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Children with single ventricle heart disease typically require a series of three operations, (1) Norwood, (2) Glenn, and (3) Fontan, which ultimately results in complete separation of the pulmonary and systemic circuits to improve pulmonary/systemic circulation. In the last stage, the Fontan operation, the inferior vena cava (IVC) is connected to the pulmonary arteries (PAs), allowing the remainder of deoxygenated blood to passively flow to the pulmonary circuit. It is hypothesized that optimizing the Fontan anatomy would lead to decreased power loss and more balanced hepatic flow distribution. One approach to optimizing the geometry is to create a patient-specific digital twin to simulate various configurations of the Fontan conduit, which requires a computational model of the proximal PA anatomy and resistance, as well as the distal Pulmonary Vascular Resistance (PVR), at the Glenn stage. To that end, an optimization pipeline was developed using 3D computational fluid dynamics (CFD) and 0D lumped parameter (LP) simulations to iteratively refine the PVR of each lung by minimizing the simulated flow and pressure error relative to patients' cardiac magnetic resonance (CMR) and catheterization (CATH) data. While the PVR can also be estimated directly by computing the ratio of pressure gradients and flow from CATH and CMR data, the computational approach can separately identify the different components of PVR along the Glenn pathway, allowing for a more detailed depiction of the Glenn vasculature. Results indicate good correlation between the optimized PVR of the CFD and LP models (n = 16), with an intraclass correlation coefficient (ICC) of 0.998 (p = 0.976) and 0.991 (p = 0.943) for the left and right lung, respectively. Furthermore, compared to CMR flow and CATH pressure data, the optimized PVR estimates result in mean outlet flow and pressure errors of less than 5%. The optimized PVR estimates also agree well with the computed PVR estimates from CATH pressure and CMR flow for both lungs, yielding a mean difference of less than 4%., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Laudenschlager et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Background: ELP1 pathogenic variants (PV) have been recently identified as the most frequent variants predisposing to Sonic Hedgehog (SHH) medulloblastomas (MB); however, guidelines are still lacking for genetic counseling in this new syndrome., Methods: We retrospectively reviewed clinical and genetic data of a French series of 29 ELP1 -mutated MB., Results: All patients developed SHH-MB, with a biallelic inactivation of PTCH1 found in 24 tumors. Other recurrent alterations encompassed the TP53 pathway and activation of MYCN/MYCL signaling. The median age at diagnosis was 7.3 years (range: 3-14). ELP1 -mutated MB behave as sporadic cases, with similar distribution within clinical and molecular risk groups and similar outcomes (5 y - OS = 86%); no unusual side effect of treatments was noticed. Remarkably, a germline ELP1 PV was identified in all patients with available constitutional DNA ( n  = 26); moreover, all tested familial trio ( n  = 11) revealed that the PVs were inherited. Two of the 26 index cases from the French series had a family history of MB; pedigrees from these patients and from 1 additional Dutch family suggested a weak penetrance. Apart from MB, no cancer was associated with ELP1 PVs; second tumors reported in 4 patients occurred within the irradiation fields, in the usual time-lapse for expected radiotherapy-induced neoplasms., Conclusions: The low penetrance, the "at risk' age window limited to childhood and the narrow tumor spectrum, question the actual benefit of genetic screening in these patients and their family. Our results suggest restricting ELP1 germline sequencing to patients with SHH-MB, depending on the parents" request., Competing Interests: None declared., (© The Author(s) 2024. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)

Introduction: Children with single ventricle heart disease (SVHD) experience significant morbidity across systems and time, with 70% of patients experiencing acute kidney injury, 33% neurodevelopmental impairment, 14% growth failure, and 5.5% of patients suffering necrotizing enterocolitis. Proteomics is a method to identify new biomarkers and mechanisms of injury in complex physiologic states., Methods: Infants with SVHD in the interstage period were compared to similar-age healthy controls. Serum samples were collected, stored at -80°C, and run on a panel of 1,500 proteins in single batch analysis (Somalogic Inc., CO). Partial Least Squares-Discriminant Analysis (PLS-DA) was used to compare the proteomic profile of cases and controls and t-tests to detect differences in individual proteins (FDR <0.05). Protein network analysis with functional enrichment was performed in STRING and Cytoscape., Results: PLS-DA readily discriminated between SVHD cases ( n  = 33) and controls ( n  = 24) based on their proteomic pattern alone (Accuracy = 0.96, R 2  = 0.97, Q 2  = 0.80). 568 proteins differed between groups (FDR <0.05). We identified 25 up-regulated functional clusters and 13 down-regulated. Active biological systems fell into six key groups: angiogenesis and cell proliferation/turnover, immune system activation and inflammation, altered metabolism, neural development, gastrointestinal system, and cardiac physiology and development., Conclusions: We report a clear differentiation in the circulating proteome of patients with SVHD and healthy controls with >500 circulating proteins distinguishing the groups. These proteomic data identify widespread protein dysregulation across multiple biologic systems with promising biological plausibility as drivers of SVHD morbidity., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2023 Thomson, Mancuso, Wolfe, Khailova, Niemiec, Ali, DiMaria, Mitchell, Twite, Morgan, Frank and Davidson.)

