Quality Improvement Identifies Healthcare Transition Disparities in Adolescents with Congenital Heart Disease and Disabilities.

Introduction: We aim to implement healthcare transition (HCT) education for youth with congenital heart disease (CHD) and assess HCT preparedness for cardiac self-care. Methods: An HCT clinic was implemented at an academic pediatric cardiology clinic for CHD youth 17 years of age and older. An educator used transition readiness assessment questionnaires and discussed HCT material. The percentage of eligible youth who received HCT education and the cause for missed occurrences were tracked. Plan-do-study-act cycles began in August 2020 to improve the number of youths reached. Secondary analyses assessed improvement differences among those without cardiac procedures or disabilities. Results: HCT education provision improved from a mean of 38% to 73% in the 17-year and older age group by December 2022. Communication failure was the leading cause of missed visits in 2021 (30%), reduced to 0 by 2022 following plan-do-study-act cycles. Other missed HCT visits included clinic add-ons after screening, limited staff availability, and unidentified eligibility. Readiness assessments were similar for youth with and without prior cardiac procedures, for example, confidence in taking charge of their health care (P = 0.47) and moving to adult care (P = 0.22). Adolescents with disabilities were significantly less confident than those without disabilities in taking charge of their heart health care (6.3 versus 7.5, P = 0.04) and moving to adult care (4.9 versus 7.4, P < 0.001). Conclusions: Implementation of a CHD HCT clinic improved successful education delivery. Provider engagement and clinic staffing are important for sustainability. HCT knowledge gaps exist for all adolescents, yet those with disabilities had the greatest deficits.

Gabapentin Can Improve Irritability and Feeding Tolerance in Single Ventricle Interstage Patients: A Case Series.

Visceral hyperalgesia is common among children with complex medical conditions. Infants with complex congenital heart disease, specifically single ventricle interstage patients, are often found to have feeding intolerance and irritability. Gabapentin treatment has shown promise for symptomatic improvement for visceral hyperalgesia in some patients. We present a case series of five patients in which four of the five patients showed improvement within 48 h of starting gabapentin. The use of gabapentin in single ventricle interstage patients to treat visceral hyperalgesia shows promise based on our case series, but future multi-center prospective studies would be beneficial.

Optimizing efficiency of a custom clinical decision support tool improves adult congenital heart disease care.

Study objective: Improve the efficiency of an inpatient clinical decision support tool (CDS) for patients with adult congenital heart disease (ACHD). Design: The efficiency of a CDS was evaluated across two time periods and compared. Setting: An academic, tertiary care center. Participants: ACHD patients roomed in an inpatient setting. Intervention: Plan-Do-Study-Act (PDSA) methods were applied starting in 2021 and included refinement of diagnostic codes and the addition of department encounter codes. Main outcome measures: True positive and false positive CDS alerts. Results: Baseline data from 2017 had a median (IQR) of 38 (17) and 2019 baseline data had 65 (19) total alerts per month. Combining both baseline data years, the median true positive CDS alerts was 47.3 %. There were 71 (6) total alerts per month for the 2021-2022 time period and with ongoing PDSA cycles and optimization in the CDS the true positive alerts improved substantially resulting in a shifting of the median to 78.9 % within 9 months. Conclusion: CDS can efficiently notify providers when an ACHD patient is encountered. The use of ICD 10 codes alone to identify ACHD patients has limited accuracy with a high proportion of false positives. Ongoing revision of the CDS system methods is important to improving efficiency and minimizing provider alert fatigue.

Test-retest variability for quantitative two-dimensional and Doppler measurements in the fetus.

