Extracardiac Manifestations Fail to Predict the Severity of Cardiac Phenotype in Children and Young Adults with Marfan Syndrome.

We performed a secondary analysis of the Pediatric Heart Network (PHN) Marfan Trial public-use database to evaluate associations between extracardiac features and cardiac and aortic phenotypes in study participants. Aortic aneurysm phenotype was defined as aortic root Z-score ≥4.5, aortic root growth rate ≥75th percentile, aortic dissection, and aortic surgery. Severe cardiac phenotype was defined as aortic dissection, aortic Z-score ≥4.5, aortic valve surgery, at least moderate mitral regurgitation, mitral valve surgery, left ventricular dysfunction, or death. Extracardiac manifestations were characterized by specific organ system involvement and by a novel aggregate extracardiac score (AES) that was created for this study based on the original Ghent nosology. Mixed effects logistic regression analysis compared AES and systems involvement to outcomes. Of 608 participants (60% male), the median age at enrollment was 10.8 years (interquartile range: 6, 15.4). Aortic aneurysm phenotype was observed in 71% of participants and 64% had severe cardiac phenotype. On univariable analysis, skeletal (OR: 1.95, 95% CI: 1.01, 3.72; p = 0.05), skin manifestation (OR: 1.62, 95% CI: 1.13, 2.34; p = 0.01) and AES (OR: 1.17, 95% CI: 1.02, 1.34; p = 0.02) were associated with aortic aneurysm phenotype but were not significant in multivariable analysis. There was no association between extracardiac manifestations and severe cardiac phenotype. Thus, the severity of cardiac manifestations in Marfan syndrome (MFS) was independent of extracardiac phenotype and AES. Severity of extracardiac involvement did not appear to be a useful clinical marker for cardiovascular risk-stratification in this cohort of children and young adults with MFS.

Extracardiac Manifestations Fail to Predict the Severity of Cardiac Phenotype in Children and Young Adults with Marfan Syndrome.

We performed a secondary analysis of the Pediatric Heart Network Marfan Trial public-use database to evaluate associations between extracardiac features and cardiac and aortic phenotypes in study participants. Aortic aneurysm phenotype was defined as aortic root Z-score ≥ 4.5, aortic root growth rate ≥ 75th percentile, aortic dissection, and aortic surgery. Severe cardiac phenotype was defined as aortic dissection, aortic Z-score ≥4.5, aortic valve surgery, at least moderate mitral regurgitation, mitral valve surgery, left ventricular dysfunction, or death. Extracardiac manifestations were characterized by specific organ system involvement and by a novel aggregate extracardiac score that was created for this study based on the original Ghent nosology. Logistic regression analysis compared aggregate extracardiac score and systems involvement to outcomes. Of 608 participants (60% male), the median age at enrollment was 10.8 years (interquartile range: 6, 15.4). Aortic aneurysm phenotype was observed in 71% of participants and 64% had severe cardiac phenotype. On univariate analysis, skeletal (OR: 1.95, 95% CI: 1.01, 3.72; p = 0.05), skin manifestation (OR: 1.62, 95% CI: 1.13, 2.34; p = 0.01) and aggregate extracardiac score (OR: 1.17, 95% CI: 1.02, 1.34; p = 0.02) were associated with aortic aneurysm phenotype but were not significant in multivariate analysis. There was no association between extracardiac manifestations and severe cardiac phenotype. Thus, the severity of cardiac manifestations in Marfan syndrome was independent of extracardiac phenotype and aggregate extracardiac score. Severity of extracardiac involvement did not appear to be a useful clinical marker for cardiovascular risk-stratification in this cohort of children and young adults with Marfan syndrome.

Interdigitating Technique for Repair of Aortic Arch Obstruction to Reduce Reintervention Rates.

