Pulsatile bidirectional Glenn is associated with better pulmonary artery growth, which might improve long-term outcomes after Fontan. However, it was associated with a higher postoperative complication rate.

The 7q11.23 microduplication syndrome, caused by the reciprocal duplication of the WilliamsBeuren syndrome (WBS) deletion region, is a genomic disorder with an emerging clinical phenotype. Dysmorphic features, congenital anomalies, hypotonia, developmental delay highlighted by variable speech delay, and autistic features are characteristic findings. Congenital heart defects, most commonly patent ductus arteriosus, have been reported in a minority of cases.Included in the duplicated region is elastin (ELN), implicated as the cause of supravalvar aortic stenosis in WBS. Here we present a series of eight pediatric patients and one adult with 7q11.23 microduplication syndrome, all of whom have aortic dilation, the opposite vascular phenotype of the typical supravalvar aortic stenosis found in WBS. The ascending aorta was most commonly involved, while dilation was less frequently identified at the aortic root and sinotubular junction.The findings in these patients support a recommendation for cardiovascular surveillance in patients with 7q11.23 microduplication syndrome.

Unstructured abstract Early extubation is increasingly common in congenital heart surgery, but there are limited outcomes data across the spectrum of case complexity. We performed a retrospective review of 201 pediatric operations using cardiopulmonary bypass between 2012 and 2014. Patients extubated in the operating room or immediately on arrival to the ICU were compared to those extubated by traditional protocols. In-hospital mortality, major complications, need for re-intubation, hospital length of stay, and chest-tube days were compared between groups and by Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery Congenital Heart Surgery (STAT) mortality category. Outcome measures of hospital length of stay and chest tube days were analyzed using multivariable regression modeling. Early extubation subjects were older, weighed more, had shorter bypass and aortic cross-clamp time, more often received caudal anesthesia, and had shorter hospital length of stay and fewer chest tube days. Subjects not extubated early had more chromosomal abnormalities, more preoperative co-morbidities, and had more major complications. Inhospital death, major complications, and re-intubation were rare outcomes for both groups across all STAT categories. Multivariable regression analysis showed that cardiopulmonary bypass time was a significant predictor of hospital length of stay and chest tube days. Hospital LOS and chest tube days were significantly lower for the early extubation group in both the unadjusted and adjusted analyses. Early extubation can be performed safely in congenital heart surgery across a spectrum of case complexity. No increased early mortality or re-intubation was observed with early extubation although there were important differences between the groups that merits further study. The potential benefits of early extubation include decreased hospital length of stay and fewer chest tube days, particularly in young children and patients with long bypass times.

The COVID-19 pandemic disproportionately affects females in the home and workplace. This study aimed to acquire information regarding the gender-specific effects of the COVID-19 lockdown on aspects of professional and personal lives of a subset of pediatric cardiologists. We sent an online multiple-choice survey to a listserv of Pediatric Cardiologists. Data collected included demographics, dependent care details, work hours, leave from work, salary cut, childcare hours before and after the COVID-19 peak lockdown/ stay at home mandate and partner involvement. Two hundred forty-two pediatric cardiologists with dependent care responsibilities responded (response rate of 20.2%). A significantly higher proportion of females reported a salary cut (29.1% of females vs 17.6% of males, p = 0.04) and scaled back or discontinued work (14% vs 5.3%; p = 0.03). Prior to the COVID-19 lockdown phase, females provided more hours of dependent care. Females also reported a significantly greater increase in childcare hours overall per week (45 hours post/30 hours pre vs 30 hours post/20 hours pre for men; p < 0.001). Male cardiologists were much more likely to have partners who reduced work hours (67% vs 28%; p < 0.001) and reported that their partners took a salary cut compared with partners of female cardiologists (51% vs 22%; p < 0.001). In conclusion, gender disparity in caregiver responsibilities existed among highly skilled pediatric cardiologists even before the COVID-19 pandemic. The pandemic has disproportionately affected female pediatric cardiologists with respect to dependent care responsibilities, time at work, and financial compensation.

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