembolic disease. The study was a retrospective chart review of Fontan patients with a chest CTA scan obtained between 2011 and 2017. Two pediatric cardiologists with additional training in cardiac CT imaging independently reviewed each CTA and awarded one point for each of 5 Fontan circuit structures clearly identified resulting in a score range of 0-5. A score of 0-2 considered not capable, 3-4 partially capable, and 5 capable to clearly identify critical structures of the Fontan circuit. During the study period, 46 CTA scans were performed on 21 patients. Of the CTA scans using a biphasic protocol, 62.5% (10/16) were considered capable to clearly identify all 5 critical structures of the Fontan circuit vs 27% (8/30) of the CTA scans using a monophasic protocol (p = 0.027). Overall our results suggest that the single-site biphasic CTA protocol has greater diagnostic capability to detect the presence of Fontan thromboembolic disease when compared to the more traditional monophasic CTA protocol. Future prospective studies are needed to confirm these findings.