Youth with Fontan circulation (Fontan) are at-risk for impairments in attention and executive functioning (EF) due to a confluence of genetic, prenatal, surgical, and medical risk factors. We sought to describe attention and EF in this population, measured via standardized performance-based tests and caregiver rating scales. We then examined how weaknesses in attention and EF were related to outcomes in other neurobehavioral domains, including adaptive behavior and academic achievement. Our sample included 93 youth with Fontan who were referred for neuropsychological evaluations as part of standard clinical care. The cohort as a whole measured between 0.18 to 0.99 standard deviations below normative means across domains of attention, EF, academic achievement, and intellectual ability. In addition, caregiver-reported concerns for attention, EF, anxiety, and depression were elevated, and approximately 0.35 to 0.85 standard deviations above normative means. Lastly, caregiver-reported adaptive behavior measured 0.93 to 1.24 standard deviations below normative values. Academic outcomes were differentially affected by demographic and attention/EF variables, while depression and caregiver-reported EF predicted adaptive behavior. Findings from this study underscore the importance of routine neuropsychological evaluation as part of comprehensive, multidisciplinary care for individuals with Fontan, with the goal of enhancing neurobehavioral and functional outcomes across the lifespan.

Germline DNA alterations affecting homologous recombination pathway genes have been associated with pancreatic cancer (PC) risk. BRCA2 is the most studied gene and affects the management of PC patients and their families. Even though recent reports have suggested a similar role of germline ATM pathogenic variants (PV) in familial PC, there is still a disagreement between experts on how it could affect patient management given the lack of proper PC risk estimates. We retrospectively analyzed the germline data of 257 PC patients among whom nearly 50% were sporadic cases. We showed similar frequencies of BRCA2 (4.9%) and ATM (4.4%) PV or likely pathogenic variants, which were not related to familial history. Based on our findings and that of the literature, we suggest including ATM gene among the panel of genes analyzed in PC patients pending the publication of prospective studies., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: There are several limitations when using creatinine to estimate glomerular filtration rate, especially in children with chronic medical conditions who are at high risk of kidney dysfunction. Cystatin C has been the recent focus of research as a replacement biomarker for creatinine. Our objective was to compare the 2 biomarkers in pediatric single-ventricle heart disease patients who have undergone the Fontan operation. We hypothesized that there would be poor correlation and agreement between the 2 estimates of renal function., Methods: This was a single center retrospective chart review of 20 patients who had previously undergone Fontan operation. Demographic and clinical data were collected from medical records. Blood samples were collected as part of routine clinical care and simultaneously measured for serum creatinine and cystatin C. Glomerular filtration rate was calculated using the creatinine-based bedside Schwartz formula and cystatin C-based Zapatelli equation. Spearman correlation and Bland-Altman analysis were used to assess correlation and agreement., Results: The median Schwartz-derived estimated glomerular filtration rate was 98.94 mL/min/1.73 m 2 while the median Zappitelli-derived estimated glomerular filtration rate was 84.76 mL/min/1.73 m 2 . The mean difference was -19.27 suggesting poor agreement. There was weak to moderate correlation between the Schwartz and cystatin C estimated glomerular filtration rate., Conclusion: The bedside Schwartz formula may be an overestimate of glomerular filtration rate in pediatric single-ventricle heart disease patients who have undergone the Fontan operation. While larger studies are necessary, cystatin C is a promising biomarker to replace creatinine and better estimate kidney function in this population., (© 2019 Wiley Periodicals, Inc.)

