Pediatric Heart Network Trial of Losartan vs. Atenolol in Children and Young Adults with Marfan Syndrome: Impact on Prescription Practices.

The Pediatric Heart Network (PHN) trial showed similar efficacy of ß-blockers (BB) and angiotensin receptor blockers (ARB) for aortic root dilation in Marfan syndrome, but the impact on prescription practices is unknown. We hypothesized BB and ARB prescriptions would increase after the trial results were published (2014). Prescription data (2007-2016) were obtained from outpatient encounters (IBM Marketscan) for Marfan syndrome patients (6 months-25 years old). Excluding 2014 as a washout period, we analyzed two intervals: 2007-2013 and 2015-2016. Medication categories included BB, ARB, angiotensin converting enzyme inhibitors (ACEI), combination (BB/ARB and/or BB/ACEI), and no drug. Interrupted time-series analysis assessed immediate level change after publication and change in slope for the trend pre- and post-publication. Odds ratios (OR) and 95% confidence intervals from logistic regressions and generalized estimating equation methods accounted for correlation of prescriptions within patients. In 1499 patients (age 14.1 ± 6.1 years, 59% female) seen 2007-2013, BB trended lower [OR 0.91 (0.89, 0.93), p < 0.001] and ARB trended higher [OR 1.12 (1.07, 1.18), p < 0.001], while combination, ACEI, and no drug remained stable. This trend persisted, but was not significant, for BB [OR 0.54 (0.27, 1.08), p = 0.37] and ARB [OR 1.91 (0.55, 6.69), p = 0.31] in 2015-2016. Combination, ACEI, and no drug remained similar. In short term follow-up, changes in prescription practices following publication of the PHN trial were not statistically significant. This may be due to a change seen prior to publication with early adoption of ARBs that was maintained after confirmation of their effectiveness.

Child Neurology: Case Report of Lambl Excrescences in a Pediatric Patient With Multifocal Strokes.

Lambl's excrescences are fibrinous connective tissue strands found on predominantly left-sided cardiac valves. These valvular strands are typically benign, but have been implicated as a potential etiology of embolic strokes in adult patients. The significance of Lambl's excrescences in pediatric stroke cases is unclear and not previously reported in the literature. Here, we describe a 10-year-old boy who presented with acute onset right-sided hemiplegia, found to have multifocal embolic strokes of various ages. Extensive stroke workup was unrevealing, aside from the presence of small, filamentous strand-like densities associated with the mitral and aortic valves noted on a transesophageal echocardiogram consistent with Lambl's excrescences. In this case report, we review Lambl's excrescences and their significance in acute stroke, as well as management options for prevention of future ischemia in these patients.

Sixth-Hour Trancutaneous Bilirubin and Need for Phototherapy in DAT Positive Newborns.

OBJECTIVES: To characterize the outcomes of ABO incompatible direct antiglobulin test (DAT) positive newborns and determine the predictive ability of a sixth-hour transcutaneous bilirubin (TcB for needing phototherapy ≤24 hours of age. METHODS: Retrospective, cross-sectional study from May 2013 to March 2017. Of 10 942 consecutive newborns ≥35 weeks estimated gestational age, 829 were ABO incompatible and DAT positive. After excluding for antibodies other than ABO (51), missing data (4), miscategorization of blood type O (1), and duplicate record (1), 772 newborns remained. Of 772, a subsample of 346 newborns with both TcB and total serum bilirubin (TSB) tests within 1 hour of the sixth hour was analyzed to determine the predictive ability. RESULTS: Phototherapy was required in 281 of 772 (36.4%); 156 (20.2%) in the first 24 hours. There were 10 (1.3%) admissions for hyperbilirubinemia to the NICU for intravenous immunoglobin. Birth weight, infant blood type B, TSB, reticulocyte count, and TcB were all significantly associated with phototherapy ≤24 hours. On multivariate analysis, significant predictors of phototherapy ≤24 hours were TSB and reticulocyte count if no TcB was done and TcB alone if no blood tests were done. TcB was highly predictive (odds ratio 3.1, 95% confidence interval: 2.4-4.0) and nearly as accurate as the TSB and reticulocyte count (area under the curve, 0.90 and 0.96, respectively). Low (<3.0 mg/dL) and high (≥5.3 mg/dL) risk TcB cutoffs demonstrated a negative predictive value of 98% and positive predictive value of 85%, respectively. CONCLUSIONS: Among high-risk ABO incompatible DAT positive newborns, the sixth-hour TcB is highly predictive of the need for phototherapy ≤24 hours.

Transcatheter and Surgical Aortic Valve Implantation in Children, Adolescents, and Young Adults With Congenital Heart Disease.

Transcatheter aortic valve implantation (TAVI) is common in adults but rare in children and adolescents. Since 2014, our institution has incorporated a transcatheter approach as an option for aortic valve replacement in this population. The purpose of this study was to compare short-term outcomes of TAVI with surgical aortic valve replacement (SAVR). This single-center, retrospective study included patients aged 10 to 21 years who had a native SAVR or TAVI between January 2010 to April 2020. Comparative analysis of baseline characteristics and a composite outcome (stroke within 6 months, readmission within 30 days, death) between SAVR and TAVI were made using chi-square test or Wilcoxon rank sum test, as appropriate. Of the 77 patients who underwent native aortic valve implantation during the study period (60 SAVR, 17 TAVI), 46 were aged 10 to 21 years (30 SAVR, 16 TAVI). Median follow-up was 3.8 years (interquartile range 1.5 to 4.9) for the SAVR group and 1.5 years (interquartile range 1.1 to 1.2) for the TAVI group. There was no difference in the composite outcome between groups. Patients in the SAVR group were more likely to have undergone concomitant surgical intervention and have longer intensive care unit and hospital stays. In conclusion, our study suggests similar short-term outcomes between SAVR and TAVI in children and young adults aged 10 to 21 years. Longer-term studies are essential to understand the utility of TAVI and to better consider the option of a transcatheter approach as an alternative to SAVR in the pediatric population.