Longitudinal Assessment of Global and Regional Left Ventricular Strain in Patients with Multisystem Inflammatory Syndrome in Children (MIS-C).

Multisystem inflammatory syndrome in children (MIS-C) is one of the most significant sequela of coronavirus disease 2019 (COVID-19) 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.

Utility of routine screening fetal echocardiogram in pregnancies conceived by in vitro fertilization.

OBJECTIVE: To study the incidence and clinical significance of congenital heart defects (CHDs) detected by fetal echocardiography in pregnancies conceived by in vitro fertilization (IVF). DESIGN: Cohort study comparing a prospectively maintained database of all fetal echocardiograms from 2012 to 2018 and pooled data from the Connecticut Birth Defects Registry and statewide hospital discharge data. SETTING: Large tertiary care center. PATIENT(S): A total of 181,749 live births and 9,252 fetal echocardiograms were analyzed. Fetal echocardiograms in patients with a previous child with a CHD, a family history of CHD, medication exposure, diabetes, anomaly in previous pregnancy, cardiac or other abnormality noted on previous ultrasound, or monochorionic twins were excluded from the final analysis. INTERVENTION(S): Treatment with IVF. MAIN OUTCOME MEASURE(S): Incidence of CHD and odds ratios with 95% confidence intervals (CIs). Infant outcomes for cases of CHD were evaluated for clinically significant disease, defined a priori as disease requiring any medical or surgical intervention or continued follow-up with pediatric cardiology. RESULT(S): Fetal echocardiography was performed in 2,230 IVF pregnancies, of which 2,040 were without other known risk factors for CHD. The mean gestational age at the time of fetal echocardiography was 22.2 ± 1.4 weeks. The odds ratio for CHD in the IVF group compared with statewide population rates was 1.4 (95% CI 0.9-2.1). CHD was diagnosed in 26 fetuses, of which 21 were clinically insignificant ventricular septal defects. One fetal echocardiogram was concerning for pulmonary stenosis that was not present at birth. Four defects were clinically significant, indicating that 510 fetal echocardiograms were performed for every diagnosis of one clinically significant CHD in the IVF group. CONCLUSION(S): The incidence of CHD in IVF pregnancies without other risk factors is not significantly different from baseline population rates, and most CHDs diagnosed by fetal echocardiography in this group are clinically insignificant. Routine screening with fetal echocardiography in all IVF pregnancies provides limited utility beyond routine prenatal care and need not be recommended without the presence of other risk factors.

Fetal Echocardiographic Measures to Improve the Prenatal Diagnosis of Coarctation of the Aorta.

The objective of this study is to identify fetal echocardiographic measures that predict postnatal coarctation of the aorta (CoA). A retrospective review of patients from 2013 to 2017 identified 13 cases of prenatal diagnosis of CoA confirmed postnatally and 14 cases of prenatal diagnosis of CoA with normal arches postnatally. There were 30 controls. Measurements were made and indices applied on all available longitudinal fetal echocardiograms for each patient. Linear mixed effects models were used to examine the between-group differences in the trajectories of the measurements. Significant differences were seen in the true CoA group for the following: smaller distal transverse arch diameter to distance between the left common carotid and left subclavian arteries (DT/LCA-LSCA) index (p = 0.04), smaller distal transverse arch diameter (p = 0.005), and longer brachiocephalic to left common carotid artery (LCA) (p = 0.004) and LCA-left subclavian artery (LSCA) distances (p < 0.0001). Additionally, the LCA/DT index trend appears to differentiate false positives from true coarctations (p < 0.03). The fetal echocardiographic DT/LCA-LSCA index, brachiocephalic-LCA distance and LCA-LSCA distance are significant predictors of postnatal coarctation. The LCA/DT index trend over time may differentiate which of those patients with prenatal concern for coarctation are more likely to develop coarctation postnatally. The use of fetal echocardiographic measures may improve prenatal detection and predication of postnatal coarctation.

A Smartphone Application to Diagnose the Mechanism of Pediatric Supraventricular Tachycardia.

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.

Subcutaneous treprostinil for pulmonary hypertension in chronic lung disease of infancy.

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 longer-acting 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.

Common atrium and pulmonary vascular disease.

This report describes a case series of six patients with congenital common atrium who developed pulmonary vascular disease. Three developed early pulmonary vascular disease within their first year of life, while the others became symptomatic at ages 2, 5, and 17. Four of the six underwent surgical repair, and five of the six patients are being treated on targeted pulmonary hypertension therapy. Based on our observations, the clinical course of children with common atrium may differ from patients with a large atrial septal defect. Early monitoring and surgical correction, if necessary, may prevent the onset of severe pulmonary vascular disease. 

Defining, navigating, and negotiating success: the experiences of mid-career Robert Wood Johnson Clinical Scholar women.

BACKGROUND: We studied female graduates of the Robert Wood Johnson Clinical Scholars Program (CSP, Class of 1984 to 1989) to explore and describe the complexity of creating balance in the life of mid-career academic woman physicians. METHODS: We conducted and qualitatively analyzed (kappa 0.35 to 1.0 for theme identification among rater pairs) data from a semi-structured survey of 21 women and obtained their curricula vitae to quantify publications and grant support, measures of academic productivity. RESULTS: Sixteen of 21 (76%) women completed the survey. Mean age was 48 (range: 45 to 56). Three were full professors, 10 were associate professors, and 3 had left academic medicine. Eleven women had had children (mean 2.4; range: 1 to 3) and 3 worked part-time. From these data, the conceptual model expands on 3 key themes: (1) defining, navigating, and negotiating success, (2) making life work, and (3) making work work. The women who described themselves as satisfied with their careers (10/16) had clarity of values and goals and a sense of control over their time. Those less satisfied with their careers (6/16) emphasized the personal and professional costs of the struggle to balance their lives and described explicit institutional barriers to fulfillment of their potential. CONCLUSION: For this group of fellowship-prepared academic women physicians satisfaction is achieving professional and personal balance.