BACKGROUND: The incidence of reintervention for aortic arch obstruction is 5% to 14% after coarctation or hypoplastic aortic arch repair and 25% after the Norwood procedure. Institutional practice review indicated higher than reported reintervention rates. Our aim was to assess the impact of an interdigitating reconstruction technique on reintervention rates for recurrent aortic arch obstruction. METHODS: Children (<18 years) were included if they had undergone aortic arch reconstruction by sternotomy or the Norwood procedure. Three surgeons participated in the intervention with staggered rollout dates between June 2017 and January 2019, with the study ending December 2020 and review for reinterventions ending February 2022. Preintervention cohorts represented patients who underwent aortic arch reconstructions with patch augmentation, and postintervention cohorts represented patients who underwent an interdigitating reconstruction technique. Reinterventions by cardiac catheterization or operation were measured within 1 year of initial operation. Wilcoxon rank sum and χ2 tests were used to compare preintervention and postintervention cohorts. RESULTS: Overall, 237 patients were included for participation in this study, with 84 patients in the preintervention cohort and 153 in the postintervention cohort. Patients undergoing the Norwood procedure represented 30% (n = 25) of the retrospective cohort and 35% (n = 53) of the intervention cohort. Overall reinterventions were significantly decreased after the study intervention from 31% (n = 26/84) to 13% (n = 20/153; P < .001). Reintervention rates were decreased for each intervention cohort: aortic arch hypoplasia (24% [n = 14/59] vs 10% [n = 10/100]; P = .019) and Norwood procedure (48% [n = 12/25] vs 19% [n = 10/53]; P = .008). CONCLUSIONS: The interdigitating reconstruction technique for obstructive aortic arch lesions was successfully implemented and is associated with a decrease in reinterventions.

The Relationship Between Patient-Reported Outcomes and Patient Satisfaction With Ligament Reconstruction Tendon Interposition.

PURPOSE: Achieving the minimal clinically important difference (MCID) on an outcomes instrument and reporting satisfaction with surgical outcomes are not equivalent. We hypothesized that improvement exceeding the QuickDASH and PROMIS UE CAT MCID is associated with a greater likelihood of reporting satisfaction with ligament reconstruction tendon interposition (LRTI) treatment. Our secondary hypothesis was that a subset of patients failing to meet MCID would still be satisfied. METHODS: Patients ≥1 year after LRTI at one academic tertiary institution were included. QuickDASH and UE CAT v1.2 scores were obtained before and after surgery. Postoperative satisfaction and levels of improvement in pain and function were also obtained. RESULTS: A total of 93 patients completed the QuickDASH, and of those, 90 also completed the UE CAT. At a mean of 2.6 ± 1.0 years after surgery, QuickDASH and UE CAT score improvement exceeded the previously published MCID estimates of 8.8 and 4.8. Although 90% (84/93) of the patients reported satisfaction, only 85% (72/93) and 72% (59/90) achieved MCID on the QuickDASH and UE CAT, respectively. Using the MCID estimate of 8.8, 96% (72/75) of the patients meeting the MCID were satisfied with their treatment. Those failing to achieve MCID reported significantly less physical function and pain improvement; however, most were satisfied nonetheless (68% [13/19] for QuickDASH, 77% [23/30] for UE CAT). CONCLUSIONS: Achieving published MCID thresholds on the QuickDASH and PROMIS UE CAT v1.2 was predictive of patients reporting general satisfaction with their LRTI outcome ≥1 year after surgery. Most patients failing to achieve MCID still reported satisfaction with their LRTI. Achieving MCID thresholds alone should not be used as a surrogate for patient satisfaction with their treatment. Patient satisfaction is a complicated construct that is potentially very different from that of high-quality care. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic IV.

Prediction Model with External Validation for Early Detection of Postoperative Pediatric Chylothorax.

Earlier diagnosis of chylothorax following pediatric cardiac surgery is associated with decreased duration of chylothorax. Pleural fluid testing is used to diagnosis chylothorax which may delay detection in patients who are not enterally fed at time of chylothorax onset. Our aim was to develop and externally validate a prediction model to detect chylothorax earlier than pleural fluid testing in pediatric patients following cardiac surgery. A multivariable logistic regression model was developed to detect chylothorax using a stepwise approach. The model was developed using data from patients < 18 years following cardiac surgery from Primary Children's Hospital, a tertiary-care academic center, between 2017 and 2020. External validation used a contemporary cohort (n = 171) from Lucille Packard Children's Hospital. A total of 763 encounters (735 patients) were analyzed, of which 72 had chylothorax. The final variables selected were chest tube output (CTO) the day after sternal closure (dichotomized at 15.6 mL/kg/day, and as a continuous variable) and delayed sternal closure. The highest odds of chylothorax were associated with CTO on post-sternal closure day 1 > 15.6 mL/kg/day (odds ratio 11.3, 95% CI 6,3, 21.3). The c-statistic for the internal and external validation datasets using the dichotomized CTO variable were 0.78 (95% CI 0.73, 0.82) and 0.84 (95% CI, 0.78, 0.9) and performance improved when using CTO as a continuous variable (OR 0.84, CI: 95% CI 0.80, 0.87). Using the models described, chylothorax after pediatric cardiac surgery may be detected earlier and without reliance on enteral feeds.

