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The study goal was to investigate electrocardiographic findings, including corrected QT interval (QTc), in patients aged 8 to 23 with eating disorders (EDs) at presentation, compared with an age-and sex-matched control population. We retrospectively reviewed 200 ED patients, and 200 controls. Blinded electrocardiograms (ECGs) were interpreted by an expert reader, and QT intervals corrected using the Bazett formula. Eating disorder patients were 89.5% female, with mean age 16.4 years and median percent median body mass index (BMI)-for-age (%mBMI)a of 91.1%. In ED patients, QTc was significantly shorter than controls (399.6 vs 415.0msec, P < .001). After adjusting for height, %mBMI, sex, magnesium level, and bradycardia, mean QTc duration in patients with anorexia nervosa-restricting subtype (AN-R) was significantly shorter than other ED patients ( P = .010). Higher %mBMI was associated with shorter QTc duration ( P = .041) after adjusting for height, magnesium, bradycardia, and Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) diagnosis. Within the ED group, no significant association was identified between QTc and medications, electrolytes, or inpatient status.

Surveillance metrics in pediatric and young adult Marfan syndrome (pMFS) are challenging. We evaluated the utility of aortic root cross-sectional area/height index (CSA/Ht) on echocardiogram among pMFS patients as a risk stratification and surgical triage metric.Genotype or phenotype positive pMFS patients aged 25 years or younger seen at our center from 2001 to 2020 were identified. Time-related transition to surgery was modeled using parametric methods. Predictive utility of CSA/Ht compared with aortic root diameter (ARd) and root Z score (ARz) were modeled using nonlinear multivariable parametric and nonparametric longitudinal regression models.Seventy-nine patients (43% female) presented at median age of 5.8 years (15th-85th percentile, 0.75-17 years) with median follow-up of 4.4 years (range, 0-18.5 years). Baseline echocardiography data were: CSA/Ht, 3.9 ± 1.4 cmCSA/Ht tracked ARd well over time. CSA/Ht between 5 and 7 cm

Background: Late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) is believed to represent dense replacement fibrosis. It is seen in ≈60% of adult patients with hypertrophic cardiomyopathy (HCM). However, the prevalence of LGE in children and adolescents with HCM is not well established. In addition, longitudinal studies describing the development and evolution of LGE in pediatric HCM are lacking. This study assesses the prevalence, progression, and clinical correlations of LGE in children and adolescents with, or genetically predisposed to, HCM. Methods: CMR scans from 195 patients ≤21 years of age were analyzed in an observational, retrospective study, including 155 patients with overt HCM and 40 sarcomere mutation carriers without left ventricular (LV) hypertrophy. The extent of LGE was quantified by measuring regions with signal intensity >6 SD above nulled remote myocardium. Results: Patients were 14.3±4.5 years of age at baseline and 68% were male. LGE was present in 70 (46%) patients with overt HCM (median extent, 3.3%; interquartile range, 0.8–7.1%), but absent in mutation carriers without LV hypertrophy. Thirty-one patients had >1 CMR (median interval between studies, 2.4 years; interquartile range, 1.5–3.2 years). LGE was detected in 13 patients (42%) at baseline and in 16 patients (52%) at follow-up CMR. The median extent of LGE increased by 2.4 g/y (range, 0–13.2 g/y) from 2.9% (interquartile range, 0.8–3.2%) of LV mass to 4.3% (interquartile range, 2.9–6.8%) ( P =0.02). In addition to LGE, LV mass and left atrial volume, indexed to body surface area, and z score for LV mass, as well, increased significantly from first to most recent CMR. Conclusions: LGE was present in 46% of children and adolescents with overt HCM, in contrast to ≈60% typically reported in adult HCM. In the subset of patients with serial imaging, statistically significant increases in LGE, LV mass, and left atrial size were detected over 2.5 years, indicating disease progression over time. Further prospective studies are required to confirm these findings and to better understand the clinical implications of LGE in pediatric HCM.

Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is a rare congenital anomaly, one which requires a high index of suspicion in order to be diagnosed promptly and managed accurately. ALCAPA from the right branch pulmonary artery (RPA) is a small subset of ALCAPA in general, with only a few reported cases in the world literature. We report two cases of ALCAPA from the RPA: the first case, an infant, presented in severe cardiac failure requiring preoperative extracorporeal membrane oxygenation (ECMO) for stabilization; and the second, a neonate, presented with severe aortic coarctation (CoA). Both patients underwent successful complete surgical repair using a modified surgical technique, and are doing well in midterm follow-up. We describe our operative technique and review the literature of this rare congenital anomaly.