Intubation and Lower Saturation in the Delivery Room are Associated with Balloon Atrial Septostomy in Neonates with Transposition of the Great Arteries.

OBJECTIVE: To identify delivery room (DR) characteristics of patients with transposition of the great arteries (TGA) who underwent preoperative balloon atrial septostomy (BAS). STUDY DESIGN: Retrospective cohort study of all patients with prenatally diagnosed TGA delivered at our center between 2013 and 2023 who underwent arterial switch operation during the newborn admission. RESULTS: A total of 168 patients were included (median gestational age 39.5 weeks, 64% male, 33% with ventricular septal defect, 8% with aortic arch hypoplasia). BAS was performed in 84 patients (50%). Patients who underwent BAS had higher proportion of intubation in the DR (87% vs 33%, P < .001), lower maximum oxygen saturation in the first 10 minutes (64% vs 74%, P < .001) and 20 minutes (71% vs 81%, P < .001) of life, and lower maximum oxygen saturation at any point in the DR (79% vs 87%, P < .001). Adjusting for confounders (ventricular septal defect, aortic arch anomaly, 5-minute Apgar, birth weight), intubation in the DR (aOR 9.5, 95% CI 3.9, 25.0) and lower maximum oxygen saturation in the DR (aOR 0.9, 95% CI 0.8, 0.9) were independently associated with BAS. By receiver operating characteristic analysis, a maximum oxygen saturation of less than 86% at any time point in the DR discriminated for BAS with a specificity of 0.88, sensitivity of 0.70, and area under the curve of 0.82. CONCLUSIONS: Intubation and lower oxygen saturation in the DR are independently associated with BAS in patients with TGA born at our center. A maximum DR saturation of less than 86% best discriminates patients who undergo BAS in our population.

Outcomes Associated with Giant Coronary Artery Aneurysms after Kawasaki Disease: A Single-Center United States Experience.

OBJECTIVES: To determine the long-term outcomes among a cohort of patients with Kawasaki disease (KD) and a history of giant coronary artery aneurysms (CAAs) at a single US center. STUDY DESIGN: Medical records for all patients with KD and giant CAAs at a pediatric academic institution were reviewed. Primary outcomes included major adverse cardiovascular events (MACE) and normalization of CA luminal diameter, using Kaplan-Meier analyses. RESULTS: There were 60 patients with KD and giant CAAs identified between 1989 and 2023. The majority of patients were male (71.7%) with a median age at diagnosis of 0.9 years (range, 0.2-13.3 years). Patients were followed for a median of 11 years, up to 34.5 years. MACE occurred in 13 patients (21.7%) at a median of 1.4 years (range, 0.04-22.6 years) after KD diagnosis. The 10-, 20-, and 30-year MACE-free rates were 75%, 75%, and 60%. Patients with maximal CA z scores of ≥20 or bilateral CAA were more likely to have MACE. During follow-up, 26.7% of CAA regressed to a normal luminal diameter at a median of 3.6 years (range, 0.6-12.0 years). The 10-, 20- and 30-year likelihood of CA regression to normal luminal diameter was 36%, 46%, and 46%. CONCLUSIONS: Over 30 years, MACE occurred in nearly 22% of patients, more often in those with bilateral CAA or CA z scores of ≥20. Despite regression to a normal luminal diameter in >25% of CAAs, patients with a history of KD-associated giant CAA require ongoing surveillance for cardiac complications, even years after the initial disease.

Outcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus.

OBJECTIVE: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. RESULTS: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P = .034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). CONCLUSIONS: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.

Current Trends in Invasive Closure of Patent Ductus Arteriosus in Very Low Birth Weight Infants in United States Children's Hospitals, 2016-2021.