Histologic features of allograft livers in patients treated for rejection before biopsy.

Liver biopsy is essential for management in liver transplant patients with clinical features suspicious for acute cellular rejection (ACR). As more patients are transplanted for noninfectious indications, it has become increasingly common for them to receive treatment for presumed ACR before biopsy. The effect of pretreatment on the classic histologic triad of ACR's mixed portal inflammation, endothelialitis, and bile duct damage is not well described. Here we report a retrospective study of 70 liver transplant biopsies performed on 53 patients for suspected ACR between 2018 and 2021. Thirty-seven biopsies had a clinical diagnosis of ACR after biopsy. Pretreatment with steroids, antithymocyte globulin, or other increased immunosuppression was given before biopsy in 17 of 37 cases; 20 not-pretreated cases acted as controls. A representative hematoxylin and eosin-stained slide from each biopsy was reviewed independently in a blinded fashion by 3 hepatic pathologists, graded according to the Banff system, assigned a Rejection Activity Index (RAI), and assessed for other histologic features. We found that pretreated biopsies had significantly less portal inflammation (P < .001), less endothelialitis (P < .001), lower RAI (P < .001), and less prominent eosinophils (P = .048) compared to not-pretreated biopsies. There was no significant difference for the other examined variables, including bile duct inflammation/damage (P = .32). Our findings suggest that portal inflammation and endothelialitis become less prominent with pretreatment, whereas bile duct inflammation/damage may take longer to resolve. When evaluating biopsies for suspected ACR, the finding of bile duct inflammation/damage should raise the possibility of partially treated ACR, even in the absence of endothelialitis and portal inflammation.

Evaluation of Preoperative Factors Affecting Midterm Patient-Reported Outcomes Following Ligament Reconstruction Tendon Interposition: A Prognostic Study.

PURPOSE: The aim of this study was to evaluate the prognostic value of preoperative patient-reported outcomes as predictors of functional improvement following ligament reconstruction tendon interposition. We hypothesized that high levels of preoperative pain interference (PI) and upper-extremity disability are associated with lower magnitudes of functional improvement ≥1 year after surgery on the shortened version of the Disabilities of the Arm, Shoulder, and Hand (QuickDASH) (primary outcome) and Patient-Reported Outcomes Measurement Information System Upper Extremity (UE) Computer Adaptive Test (CAT) (UE CAT) v1.2 (secondary outcome). METHODS: Adult patients who underwent ligament reconstruction tendon interposition between February 2014 and April 2018 at an academic tertiary institution were considered for inclusion in this longitudinal cohort study. Patient-reported outcomes were collected at baseline and ≥1 year after surgery. Univariate and multivariable linear regression analyses were performed to identify factors associated with the magnitude of functional improvement on the QuickDASH and UE CAT. RESULTS: Among 93 included participants, the mean age was 61 ± 7 years, and 75 (81%) were women. At 2.5 ± 1.0 years after surgery, the QuickDASH and UE CAT improved by a mean of 24.5 ± 20.9 and 9.9 ± 10.7 points, respectively. In the primary multivariable model, a greater preoperative QuickDASH (indicative of lower function; coefficient, 0.8; 95% confidence interval [CI], 0.6 to 0.9) and lower preoperative Performance of Patient-Reported Outcomes Measurement Information System PI CAT (eg, less pain interference; coefficient, -0.7; 95% CI, -1.2 to -0.2) were associated with greater QuickDASH improvement independent of potential confounders. In the secondary multivariable model, lower preoperative UE CAT (indicative of worse function; coefficient, -0.9; 95% CI, -1.1 to -0.7) and lower preoperative Patient-Reported Outcomes Measurement Information System PI CAT (coefficient, -0.3; 95% CI, -0.6 to -0.1) were associated with greater UE CAT improvement. CONCLUSIONS: Preoperative patient-reported outcomes may be useful in understanding the degree of improvement that certain patient populations can expect from ligament reconstruction tendon interposition. Those with lower baseline (preoperative) upper-extremity function and PI are expected to derive the greatest functional improvement in the midterm. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic IV.

