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Abstract Cardiovascular magnetic resonance (CMR) has been utilized in the management and care of pediatric patients for nearly 40 years. It has evolved to become an invaluable tool in the assessment of the littlest of hearts for diagnosis, pre-interventional management and follow-up care. Although mentioned in a number of consensus and guidelines documents, an up-to-date, large, stand-alone guidance work for the use of CMR in pediatric congenital 36 and acquired 35 heart disease endorsed by numerous Societies involved in the care of these children is lacking. This guidelines document outlines the use of CMR in this patient population for a significant number of heart lesions in this age group and although admittedly, is not an exhaustive treatment, it does deal with an expansive list of many common clinical issues encountered in daily practice.

Background: Adults with congenital heart disease (CHD) may be at increased risk of acquired cardiovascular disease (CVD). Understanding the prevalence of CV risk factors (CVRF) in this population is an important step in developing strategies to mitigate long-term risk. Methods: The Oregon All Payer All Claims database for the years 2010–2017 was queried for adults with CHD International Classification of Diseases (ICD) codes. The prevalence of CVRF was measured, and we then evaluated the association with patient characteristics. Results: There were 13,896 individuals with CHD. 72.8% (99% CI: 71.8–73.7) had at least one RF and 52.3% (99% CI: 51.2–53.4) had ≥2 RF. The prevalence of ≥1 RF increased with age (18–24: 39.6% (99% CI: 37.0–42.1) vs. 93.6% (99% CI: 92.6–94.6) in those 55–65). Hypertension (aOR 1.49 (99% CI: 1.36–1.63)), diabetes (aOR 1.24 (99% CI: 1.13–1.36)), sleep apnea (aOR 1.40 (99% CI 1.26–1.55)) and kidney disease (aOR 1.33 (99% CI:1.14–1.54)) were more prevalent in moderate-complex as opposed to simple CHD. When compared with a matched non-CHD population, there was higher prevalence of CVRF in ACHD (≥1 RF: 76.1 vs. 64.1%, OR 1.79 (99% CI: 1.69–1.89); ≥2 RF: 52.6 vs. 36.5%, OR 1.92 (99% CI: 1.83–2.03). Conclusions: To our knowledge, this is the first comprehensive attempt to measure both traditional and non-traditional CVRF in US adults with CHD. We show that CVRF are common even in young adults. Given the additive effect of acquired CVD on CHD, addressing RF should be an important priority for in ACHD.

Background: Adverse childhood experiences (ACEs) are associated with poorer health outcomes, including a higher risk of acquired cardiovascular disease. This was the first investigation of ACEs in adults with congenital heart disease (CHD). Methods: Adults with CHD completed the following surveys in the outpatient setting: ACE questionnaire, measure of attachment style, and linear analogue measure of quality of life. We performed Chi-square tests and t-tests to explore relationships between ACE scores with other surveys as well as demographic and clinical characteristics. Results: A total of 100 patients participated in the study (age 40 ​± ​13 years; 60% female); 90% had CHD of moderate or great complexity. The total number of reported ACEs ranged from 0 to 9 out of a possible total of 10, with a mean of 2.6 ​± ​2.5.30% of participants reported ≥ 4 ACEs, which is higher than the reported prevalence in the general population. The most frequently reported ACEs were parental divorce (46%), emotional abuse (42%), and parental substance abuse (32%). ACE scores did not differ as a function of age, sex or CHD defect complexity. Total ACE scores were significantly associated with two attachment styles (preoccupied: p ​

Background In Fontan patients with atrial arrhythmias (AA), non-vitamin K antagonist oral anticoagulants(NOACs) have a class III recommendation according to the Pediatric & Congenital Electrophysiology Society (PACES)/Heart Rhythm Society (HRS) guideline in 2014, due to lack of data on outcomes as opposed to evidence of harm. To address this gap in data, we investigated the safety and efficacy of NOACs in adults with a Fontan circulation in a worldwide study.Methods This is an international multicentre prospective cohort study, using data from the NOTE (non-vitamin K antagonist oral anticoagulants for thromboembolic prevention in patients with congenital heart disease) registry. The study population comprised consecutive adults with a Fontan circulation using NOACs. Follow-up took place at 6 months and yearly thereafter. The primary endpoints were thromboembolism and major bleeding. Secondary endpoint was minor bleeding.Results From April 2014 onward, 74 patients (mean age 32±10 years (range 18–68), 54% male) with a Fontan circulation using NOACs were included. During a median follow-up of 1.2 (IQR 0.8–2.0) years, three thromboembolic events (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) and three major bleedings (2.9 per 100 patient-years (95% CI 0.7 to 7.6)) occurred in five atriopulmonary Fontan and one total cavopulmonary connection Fontan patients with AA. Fifteen patients experienced minor bleeding episodes (15.8 per 100 patient-years (95% CI 9.1 to 25.2)). In patients (n=37) using vitamin K antagonists (VKAs) prior to the initiation of NOAC, annual incidence of historical thromboembolic events and major bleeding were 2.4% (95% CI 0.4% to 7.4%) (n = 2) and 1.2% (95% CI 0.7% to 5.1%) (n = 1), respectively.Conclusions In this review of the largest Fontan cohort using NOACs with prospective follow-up, NOACs appear to be well tolerated and their efficacy and safety during short-term follow-up seem comparable to VKAs. Longer term data are required to confirm these promising short-term results.