Background-Preoperative brain injury is an increasingly recognized phenomenon in neonates with complex congenital heart disease. Recently, reports have been published that associate preoperative brain injury in neonates with transposition of the great arteries with the performance of balloon atrial septostomy (BAS), a procedure that improves systemic oxygenation preoperatively. It is unclear whether BAS is the cause of brain injury or is a confounder, because neonates who require BAS are typically more hypoxemic. We sought to determine the relationship between preoperative brain injury in neonates with transposition of the great arteries and the performance of BAS. We hypothesized that brain injury results from hypoxic injury, not from the BAS itself. Methods and Results-Infants with transposition of the great arteries (nϭ26) were retrospectively included from a larger cohort of infants with congenital heart disease who underwent preoperative brain MRI as part of 2 separate prospective studies. Data collected included all preoperative pulse oximetry recordings, all values from preoperative arterial blood gas measurements, and BAS procedure data. MRI scans were performed on the day of surgery, before the surgical repair.Of the 26 neonates, 14 underwent BAS. No stroke was seen in the entire cohort, whereas 10 (38%) of 26 patients were found to have hypoxic brain injury in the form of periventricular leukomalacia. Periventricular leukomalacia was not associated with BAS; however, neonates with periventricular leukomalacia had lower preoperative oxygenation (Pϭ0.026) and a longer time to surgery (Pϭ0.028) than those without periventricular leukomalacia. Conclusions-Preoperative brain injury in neonates with transposition of the great arteries is associated with hypoxemia and longer time to surgery. We found no association between BAS and brain injury.

In this multicenter comparison of palliative PDA stent and BT shunt for infants with ductal-dependent pulmonary blood flow adjusted for differences in patient factors, there was no difference in the primary end point, death or unplanned reintervention to treat cyanosis. However, other markers of morbidity and pulmonary artery size favored the PDA stent group, supporting PDA stent as a reasonable alternative to BT shunt in select patients.

The Single Ventricle Program improved interstage weight gain, thereby allowing for early second-stage palliation at an equivalent patient weight. Interstage mortality was not significantly reduced by our program. However, 1-year transplant-free survival was significantly improved in patients in the Single Ventricle Program.

Objectives To devise a classification scheme for ductal morphology in patients with ductal dependent pulmonary blood flow (PBF) that can be used to assess outcomes. Background The impact of ductal morphology on outcomes following patent ductus arteriosus (PDA) stenting is not well defined. Methods Patients <1 year of age who underwent PDA stenting for ductal dependent PBF at the four centers comprising the Congenital Catheterization Research Collaborative (CCRC) were included. A classification scheme for PDA morphology was devised based on a tortuosity index (TI)—Type I (straight), Type II (one turn), and Type III (multiple turns). A subtype classification was used based upon the ductal origin. Results One hundred and five patients underwent PDA stenting. TI was Type I in 58, Type II in 24, and Type III in 23 PDAs, respectively. There was a significant association between ductal origin and vascular access site (p < 0.001). Procedure times and need for >1 stent did not differ based on TI. Greater TI was associated with pulmonary artery (PA) jailing (p = 0.003). Twelve (11.4%) patients underwent unplanned reintervention, more commonly with greater TI (p = 0.022) and PA jailing (p < 0.001). At the time of subsequent surgical repair/palliative staging, PA arterioplasty was performed in 32 patients, more commonly when a PA was jailed (p = 0.048). PA jailing did not affect PA size at follow up. Conclusions The proposed qualitative and quantitative PDA morphology classification scheme may be helpful in anticipating outcomes in patients with ductal dependent PBF undergoing PDA stenting.

Conclusions-PCA is safe even in small infants, and hemostasis can be achieved without surgical repair, with a carotid patency rate superior to published data after surgical cutdown. Surgical cutdown is not routinely required for pediatric cardiac catheterization via the carotid artery. (Circ Cardiovasc Interv. 2016;9:e003003.

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Background: The prevalence of obesity in long-term survivors with complex congenital heart disease may be increasing, and little is known about the timing and onset of weight gain and growth patterns in these high-risk patients. Methods and Results: Prevalence rates of overweight/obesity and longitudinal changes in body mass index (BMI) with age were determined in 606 patients with Fontan circulation seen at a tertiary care cardiology center from 1992 – 2012. The number of clinic encounters (n) was stratified by age group (n=401, 2–5y; n= 333, 6–11y; n=217, 12–19y; and n=129, >20y). Among adults, 39% were overweight/obese at last clinic visit; 22% overweight and 17% obese. Childhood anthropometric data were available for 82 adults; of which 15% (n=12/82) were overweight/obese in childhood. The likelihood of being overweight/ obese as an adult was 3 times higher if there was a BMI ≥ 85th percentile in childhood (CI 2.1–4.5, p <0.01). Overweight/obesity in adulthood was associated with lower heart failure rates (4% vs. 19%, P=0.03). Pediatric rates of overweight/obesity were comparable to national data (NHANES 2011–2012) in every age group: at 2–5y, (25 vs. 23%), 6–11y (26 vs. 34%), and 12–19y (15 vs. 35%). Systolic blood pressure was higher in overweight/obese children as young as 2–5 years of age. Conclusions: Childhood and adult survivors with Fontan circulation have high rates of overweight/obesity. Childhood obesity is a strong predictor of future adiposity and is linked to changes in systolic blood pressure at a very young age.