OBJECTIVE: To describe the current practices in invasive patent ductus arteriosus (PDA) closure (surgical ligation or transcatheter occlusion) in very low birth weight (VLBW) infants and changes in patient characteristics and outcomes from 2016 to 2021 among US children's hospitals. STUDY DESIGN: We evaluated a retrospective cohort of VLBW infants (birth weight 400-1499 g and gestational age 22-31 weeks) who had invasive PDA closure within 6 months of age from 2016 to 2021 in children's hospitals in the Pediatric Health Information System. Changes in patient characteristics and outcomes over time were evaluated using generalized linear models and generalized linear mixed models. RESULTS: 2418 VLBW infants (1182 surgical ligation; 1236 transcatheter occlusion) from 42 hospitals were included. The proportion of infants receiving transcatheter occlusion increased from 17.2% in 2016 to 84.4% in 2021 (P < .001). In 2021, 28/42 (67%) hospitals had performed transcatheter occlusion in > 80% of their VLBW infants needing invasive PDA closure, compared with only 2/42 (5%) in 2016. Although median postmenstrual age (PMA) at PDA closure did not change for the overall cohort, PMA at transcatheter occlusion decreased from 38 weeks in 2016 to 31 weeks by 2020, P < .001. Among those infants not intubated prior to PDA closure, extubation within 3 days postprocedure increased over time (yearly adjusted odds ratios of 1.26 [1.08-1.48]). Length of stay and mortality did not change over time. CONCLUSION: We report rapid adoption of transcatheter occlusion for PDA among VLBW infants in US children's hospitals over time. Transcatheter occlusions were performed at younger PMA over time.

High pressure endoscopic irrigation: impact on renal histology

ABSTRACT Purpose: High intra-renal pressures during flexible ureteroscopy have been associated with adverse renal tissue changes as well as pyelovenous backflow. Our objective was to investigate the effect of various intra-renal pressures on histologic changes and fluid extravasation during simulated ureteroscopy. Materials and Methods: Twenty-four juvenile pig kidneys with intact ureters were cannulated with an Olympus flexible ureteroscope with and without a ureteral access sheath and subjected to India ink-infused saline irrigation for 30 minutes at constant pressures ranging from sphygmomanometer settings of 50mm, 100mm and 200mmHg. Renal tissue samples were collected, processed and stained, and were evaluated by a blinded pathologist for depth of ink penetration into renal parenchyma as a percentage of total parenchymal thickness from urothelium to renal capsule. Results: The mean percentage of tissue penetration for kidneys with ink present in the cortical tubules at sphygmomanometer pressure settings of 50, 100, and 200mm Hg without a ureteral access sheath was 33.1, 31.0 and 99.3%, respectively and with ureteral access sheath was 0, 0 and 18.8%, respectively. Overall, kidneys with an access sheath demonstrated a smaller mean tissue penetration among all pressure compared to kidneys without a sheath (6.3% vs. 54.5%, p=0.0354). Of kidneys with sheath placement, 11% demonstrated any ink compared to 56% of kidneys without sheath placement. Conclusions: Pressurized endoscopic irrigation leads to significant extravasation of fluid into the renal parenchyma. Higher intra-renal pressures were associated with increased penetration of irrigant during ureteroscopy in an ex-vivo porcine model.

High pressure endoscopic irrigation: impact on renal histology.

PURPOSE: High intra-renal pressures during flexible ureteroscopy have been associated with adverse renal tissue changes as well as pyelovenous backflow. Our objective was to investigate the effect of various intra-renal pressures on histologic changes and fluid extravasation during simulated ureteroscopy. MATERIALS AND METHODS: Twenty-four juvenile pig kidneys with intact ureters were cannulated with an Olympus flexible ureteroscope with and without a ureteral access sheath and subjected to India ink-infused saline irrigation for 30 minutes at constant pressures ranging from sphygmomanometer settings of 50mm, 100mm and 200mmHg. Renal tissue samples were collected, processed and stained, and were evaluated by a blinded pathologist for depth of ink penetration into renal parenchyma as a percentage of total parenchymal thickness from urothelium to renal capsule. RESULTS: The mean percentage of tissue penetration for kidneys with ink present in the cortical tubules at sphygmomanometer pressure settings of 50, 100, and 200mm Hg without a ureteral access sheath was 33.1, 31.0 and 99.3%, respectively and with ureteral access sheath was 0, 0 and 18.8%, respectively. Overall, kidneys with an access sheath demonstrated a smaller mean tissue penetration among all pressure compared to kidneys without a sheath (6.3% vs. 54.5%, p=0.0354). Of kidneys with sheath placement, 11% demonstrated any ink compared to 56% of kidneys without sheath placement. CONCLUSIONS: Pressurized endoscopic irrigation leads to significant extravasation of fluid into the renal parenchyma. Higher intra-renal pressures were associated with increased penetration of irrigant during ureteroscopy in an ex-vivo porcine model.