OBJECTIVES: Echocardiography is used to quantitatively characterize cardiovascular function in fetuses with cardiac abnormalities and inform decisions for fetal or perinatal interventions. It is clinically important to understand the reproducibility of these measures, particularly between testers. While studies have reported intra-observer variability and inter-observer variability, little is known about test-retest variability for these measures. We hypothesized that even in a high volume echocardiography laboratory, quantitative measurements will demonstrate higher test-retest variability compared with inter-observer variability and intra-observer variability of the same measurements. METHODS: Prospective study of uncomplicated, singleton pregnancies to evaluate fetal measures of cardiovascular function obtained by echocardiography. One sonographer obtained predefined variables, and then, a second sonographer obtained the same variables 15 minutes after the first sonographer. Separate data acquisitions were obtained by the two sonographers to evaluate test-retest variability. Intra-observer variability and inter-observer variability were also evaluated. RESULTS: Thirty fetuses between 17- and 36-week gestation were enrolled. Time-based variables had the best intra-observer agreement and inter-observer agreement (1.2%-7.4%), while 2D (7.5%-10%), M-mode (4.9%-10.1%), and velocity-time integral (VTI; 2.6%-13.8%) measurements had poorer agreement. For all variables, test-retest agreement was worse (3%-32.1%), particularly for measurement of myocardial performance index (MPI; 19.7%-21.1%), cardiac output estimation (27.2%-27.9%), and VTI-based indices (14.7%-32.1%). CONCLUSIONS: In a laboratory highly experienced in quantitative fetal echocardiography, intra-observer agreement and inter-observer agreement are good for most quantitative parameters. However, test-retest agreement is fair or poor for several variables, notably the MPI, cardiac output estimation, and VTI-based indices. Understanding how these measures vary between separate acquisitions is important for clinical interpretation and decision making.

Neonatal Enterovirus Myocarditis With Severe Dystrophic Calcification: Novel Treatment With Pocapavir.

Dystrophic myocardial calcification occurs in the setting of myocardial injury and normal serum calcium. We present a case of a neonate with prominent dystrophic calcification and severe left ventricular systolic dysfunction in the setting of enterovirus myocarditis. These findings are superbly illustrated by multiple imaging modalities. The patient was treated with the novel antiviral, pocapavir, in addition to a standard heart failure regimen. The dystrophic calcification persisted but the left ventricle remodeled significantly. To our knowledge, this is the first reported use of pocapavir for this indication. The literature regarding enterovirus myocarditis and pocapavir is briefly reviewed.

Strategies for thromboprophylaxis in Fontan circulation: a meta-analysis.

BACKGROUND: The Fontan circulation is associated with an increased risk of thromboembolic events (TEs). As many as 25% of these thrombotic events result in fatality. More subtle adverse effects on the pulmonary circulation from embolic thrombi may further impair adequate functioning of the circuit. Despite these well-documented phenomena, the most optimal approaches to thromboprophylaxis are still not clearly defined. OBJECTIVE: A meta-analysis of published trials in English on PubMed and Cochrane libraries that evaluated the role of using TE prophylaxis in patients who underwent the Fontan procedure was conducted. METHODS: 10 studies with a total number of 1200 patients with an average follow-up time of 7.1 years were identified. A random effect model was used. RESULTS: The incidence of TE was significantly less in patients who received TE prophylaxis (using either aspirin or warfarin) compared with patients who did not receive TE prophylaxis (OR 0.425, 95% CI 0.194 to 0.929, p<0.01, I(2)=37%). The incidence of TE was significantly lower in patients who received aspirin compared with no TE prophylaxis (OR 0.363, 95% CI 0.177 to 0.744, p<0.01, I(2)=0%) and who received warfarin compared with no TE prophylaxis (OR 0.327, 95% CI 0.168 to 0.634, p<0.01, I(2)=2.5%). There was no significant difference in incidence of TE between warfarin and aspirin (OR 0.936, 95% CI 0.609 to 1.438, p=0.54, I(2)=0%). Furthermore, there was no significant difference in incidence of early TE (within 6 months of the operation) or late TE (>6 months) between patients receiving warfarin and aspirin (OR 0.784, 95% CI 0.310 to 1.982, p=0.37, I(2)=8%) and (OR 0.776, 95% CI 0.249 to 2.42, p=0.3, I(2)=45%), respectively. When only total cavopulmonary connection patients were included, there was again no difference between warfarin and aspirin in the incidence of TE (OR 0.813, 95% CI 0.471 to 1.401, p=0.34, I(2)=11%). CONCLUSIONS: This study shows a significantly lower incidence of TE after Fontan procedure if either aspirin or warfarin is used. This meta-analysis suggests no significant difference in incidence of early or late TE in patients receiving aspirin compared with warfarin.