The final hospital need in children discharged from a cardiology acute care unit: a single-centre survey study.

OBJECTIVE: Children with heart disease may require inpatient care for many reasons, but ultimately have a final reason for hospitalisation prior to discharge. Factors influencing length of stay in paediatric cardiac acute care units have been described but the last reason for hospitalisation has not been studied. Our aim was to describe Final Hospital Need as a novel measure, determine Final Hospital Need in our patients, and describe factors associated with this Need. METHODS: Single-centre survey design. Discharging providers selected a Final Hospital Need from the following categories: cardiovascular, respiratory, feeding/fluid, haematology/ID, pain/sedation, systems issues, and other/wound issues. Univariable and multivariable analyses were performed separately for outcomes "cardiovascular" and "feeding/fluid." MEASUREMENTS AND RESULTS: Survey response rate was 99% (624 encounters). The most frequent Final Hospital Needs were cardiovascular (36%), feeding/fluid (24%) and systems issues (13%). Probability of Final Hospital Need "cardiovascular" decreased as length of stay increased. Multivariate analysis showed Final Hospital Need "cardiovascular" was negatively associated with aortic arch repair, Norwood procedure, and Final ICU Need "respiratory" and "other." Final Hospital Need "feeding/fluid" was negatively associated with left-sided valve procedure, but positively associated with final ICU need "respiratory," and tube feeding at discharge. CONCLUSIONS: Final Hospital Need is a novel measure that can be predicted by clinical factors including age, Final ICU Need, and type of surgery. Final Hospital Need may be utilised to track changes in clinical care over time and as a target for improvement work.

Fontan-associated liver disease after heart transplant.

BACKGROUND: Fontan associated liver disease (FALD) potentially impacts Fontan patients undergoing heart transplant. This multi-center study sought to identify pre-transplant risk factors and characterize any post-transplant liver recovery in those patients undergoing heart-alone transplant. METHODS: Review of Fontan patients at 12 pediatric institutions who underwent heart transplant between 2001-2019. Radiologists reviewed pre and post-transplant liver imaging for fibrosis. Laboratory, pathology and endoscopy studies were reviewed. RESULTS: 156 patients underwent transplant due to decreased ventricular function (49%), protein losing enteropathy (31%) or plastic bronchitis (10%); median age at transplant was 13.6 years (interquartile range IQR 7.8, 17.2) with a median of 9.3 years (IQR 3.2, 13.4) between the Fontan operation and transplant. Few patients had pre-transplant endoscopy (18%), and liver biopsy (19%). There were 31 deaths (20%). The median time from transplant to death was 0.5 years (95% Confidence Interval CI 0.0, 3.6). The five-year survival was 73% (95% CI 64%, 83%). Deaths were related to cardiac causes in 68% (21/31) and infection in 6 (19%). A pre-transplant elevation in bilirubin was a predictor of death. Higher platelet levels were protective. Immediate post-transplant elevations in creatinine, AST, ALT, and INR were predictive of death. Advanced liver fibrosis identified on ultrasound, computed tomography, or magnetic resonance imaging was not predictive of death. Liver imaging suggested some improvement in liver congestion post-transplant. CONCLUSIONS: Elevated bilirubin, but not fibrosis on liver imaging, was associated with post-heart transplant mortality in Fontan patients in this multicenter retrospective study. Additionally, heart transplant may alter the progression of FALD.

Impact of a Surgical Wound Infection Prevention Bundle in Pediatric Cardiothoracic Surgery.