Despite different developmental and pathological processes affecting lung vascular remodeling in both patient populations, differences in 4D MRI findings between children and adults with PAH have not been studied. The purpose of this study was to compare flow hemodynamic state, including flow-mediated shear forces, between pediatric and adult patients with PAH matched by severity of pulmonary vascular resistance index (PVRi). Adults ( n = 10) and children ( n = 10) with PAH matched by pulmonary vascular resistance index (PVRi) and healthy adult ( n = 10) and pediatric ( n = 10) subjects underwent comprehensive 4D-flow MRI to assess peak systolic wall shear stress (WSS max ) measured in the main (MPA), right (RPA), and left pulmonary arteries (LPA), viscous energy loss (E L ) along the MPA-RPA and MPA-LPA tract, and qualitative analysis of secondary flow hemodynamics. WSS max was decreased in all pulmonary vessels in children with PAH when compared with the same age group (all P < 0.05). Similarly, WSS max was decreased in all pulmonary vessels in adult PAH patients when compared with healthy adult subjects (all P < 0.01). Average E L was increased in adult patients with PAH when compared with the same age group along both MPA-RPA ( P = 0.020) and MPA-LPA ( P = 0.025) tracts. There were no differences in E L indices between adults and pediatric patients. Children and adult patients with PAH have decreased shear hemodynamic forces. However, pathological flow hemodynamic formations appear to be more consistent in adult patients, whereas flow hemodynamic abnormalities appear to be more variable in children with PAH for comparable severity of PVRi. NEW & NOTEWORTHY Both children and adult patients with PAH have decreased shear hemodynamic forces inside the pulmonary arteries associated with the degree of vessel dilation and stiffness. These differences also exist between healthy normotensive children and adults. However, pathological flow hemodynamic formations appear to more uniform in adult patients, whereas in children with PAH flow, hemodynamic abnormalities appear to be more variable. Pathological flow formations appear not to have a major effect on viscous energy loss associated with the flow conduction through proximal pulmonary arteries.

Background: Patients with diagnosed Kawasaki disease (KD) are known to develop extracardiac vascular lesions and are prone to accelerated stiffening of medium-size arteries., Purpose: To noninvasively evaluate great vessel (central aorta and main pulmonary artery (MPA)) stiffness using phase-contrast MRI (PC-MRI)., Study Type: Retrospective review., Subjects: Thirty-three patients with previously diagnosed KD and 15 control subjects underwent PC-MRI evaluation., Field Strength/sequence: A free-breathing PC-MRI sequence was applied with Cartesian encoding and retrospective sorting using a 1.5 or 3.0T system., Assessment: We evaluated regionally specific vessel stiffness using pulse-wave velocity (PWV) and relative area change (RAC) at the ascending aorta, descending aorta, and MPA., Statistical Tests: Hemodynamics among patients with KD and controls were compared using Student's t-test, Wilcoxon Rank-sum, and χ 2 . Additional group-specific comparisons were performed using Kruskal-Wallis or one-way analysis of variance (ANOVA)., Results: Patients with KD showed elevated PWV in both ascending (5.0 ± 1.2 vs. 2.4 ± 0.5, P < 0.001) and descending aorta (4.4 ± 2.1 vs. 2.8 ± 0.8, P < 0.001). RAC was correspondingly reduced in both segments (both P < 0.01). PWV measured in MPA was increased in KD patients (2.2 ± 0.5 vs. 1.5 ± 0.6, P = 0.045) while the RAC was reduced (34 ± 6 vs. 47 ± 3, P = 0.045). There were no associations between considered vessel stiffness indices and respective ventricular size and function, functional indices, and no correlations were observed with KD severity markers., Data Conclusion: Patients with KD have elevated great vessel stiffness measured at the chronic stage of the disease. Accelerated stiffness process does not appear to affect biventricular function in youth Level of Evidence: 1 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018;47:1228-1236., (© 2018 International Society for Magnetic Resonance in Medicine.)