A user guide to the American Society of Hematology clinical practice guidelines.

Since November 2018, Blood Advances has published American Society of Hematology (ASH) clinical practice guidelines on venous thromboembolism, immune thrombocytopenia, and sickle cell disease. More ASH guidelines on these and other topics are forthcoming. These guidelines have been developed using consistent processes, methods, terminology, and presentation formats. In this article, we describe how patients, clinicians, policymakers, researchers, and others may use ASH guidelines and the many related derivates by describing how to interpret information and how to apply it to clinical decision-making. Also, by exploring how these documents are developed, we aim to clarify their limitations and possible inappropriate usage.

Risk Factors for Major Early Adverse Events Related to Cardiac Catheterization in Children and Young Adults With Pulmonary Hypertension: An Analysis of Data From the IMPACT (Improving Adult and Congenital Treatment) Registry.

BACKGROUND: Cardiac catheterization is the gold standard for assessment and follow-up of patients with pulmonary hypertension (PH). To date, there are limited data about the factors that influence the risk of catastrophic adverse events after catheterization in this population. METHODS AND RESULTS: A retrospective multicenter cohort study was performed to measure risk of catastrophic adverse outcomes after catheterization in children and young adults with PH and identify risk factors for these outcomes. All catheterizations in children and young adults, aged 0 to 21 years, with PH at hospitals submitting data to the IMPACT (Improving Adult and Congenital Treatment) registry between January 1, 2011, and December 31, 2015, were studied. Using mixed-effects multivariable regression, we assessed the association between prespecified subject-, procedure-, and center-level covariates and the risk of death, cardiac arrest, or mechanical circulatory support during or after cardiac catheterization. A total of 8111 procedures performed in 7729 subjects at 77 centers were studied. The observed risk of the composite outcome was 1.4%, and the risk of death before discharge was 5.2%. Catheterization in prematurely born neonates and nonpremature infants was associated with increased risk of catastrophic adverse event, as was precatheterization treatment with inotropes and lower systemic arterial saturation. Secondary analyses demonstrated the following: (1) increasing volumes of catheterization in patients with PH were associated with reduced risk of composite outcome (odds ratio, 0.8 per 10 procedures; P=0.002) and (2) increasing pulmonary vascular resistance and pulmonary artery pressures were associated with increased risk (P<0.0001 for both). CONCLUSIONS: Young patients with PH are a high-risk population for diagnostic and interventional cardiac catheterization. Hospital experience with PH is associated with reduced risk, independent of total catheterization case volume.

Hepatic Fibrosis Is Universal Following Fontan Operation, and Severity is Associated With Time From Surgery: A Liver Biopsy and Hemodynamic Study.

BACKGROUND: Congestive hepatopathy is a recognized complication of Fontan physiology. Data regarding the incidence of hepatopathy and risk factors are lacking. METHODS AND RESULTS: Liver biopsies and cardiac catherizations were performed as part of an evaluation offered to all patients ≥10 years after Fontan. Quantitative determination of hepatic fibrosis was performed using Sirius red staining with automated calculation of collagen deposition per slide (%CD). Biopsies from included subjects were compared to stained specimens from controls without known fibrotic liver disease. Patient characteristics, echocardiographic findings, and hemodynamic measures were evaluated as potential risk factors. The cohort consisted of 67 patients (31 female) at mean age of 17.3±4.5 years and mean time from Fontan of 14.9±4.5 years. Right ventricular morphology was present in 37 subjects. Median %CD by Sirius red staining was 21.6% (range 8.7% to 49.4%) compared to 2.6% (range 2.2% to 3.0%) in controls. There was a significant correlation between time from Fontan and degree of Sirius red staining (r=0.33, P<0.01). Serum liver enzymes and platelet count did not correlate with %CD. The median inferior vena cava pressure was 13 mm Hg (range 6-24 mm Hg) and did not correlate with %CD. There was no difference in %CD based on ventricular morphology or severity of atrioventricular valve insufficiency. CONCLUSIONS: In this cohort of predominantly asymptomatic children and adolescents electively evaluated after a Fontan operation, all exhibited evidence for hepatic fibrosis as measured by collagen deposition in the liver. Time from Fontan was the only factor significantly associated with collagen deposition. These findings demonstrate that liver fibrosis is an inherent feature of Fontan physiology and that the degree of fibrosis increases over time.