BACKGROUND: The aim of this project was to decrease the incidence of surgical wound infection (SWI) to <1.5% in our pediatric cardiothoracic surgery patients using a prevention bundle and quality improvement process. METHODS: An SWI prevention bundle addressing preoperative, intraoperative, and postoperative risks was implemented. The primary outcome was SWI based on Society of Thoracic Surgeons criteria (superficial, deep, or mediastinitis). Novel aspects of the bundle included standardization of surgical closure and wound coverage for 14 days with a negative pressure dressing or a silicone dressing. Data were collected from January 2017 to November 2021; bundle intervention began in December 2019. SWIs were tracked using a g-chart. Preintervention and postintervention cohorts were compared by standard descriptive statistics. There were no changes in SWI tracking methods during the study. RESULTS: During the study, 1159 individuals underwent 1768 surgical interventions. Preintervention (n = 931) and postintervention (n = 837) groups were clinically similar, with fewer neonatal surgeries in the postintervention group. SWI decreased in all patients (preintervention period: 1 SWI per 22 surgeries; postintervention period: 1 SWI per 62.6 surgeries) and in neonates (preintervention period: 1 SWI per 12 surgeries; postintervention period: 1 SWI per 26.7 surgeries). Special cause variation was achieved in the entire cohort by March 2021 and in neonates by April 2021. Decreases in SWI occurred in superficial and deep wounds but not in mediastinitis. Annual rate of total SWIs decreased from 2.83% in 2019 to 1.15% in 2021. Intensive care unit and hospital length of stay did not change. CONCLUSIONS: We demonstrated a reduction in SWI rates after implementing an SWI prevention bundle including standardized surgical closure and prolonged wound protection.

Association of Cam Deformity on Anteroposterior Pelvic Radiographs and More Severe Chondral Damage in Femoroacetabular Impingement Syndrome.

BACKGROUND: Abnormal proximal femoral morphology in femoroacetabular impingement syndrome (FAIS) identifiable on the anteroposterior (AP) pelvic radiograph has been described as the "pistol grip" deformity. The effect of a superior cam deformity identified on this radiographic view remains unknown. PURPOSES: To assess the relationship between AP cam deformity (AP alpha angle [AP AA] >55°) and cartilage injury; to assess the relationship between AP cam deformity and labral injury; and to determine if patient factors are correlated with cartilage and labral injury. STUDY DESIGN: Case-control study; Level of evidence, 3. METHODS: Patients undergoing osteochondroplasty and labral repair for FAIS with a single surgeon were identified retrospectively. Intraoperative size, location, and severity of cartilage and labral damage were prospectively collected. Chondrolabral and femoral/acetabular cartilage damage were graded with the Beck labral and cartilage injury criteria. Radiographic data included the AP AA, lateral center-edge angle (LCEA), and maximum femoral alpha angle (MF AA). Patients with hip dysplasia (LCEA <18°; LCEA 18°-25° with acetabular index >10°) were excluded. Outcomes were compared across cartilage and labral severity grades. We report odds ratios (ORs) with 95% confidence intervals and P values from multivariable logistic regressions. RESULTS: Of the 158 included hips (154 patients), 69% were female and the mean (± standard deviation [SD]) age was 30.3 ± 9.7 years. Increase in age, increase in body mass index (BMI), and male sex were associated with increasing severity of cartilage and labral injury. More severe cartilage damage was associated with increasing AP AA (P < .001) and MF AA (P < .001). The odds of developing a higher category of cartilage injury severity (grade ≥3) were 119% higher (OR, 2.19 [95% CI, 1.36-3.64]; P = .002) for every SD increase (16.7°) in AP AA, adjusting for age, sex, and BMI. The odds of developing a severe labral injury (grade ≥3) were 118% higher (OR, 2.18 [95% CI 1.17-4.32]; P = .018) for every SD increase (10.5°) in MF AA, adjusting for age, sex, BMI, and AP AA. CONCLUSION: Increasing age and BMI, and male sex are associated with increasing severity of cartilage and labral injury in FAIS. The odds of developing a cartilage injury are significantly higher with independent increases in AP AA and MF AA.

Anxiety symptoms and associated functional impairment in children with CHD in a neurodevelopmental follow-up clinic.