Objectives: Turner syndrome (TS) and Marfan syndrome (MFS) are partially characterized by aortopathies with a risk of developing severe aortic dilation, stiffness and consequent dissection and aneurysm formation. The incidence of a bicuspid aortic valve (BAV) is also increased in TS. We investigated aortic stiffness in teenage TS and MFS patients and evaluated to what degree stiffness in TS patients is augmented by the presence of a BAV., Methods: Fifty-seven patients with TS (n = 37) and MFS (n = 20), as well as 22 controls with similar age and size distribution underwent evaluation of thoracic aortic stiffness using phase-contrast magnetic resonance imaging. Calculated stiffness indices including pulse wave velocity (PWV), distensibility and relative area change (RAC) were collected to characterize the ascending aorta and descending aorta. PWV was also determined to evaluate global aortic arch stiffness., Results: Patients with TS had reduced distensibility (0.43 vs 0.58%/mmHg, P < 0.05) and RAC (21 vs 29%, P < 0.01) in the ascending aorta when compared with normal controls. Similarly, patients with MFS had reduced ascending aortic distensibility (0.39 vs 0.58%/mmHg, P < 0.05) and RAC (22 vs 29%, P < 0.05). There were no differences in measured PWV in the ascending aorta. Patients with TS had significantly elevated PWV measured in the aortic arch when compared with controls (2.7 vs 1.9 m/s, P < 0.05). Patients with MFS had more prominent elevation in aortic arch PWV (4.2 vs 1.9 m/s, P < 0.01). The descending aortas had decreased distensibility (0.36 vs 0.55%/mmHg, P < 0.05) and RAC (18 vs 25%, P < 0.01) only in MFS patients. Additionally, 18 TS patients with a BAV were compared with 19 TS patients with a trileaflet aortic valve, without significant differences observed in any of the considered stiffness indices., Conclusions: TS and MFS teenage patients display evidence of increased aortic stiffness. In TS patients, this is focused in the ascending aorta and is independent of the presence of a BAV. MFS patients display a generalized reduction in compliance of the entire aorta.

Coarctation of the aorta has been associated with increased thoracic aortic stiffness in adolescents and young adults. However, the effects of different therapeutic strategies on aortic stiffness in a young population is unknown. This study aimed to non-invasively assess aortic stiffness between different repair or intervention strategies in patients with coarctation of the aorta. Forty-nine coarctation patients who underwent either surgery (n = 26), balloon angioplasty (n = 14), or stent implantation (n = 12), and 26 age- and size-matched normotensive healthy controls underwent evaluation of thoracic aortic stiffness and flow hemodynamics via phase-contrast cardiac magnetic resonance. In children who had undergone surgical repair or balloon angioplasty, ascending aortic stiffness was increased when measured via pulse wave velocity (PWV) when compared to normal controls (all P < 0.05). Furthermore, ascending aortic distensibility and relative area change (RAC) was significantly lower in surgically and balloon treated groups (both P < 0.01). Stiffness (PWV), distensibility, and RAC in the ascending aorta were not statistically different between stented patients and controls. The ascending aorta of children following surgical repair or balloon angioplasty demonstrated signs of elevated stiffness, whereas those treated by stent implantation showed no difference in stiffness markers when compared to normal controls.

Background: Germline mutations of suppressor of fused homolog (SUFU) predispose to sonic hedgehog (SHH) medulloblastoma. Germline SUFU mutations have been reported in nevoid basal cell carcinoma syndrome (NBCCS), but little is known about the cancer risk and clinical spectrum., Methods: We performed a retrospective review of all patients with medulloblastoma and a germline SUFU mutation in France., Results: Twenty-two patients from 17 families were identified with medulloblastoma and a germline SUFU mutation (median age at diagnosis: 16.5 mo). Macrocrania was present in 20 patients, but only 5 met the diagnostic criteria for NBCCS. Despite treatment with surgery and chemotherapy, to avoid radiotherapy in all patients except one, the outcome was worse than expected for SHH medulloblastoma, due to the high incidence of local relapses (8/22 patients) and second malignancies (n = 6 in 4/22 patients). The 5-year progression-free survival and overall survival rates were 42% and 66%. Mutations were inherited in 79% of patients, and 34 additional SUFU mutation carriers were identified within 14 families. Medulloblastoma penetrance was incomplete, but higher than in Patched 1 (PTCH1) mutation carriers. Besides medulloblastoma, 19 other tumors were recorded among the 56 SUFU mutation carriers, including basal cell carcinoma (BCC) in 2 patients and meningioma in 3 patients., Conclusion: Germline SUFU mutations strongly predispose to medulloblastoma in the first years of life, with worse prognosis than usually observed for SHH medulloblastoma. The clinical spectrum differs between SUFU and PTCH1 mutation carriers, and BCC incidence is much lower in SUFU mutation carriers. The optimal treatment of SUFU mutation-associated medulloblastoma has not been defined.

