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Background: Congenital heart disease (CHD) is a public health concern as the most common birth defect with growing prevalence in adults. Valid CHD data are critical in clinical epidemiological CHD research. Objectives: To evaluate the positive predictive value (PPV) of having a CHD diagnosis and related surgery registered in the population-based Danish National Patient Registry (DNPR) according to the International Classification of Diseases, 8th and 10th revision, using predefined exclusion criteria to increase diagnostic validity. Methods: Using medical record review as reference standard, we computed the PPV of registered diagnostic codes for CHD and CHD subtypes including 95% confidence intervals (CIs) as well as the PPV of presence of CHD-related surgery. A sample of 480 patients diagnosed with CHD at any hospital in the Central Jutland region, during 1977–2013, was randomly selected for the medical record review. Reviews were performed by two paediatric cardiology nurses, blinded to the specific CHD diagnosis, and a paediatric cardiologist was consulted in cases of uncertainty. Results: We located medical records of 463 of the 480 patients included (96%), and found an overall PPV of the CHD diagnosis of 95.5 (95%CI: 93.3–97.1). Subtype specific PPVs were generally lower. In example PPV for atrial septal defect was 69%. It increased to 78% after inclusion of persistent foramen ovale in the reference standard subtype definition. The overall PPV of CHD-related surgery codes was 92.7% Conclusions: The overall PPV of CHD diagnoses in the DNPR is high. Data indicated lower PPVs for CHD subtypes.

Background: Statin use for hypercholesterolemia in children is predominantly reported from short-term clinical trials. In this study, we assess the efficacy and safety of statin treatment in clinical pediatric practice. Methods: Records of all patients who began statin treatment at age 6 months from 5 pediatric lipid clinics were reviewed. Information at baseline and from all clinic evaluations after statin initiation was recorded, including lipid measurements, statin drug/dose, safety measures (anthropometry, hepatic enzymes, creatine kinase levels), and symptoms. Lipid changes on statin therapy were assessed from baseline to 6 ± 3 months and from 6 ± 3 months to last follow-up with a mixed-effects model, using piecewise linear splines to describe temporal changes, controlling for repeated measures, sex, and age. Results: There were 289 patients with median low-density lipoprotein cholesterol (LDL-C) of 5.3 mmol/L (interquartile range [IQR]:4.5–6.5) and mean age of 12.4 ± 2.9 years at statin initiation. Median duration of therapy was 2.7 years (IQR: 1.6–4.5) with 95% on statins at last evaluation. There were significant decreases in total cholesterol, LDL-C, and non–high-density lipoprotein cholesterol (non–HDL-C) from baseline to 6 ± 3 months (P < 0.001) and from 6 ±3 months to last follow-up (P < 0.001). Triglycerides and HDL-C were unchanged but the triglyceride to HDL-C ratio decreased significantly by late follow-up. At final evaluation, median LDL-C had decreased to 3.4 mmol/L (IQR:2.8–4.2). No patient had statins discontinued for safety measures or symptoms. Conclusions: In real-world clinical practice, intermediate-term statin treatment is effective and safe in children and adolescents with severe LDL-C elevation. Résumé: Contexte: Les statines sont fréquemment employées pour traiter l’hypercholestérolémie chez les enfants dans le cadre d’essais cliniques de courte durée. Dans l’étude présentée ici, nous évaluons l’efficacité et l’innocuité de l’emploi de statines dans la pratique clinique en pédiatrie. Méthodologie: Nous avons passé en revue les dossiers de tous les patients de cinq cliniques pédiatriques des lipides qui ont commencé à prendre une statine avant l’âge de 18 ans et qui ont poursuivi le traitement pendant plus de six mois. Les valeurs mesurées au départ et à chacune des évaluations cliniques après l’instauration d’un traitement par une statine ont été consignées, notamment la lipidémie, le type et la dose de la statine prescrite, les paramètres d’évaluation de l’innocuité (anthropométrie, enzymes hépatiques, taux de créatine kinase) et les symptômes. La variation de la lipidémie chez les patients recevant une statine a été évaluée sur deux périodes, soit entre le début du traitement et l’évaluation effectuée à 6 ± 3 mois ainsi qu’entre l’évaluation effectuée à 6 ± 3 mois et la dernière évaluation de suivi, à l’aide d’un modèle à effets mixtes et de splines linéaires par morceaux pour décrire les changements temporels, en contrôlant pour les mesures répétées, le sexe et l’âge. Résultats: L’étude portait sur 289 patients ayant un taux de cholestérol des lipoprotéines de basse densité (C-LDL) médian de 5,3 mmol/l (intervalle interquartile [IIQ] : 4,5 à 6,5) et âgés de 12,4 ± 2,9 ans en moyenne au moment de l’instauration du traitement par une statine. La durée médiane du traitement était de 2,7 ans (IIQ : 1,6 à 4,5), 95 % des sujets étant toujours sous statine à la dernière évaluation. Les taux de cholestérol total, de C-LDL et de cholestérol des lipoprotéines non de haute densité (C-non-HDL) avaient diminué de manière significative entre le début du traitement et l’évaluation à 6 ± 3 mois (p < 0,001) et entre l’évaluation à 6 ± 3 mois et la dernière évaluation de suivi (p < 0,001). Les taux des triglycérides et du C-HDL n’avaient pas bougé, mais le rapport triglycérides/C-HDL avait diminué considérablement vers la fin du suivi. À l’évaluation finale, le taux de C-LDL avait diminué à 3,4 mmol/l (IIQ : 2,8 à 4,2). Aucun patient n’avait abandonné le traitement par une statine en raison de problèmes d’innocuité ou des symptômes. Conclusions: En situation réelle dans la pratique clinique, le traitement à moyen terme par une statine est efficace et sûr chez les enfants et les adolescents présentant une élévation grave du taux de C-LDL.