OBJECTIVES: To examine the prevalence of anxiety symptoms and associated functional impairment to adaptive skills among elementary-aged children with CHD and to determine the need for anxiety screening in this high-risk population. STUDY DESIGN: In a single-centre retrospective, cohort design, caregivers reported anxiety symptoms using Conner's scales and functional impairment to adaptive skills using the Adaptive Behavior Assessment System. A total of 194 children were stratified across two cohorts: early elementary (ages 3-6 years) and late elementary (ages 6-14 years). Descriptive statistics summarised the frequency of anxiety symptoms and functional impairment. Spearman's correlations compared anxiety symptoms to functional impairment of adaptive functioning. Univariable logistic regressions examined demographic and clinical characteristics associated with anxiety symptoms. RESULTS: The majority of patients presented with anxiety, early elementary (63%), and late elementary cohorts (78%). Functional impairment was moderately correlated with anxiety symptoms in the early elementary cohort (rs = -.42, 95% CI [-0.58, -0.21], p = <.001). Greater anxiety symptoms were associated with lower cardiac complexity at primary age of surgery in the late elementary cohort (OR = 12.15, p = 0.019). Lesser anxiety symptoms were associated with having private insurance (OR = 0.25, p = 0.014). CONCLUSION: This study demonstrates anxiety symptoms are common and associated with functional impairment to adaptive functioning in younger children with CHD. No clear clinical predictors exist for anxiety symptoms or functional impairment; therefore, screening for anxiety symptoms may need to be added to standard clinical assessment of all children with CHD participating in neurodevelopmental follow-up.

Relation of Right Atrial Strain to Mortality in Infants With Single Right Ventricles.

We explored associations of surveillance testing in infants with single right ventricle (sRV) physiology with clinical outcomes. This prospective, single-center study included patients with sRV who had initial palliative surgery (September 2019 to December 2020). Echocardiograms and B-type naturetic peptide (BNP) obtained as a pair within 24 hours as part of clinical care were included. The primary outcome was death/heart transplant. Secondary outcomes included interstage duration of milrinone use, hospital length of stay, and no digoxin use. sRV functional assessment (subjective grade, fractional area change, tricuspid annular plane systolic excursion, global longitudinal strain, right atrial strain [RAS]) was performed offline. Associations between echocardiography, BNP, and clinical outcomes were determined. Of 26 subjects (47 encounters), 20 had hypoplastic left heart syndrome (77%). Median age at data collection was 50 days (interquartile range 26 to 90). In most encounters (73%), sRV function was subjectively normal. Median BNP was 332 pg/ml (interquartile range 160 to 1,085). A total of 5 patients (19%) met the primary outcome and had lower RAS (14.1 vs 21.3, p = 0.038), but all other parameters were similar to transplant-free survivors. RAS (16.1%, 0.83) had the highest area under curve, followed by global longitudinal strain (-14.4%, 0.77). Higher RAS was associated with fewer days on milrinone (coefficient -1.37, 95% confidence interval [CI] -2.54 to -0.20, p = 0.02) and higher odds of digoxin use (odds ratio 1.09, 95% CI 1.01 to 1.18, p = 0.047). Higher BNP was only associated with a lower odds of digoxin use (odds ratio 0.69, 95% CI 0.5 to 0.96, p = 0.03). In conclusion, RAS is a potentially important imaging marker in infants with sRV and merits further investigation in larger studies.

Social Needs Screening During Pediatric Emergency Department Visits: Disparities in Unmet Social Needs.

OBJECTIVE: To determine the prevalence of unmet social needs (USN) in a pediatric emergency department (PED) patient population and examine disparities in USN by self-selected language and patient demographics. METHODS: We surveyed a convenience sample of English- and Spanish-speaking caregivers of patients <18-years-old presenting to a free-standing children's hospital in Salt Lake City, Utah. In the caregiver's self-selected language, the pediatric version of the Screener for Intensifying Community Referrals for Health (p-SINCERE) assessed patient demographics and 10 areas of social needs. The primary outcome was presence of USN. Descriptive statistics compared 1) self-selected languages and 2) absence versus presence of USN. Patient and caregiver-level risk factors associated with USN were identified using multivariable logistic regression. RESULTS: Of the 10,156 patients seen in our PED from 04/01/2021 to 08/03/2021, there were 9922 eligible, 5357 approached, and 3987 enrolled caregivers. Of the 3987 caregivers enrolled, self-selected language was English for 3662 (91.8%) and Spanish for 325 (8.2%). There were 1680 enrolled caregivers with ≥1 USN, representing 39.7% of English-speaking and 70.2% of Spanish-speaking caregivers (P < .001). The odds of having ≥1 USN was more than 2 times higher in Spanish-speakers than in English-speakers after adjustment. CONCLUSIONS: USN are common for families presenting for care to a PED, especially among Spanish-speaking caregivers. Furthermore, this study demonstrates disparities in limited English proficiency, race and ethnicity, and child insurance status. These findings support the practicality of utilizing the PED as an access point to initiate social need screening and referrals to address social determinants of health and health disparities.