Background: Electrical and right ventricular (RV) mechanical dyssynchrony has been previously described in pediatric pulmonary arterial hypertension (PAH), but less is known about the relationship between electrical dyssynchrony and biventricular function. In this study we applied cardiac magnetic resonance (CMR) imaging to evaluate biventricular size and function with a focus on left ventricular (LV) strain mechanics in pediatric PAH patients with and without electrical dyssynchrony., Methods: Fifty-six children with PAH and comprehensive CMR evaluation were stratified based on QRS duration z-score, with electrical dyssynchrony defined as z-score ≥2. Comprehensive biventricular volumetric, dyssynchrony, and strain analysis was performed., Results: Nineteen PAH patients had or developed electrical dyssynchrony. Patients with electrical dyssynchrony had significantly reduced RV ejection fraction (35% vs 50%, p = 0.003) and greater end-diastolic (168 vs 112 ml/m 2 , p = 0.041) and end-systolic (119 vs 57, ml/m 2 , p = 0.026) volumes. Patients with electrical dyssynchrony had reduced RV longitudinal strain (-14% vs -19%, p = 0.007), LV circumferential strain measured at the free wall (-19% vs -22%, p = 0.047), and the LV longitudinal strain in the septal region (-10% vs -15%, p = 0.0268). LV mechanical intraventricular dyssynchrony was reduced in patients with electrical dyssynchrony at the LV free wall (43 vs 19 ms, p = 0.019)., Conclusions: The electrical dyssynchrony is associated with the reduced LV strain, enlarged RV volumes, and reduced biventricular function in children with PAH. CMR assessment of biventricular mechanical function with respect to QRS duration may help to detect pathophysiologic processes associated with progressed PAH., (Copyright © 2018 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Feeding practices after neonatal and congenital heart surgery are complicated and variable, which may be associated with prolonged hospitalization length of stay (LOS). Systematic assessment of feeding skills after cardiac surgery may earlier identify those likely to have protracted feeding difficulties, which may promote standardization of care., Methods: Neonates and infants ≤3 months old admitted for their first cardiac surgery were retrospectively identified during a 1-year period at a single center. A systematic feeding readiness assessment (FRA) was utilized to score infant feeding skills. FRA scores were assigned immediately prior to surgery and 1, 2, and 3 weeks after surgery. FRA scores were analyzed individually and in combination as predictors of gastrostomy tube (GT) placement prior to hospital discharge by logistic regression., Results: Eighty-six patients met inclusion criteria and 69 patients had complete data to be included in the final model. The mean age of admit was five days and 51% were male. Forty-six percent had single ventricle physiology. Twenty-nine (42%) underwent GT placement. The model containing both immediate presurgical and 1-week postoperative FRA scores was of highest utility in predicting discharge with GT (intercept odds = 10.9, P = .0002; sensitivity 69%, specificity 93%, AUC 0.913). The false positive rate was 7.5%., Conclusions: In this analysis, systematic and standardized measurements of feeding readiness employed immediately before and one week after congenital cardiac surgery predicted need for GT placement prior to hospital discharge. The FRA score may be used to risk stratify patients based on likelihood of prolonged feeding difficulties, which may further improve standardization of care., (© 2017 Wiley Periodicals, Inc.)

Introduction: Variable compliance to postoperative feeding algorithms after pediatric cardiac surgery may be associated with suboptimal growth, decreased parental satisfaction, and prolonged hospital length of stay (LOS). Our heart center performed an audit of compliance to a previously introduced postoperative feeding algorithm to guide quality improvement efforts. We hypothesized that algorithm noncompliance would be associated with increased LOS., Methods: We retrospectively identified children ≤ 3 months admitted for their first cardiac surgery between January 1, 2015 and December 31, 2016. The algorithm uses objective oral feeding readiness assessments (FRA). At the end of a predefined evaluation period, a "sentinel" FRA score is assigned. The sentinel FRA and FRA trend guide decisions to pursue gastrostomy tube (GT) or oral-only feeds. Among those who reached the sentinel FRA, we defined compliance as ≤ 3 days before pursuing GT or oral-only feeds once indicated by the algorithm., Results: Sixty-nine patients were included. Forty-nine complied with the algorithm (71%), and 45 received GT (65.2%). Noncompliers had significantly longer LOS (34 versus 25 days; P = 0.01). Among GT recipients, noncompliers waited 6 additional days for a GT compared with compliers ( P ≤ 0.001). Subjective decisions to extend oral feeding trials or await results of a swallow study were associated with algorithm noncompliance., Conclusions: This audit of compliance to a feeding algorithm after pediatric cardiac surgery highlighted variability of practice, including relying on subjective appraisals of feeding skills over objective FRAs. This variability was associated with increased LOS and can be hypothesis-generating for future quality improvement efforts.

