congenital heart disease (cHD) survivors are at risk for neurodevelopmental disability (nDD), and recent studies identify genes associated with both disorders, suggesting that nDD in cHD survivors may be of genetic origin. Genes contributing to neurogenesis, dendritic development and synaptogenesis organize neural elements into networks known as the connectome. We hypothesized that nDD in cHD may be attributable to genes altering both neural connectivity and cardiac patterning. to assess the contribution of de novo variants (DNVs) in connectome genes, we annotated 229 published NDD genes for connectome status and analyzed data from 3,684 CHD subjects and 1,789 controls for connectome gene mutations. cHD cases had more protein truncating and deleterious missense DnVs among connectome genes compared to controls (oR = 5.08, 95%CI:2.81-9.20, Fisher's exact test P = 6.30E-11). When removing three known syndromic CHD genes, the findings remained significant (OR = 3.69, 95%CI:2.02-6.73, Fisher's exact test P = 1.06E-06). In CHD subjects, the top 12 NDD genes with damaging DNVs that met statistical significance after Bonferroni correction (PTPN11,

Smartphone applications that record a single-lead ECG are increasingly available. We sought to determine the utility of a smartphone application (AliveCor) to record supraventricular tachycardia (SVT) and to distinguish atrioventricular reentrant tachycardia (AVRT) from atrioventricular nodal reentrant tachycardia (AVNRT) in pediatric patients. A prior study demonstrated that interpretation of standard event and Holter monitors accurately identifies the tachycardia mechanism in only 45 % of recordings. We performed an IRB-approved prospective study in pediatric patients undergoing an ablation for SVT. Tracings were obtained by placing the smartphone in three different positions on the chest (PI-horizontal, PII-rotated 60° clockwise, and PIII-rotated 120° clockwise). Two blinded pediatric electrophysiologists jointly analyzed a pair of sinus and tachycardia tracings in each position. Tracings with visible retrograde P waves were classified as AVRT. The three positions were compared by Chi-square test. Thirty-seven patients (age 13.7 ± 2.8 years) were enrolled in the study. Twenty-four had AVRT, and 13 had AVNRT. One hundred and eight pairs of tracings were obtained. The correct diagnosis was made in 27/37 (73 %) with position PI, 28/37 (76 %) with PII, and 20/34 (59 %) with PIII (p = 0.04 for PII vs. PIII and p = NS for other comparisons). A single-lead ECG obtained with a smartphone monitor can successfully record SVT in pediatric patients and can predict the SVT mechanism at least as well as previously published reports of Holter monitors, along with the added convenience of not requiring patients to carry a dedicated monitor.

Pulmonary arterial hypertension (PAH) associated with chronic lung disease of infancy can be a life-threatening disease affecting an increasing number of former premature infants. There is a need for improved delivery of targeted PAH therapies for this subgroup of patients who have severe and persistent PAH despite standard respiratory care for chronic lung disease. Currently infants who have severe PAH despite oral or inhaled therapy receive continuous intravenous prostanoid therapy (mostly epoprostenol), which is complicated because of the need for central venous access and associated catheter-related complications. We present a series of 5 infants who were successfully treated with a continuous infusion of subcutaneous treprostinil, which is a longeracting prostanoid with similar hemodynamic effects. There were improvements in echocardiographic assessment of right ventricular function and estimated pulmonary hypertension, and in respiratory support required within weeks of therapy. Unlike commonly in adults, these 5 infants had no instances of severe site erythema, bleeding, bruising, or infection. In our experience with 5 former extremely preterm infants who had PAH associated with chronic lung disease, subcutaneous treprostinil was safe, efficacious, and well tolerated. We believe that subcutaneous treprostinil can be beneficial in a select group of former premature infants who have chronic lung disease and severe pulmonary arterial hypertension who have not responded adequately to conservative therapies. Pediatrics 2014;134:e274-e278 AUTHORS:

Multisystem inflammatory syndrome in children (MIS-C) is one of the most significant sequela of coronavirus disease 2019 in children. Emerging literature has described myocardial dysfunction in MIS-C patients using traditional and two-dimensional speckle tracking echocardiography in the acute phase. However, data regarding persistence of subclinical myocardial injury after recovery is limited. We aimed to detect these changes with deformation imaging, hypothesizing that left ventricular global longitudinal (GLS) and circumferential strain (GCS) would remain impaired in the chronic phase despite normalization of ventricular function parameters assessed by two-dimensional echocardiography. A retrospective, single-institution review of 22 patients with MIS-C was performed. Fractional shortening, GLS, and GCS, along with regional longitudinal (RLS) and circumferential strain (RCS) were compared across the acute, subacute, and chronic timepoints (presentation, 14-42, and > 42 days, respectively). Mean GLS improved from − 18.4% in the acute phase to − 20.1% in the chronic phase (p = 0.4). Mean GCS improved from − 19.4% in the acute phase to − 23.5% in the chronic phase (p = 0.03). RCS and RLS were impaired in the acute phase and showed a trend towards recovery by the chronic phase, with the exception of the basal anterolateral segment. In our longitudinal study of MIS-C patients, GLS and GCS were lower in the acute phase, corroborating with left ventricular dysfunction by traditional measures. Additionally, as function globally recovers, GLS and GCS also normalize. However, some regional segments continue to have decreased strain values which may be an important subclinical marker for future adverse events. KeywordsCoronavirus disease 2019 (COVID-19) • Multisystem inflammatory syndrome in children (MIS-C) • Twodimensional speckle tracking echocardiography (2D-STE) • Longitudinal strain • Circumferential strain Abbreviations MIS-C Multisystem inflammatory syndrome in children COVID-19 Coronavirus disease 2019 2D-STE Two-dimensional speckle tracking echocardiography GLS Global longitudinal strain GCS Global circumferential strain RLS Regional longitudinal strain RCS Regional circumferential strain FS Fractional shortening KD Kawasaki disease PICU Pediatric intensive care unit * Michael He

For this group of fellowship-prepared academic women physicians satisfaction is achieving professional and personal balance.