BackgroundNo previous study has examined the effect of maternal hypothyroidism on a broad spectrum of cardiovascular disease (CVD) endpoints in the offspring.MethodsA nationwide population-based cohort study based on the linkage of several Danish nationwide registries was conducted to explore whether maternal hypothyroidism is associated with offspring’s CVD. Altogether 1,041,448 singletons born between the 1st of January 1978 and the 31st of December 1998 were investigated from the age of 8 years to the 31st of December 2016. Exposure was maternal diagnosis of hypothyroidism across lifespan and the outcome of interest was a CVD diagnosis in the offspring. Cox regression models were performed to estimate the hazard ratios (HRs) of CVD.ResultsOffspring born to mothers with hypothyroidism had an increased risk of CVD (hazard ratios (HR)=1.23, 95% confidence interval (CI): 1.12-1.35), and of several subcategories of CVD including hypertension, arrhythmia, and acute myocardial infarction in offspring. The magnitude of association was the most pronounced in an exposure occur during pregnancy (HR=1.71, 95% CI: 1.10-2.67), which is consistent across all the subgroup analysis, including sibling analysis.ConclusionsMaternal hypothyroidism is associated with an increased risk of CVD in offspring. Thyroid hormone insufficiency during pregnancy may predominantly contribute to the observed associations; however, the effects of a shared genetic background and a time-stable familial environment/lifestyle factors cannot be excluded.

There are multiple cardiac etiologies for wheezing and respiratory distress which require a high degree of suspicion for the pediatrician to diagnose. We present a case of a patient with a history of long-standing mild persistent asthma with minimal improvement on controller and bronchodilator therapies who presented to the emergency room with acute respiratory distress. When he demonstrated a lack of improvement with traditional respiratory therapies, additional etiologies of respiratory distress were considered. Ultimately an echocardiogram was performed, which revealed the diagnosis of cor triatriatum. He underwent surgical resection of his accessory membrane and has had no additional symptoms of asthma since repair.

Aims/hypothesis: Heart failure is a complication of type 2 diabetes (T2DM). Echocardiography can identify subclinical systolic dysfunction in adults with T2DM. We hypothesized that reduced systolic strain was present in youth with T2DM. Methods: Global longitudinal strain (GLS) was measured in 151 subjects with T2DM matched to lean (L = 146), and obese (O = 162) subjects (23.0 ± 4.0 years, 35% male, 63% African American). Anthropometrics, BP, HR, labs, and echocardiograms were obtained. ANOVA was performed to compare differences among groups, and ANCOVA to determine if T2DM remained an independent predictor after corrections. Results: BP, lipid levels, and metabolic control worsened and GLS was reduced from L to O to T2DM. BMI was lower in L than O or T2DM. Global longitudinal strain rate (GLSR) was lower and LVM/ht2.7 was higher in O and T2DM as compared to L (all P ≤.05). Presence of T2DM was an independent determinant of GLS and GLSR adjusted for most CV risk factors, but lost significance when BMI was added to the model. GLS = −21.6‐age*0.088 ‐ male*1.8 + 0.12*BMI + 0.045*DBP + 0.058*HR ‐ 0.023*HDL (R2 = 0.38, P ≤.0001); GLSR = −1.20‐male*0.093 + WHR*0.48 + DBP*0.0029 (R2 = 0.23, P ≤.0001). Conclusion: Both adiposity and T2DM have a deleterious effect on systolic cardiac function. Treatment of obesity in youth is necessary for prevention of future heart failure. [ABSTRACT FROM AUTHOR]