Percutaneous pulmonary valve intervention (PPVI) is a less invasive and less costly approach to pulmonary valve replacement compared with the surgical alternative. Potential complications of PPVI include coronary compression and pulmonary arterial injury/rupture. The purpose of this study was to characterize the morphological risk factors for PPVI complication with cardiac MRI and cardiac CTA. A retrospective review of 88 PPVI procedures was performed. 44 patients had preprocedural cardiac MRIs or CTAs available for review. Multiple morphological variables on cardiac MRI and CTA were compared with known PPVI outcome and used to investigate associations of variables in determining coronary compression or right ventricular-pulmonary arterial conduit injury. The most significant risk factor for coronary artery compression was the proximity of the coronary arteries to the conduit. In all patients with coronary compression during PPVI, the coronary artery touched the conduit on the preprocedural CTA/MRI, whilst in patients without coronary compression the mean distance between the coronary artery and the conduit was 4.9 mm (range of 0.8-20 mm). Multivariable regression analysis demonstrated that exuberant conduit calcification was the most important variable for determining conduit injury. Position of the coronary artery directly contacting the conduit without any intervening fat may predict coronary artery compression during PPVI. Exuberant conduit calcification increases the risk of PPVI-associated conduit injury. Close attention to these factors is recommended prior to intervention in patients with pulmonary valve dysfunction.

Real time three-dimensional echocardiography (RT3DE) allows for accurate morphological characterization of congenital heart disease and complements two-dimensional (2D) echocardiography. Accurate evaluation of the mitral valve and supramitral ring prior to surgery aids the surgeon in successful resection of the ring and repair of associated mitral valve abnormalities. We report a case of congenital mitral valve stenosis from a supramitral ring in a young child using real time three-dimensional transesophageal echocardiography., (© 2014, Wiley Periodicals, Inc.)

In anomalous left coronary artery from the pulmonary artery (ALCAPA), infants 6 to 12 weeks will often present with symptoms consistent with reflux or bronchiolitis. In those infants diagnosed with both ALCAPA and concomitant active respiratory syncytial virus (RSV) bronchiolitis, others have reported delaying revascularization therapy until resolution of the RSV bronchiolitis. Here, we report the case of a three-month-old infant, diagnosed with ALCAPA and active RSV bronchiolitis, who underwent successful myocardial revascularization within 24 hours of presentation and diagnosis.

Study Objectives: To determine the effects of bilevel positive airway pressure with pressure release technology (Bi-Flex) on adherence and efficacy in children and adolescents compared to standard continuous positive airway pressure (CPAP) therapy. We hypothesized that Bi-Flex would result in improved adherence but similar efficacy to CPAP., Methods: This was a randomized, double-blinded clinical trial. Patients with obstructive sleep apnea were randomized to CPAP or Bi-Flex. Repeat polysomnography was performed on pressure at 3 months. Objective adherence data were obtained at 1 and 3 months., Results: 56 children and adolescents were evaluated. There were no significant differences in the number of nights the device was turned on, or the mean number of minutes used at pressure per night for CPAP vs Bi-Flex (24 ± 6 vs 22 ± 9 nights, and 201 ± 135 vs 185 ± 165 min, respectively, for Month 1). The apnea hypopnea index decreased significantly from 22 ± 21/h to 2 ± 3/h on CPAP (p = 0.005), and 18 ± 15/h to 2 ± 2/h on Bi-Flex (p < 0.0005), but there was no significant difference between groups (p = 0.82 for CPAP vs Bi-Flex). The Epworth Sleepiness Scale decreased from 8 ± 5 to 6 ± 3 on CPAP (p = 0.14), and 10 ± 6 to 5 ± 5 on Bi-Flex (p < 0.0005; p = 0.12 for CPAP vs Bi-Flex)., Conclusions: Both CPAP and Bi-Flex are efficacious in treating children and adolescents with OSAS. However, adherence is suboptimal with both methods. Further research is required to determine ways to improve adherence in the pediatric population.