Assessing the Impact of Nasotracheal Intubation on Postoperative Neonates With Congenital Heart Disease: A Quality Improvement Project at a Single Heart Center.

OBJECTIVES: Nasotracheal intubation (NTI) is associated with fewer unplanned extubations and improved oral motor skills compared with orotracheal intubation (OTI). Our study aimed to implement a practice change from OTI to NTI for neonatal cardiac surgery and assess impact on postoperative outcomes. DESIGN: Single-center, prospective, quality improvement study. SETTING: Academic children's hospital. PATIENTS: One hundred thirty neonates undergoing cardiac surgery with cardiopulmonary bypass. INTERVENTIONS: NTI during index cardiac surgery. MEASUREMENTS AND MAIN RESULTS: Data were collected between January 2019 and April 2021. The study was implemented in three phases: retrospective: OTI neonates ( n = 43), I: safety and feasibility of NTI ( n = 17), and II: speech language pathology (SLP) evaluation on postoperative day 1 facilitated by NTI ( n = 70). Retrospective and phase I patients were combined for analysis. Groups were compared using Kruskal-Wallis test or Wilcoxon rank-sum test.Ninety-one percent of eligible neonates were nasotracheally intubated. There were no clinically significant complications. Days to first SLP encounter decreased from a median 4.5 days (interquartile range [IQR], 3.8-6.2) to 1.1 days (IQR, 1.0-1.9; p < 0.001). Oral readiness time decreased from a median of 6.6 days (IQR, 5.4-8.9) to 4.3 days (IQR, 3.4-8.6; p < 0.001). . CONCLUSIONS: NTI is feasible and safe in neonatal cardiac surgery. System-level engagement with stakeholders is necessary to change clinical practice. NTI facilitates early SLP evaluation and treatment and significantly affects oral readiness after neonatal cardiac surgery.

The association of modifiable mechanical ventilation settings, blood gas changes and survival on extracorporeal membrane oxygenation for cardiac arrest.

RESEARCH QUESTION: Given the relative independence of ventilator settings from gas exchange and plasticity of blood gas values during extracorporeal cardiopulmonary resuscitation (ECPR), do mechanical ventilation parameters and blood gas values influence survival? METHODS: Observational cohort study of 7488 adult patients with ECPR from the Extracorporeal Life Support Organization (ELSO) Registry. We performed case-mix adjustment for severity of illness and patient type using generalized estimating equation logistic regression to determine factors associated with hospital survival accounting for clustering by center, standardizing variables by 1 standard deviation (SD) of their values. We examined non-linear relationships between ventilatory and blood gas values with hospital survival. RESULTS: Hospital survival was decreased with higher PaO2 on ECMO (OR 0.69, per 1SD increase [95% CI 0.64, 0.74]; p < 0.001) and with any relative changes in PaCO2 (pre-arrest to on-ECMO) in a non-linear fashion. Survival was worsened with any peak inspiratory pressure >20 cmH20 (OR 0.69, per 1SD [0.64, 0.75]; p < 0.001) and above 40% fraction of inspired oxygen (OR 0.75, per 1SD [0.69, 0.82]; p < 0.001), and with higher dynamic driving pressure (OR 0.72, per 1 SD increase [0.65, 0.79]; <0.001). Ventilation settings and blood gas values varied widely across hospitals, but were not associated with annual hospital ECPR case volume. CONCLUSION: Lower ventilatory pressures, avoidance of hyperoxia, and relatively unchanged CO2 (pre- to on-ECMO) were all associated with survival in patients after ECPR, yet varied across hospitals. Our findings represent potential targets for prospective trials for this rapidly growing therapy to test if these associations have causality.

Physiology of Dysphagia in Those with Unilateral Vocal Fold Immobility.

Quantitative measures of swallowing function were extracted from modified barium swallowing studies (MBS) to characterize swallowing pathophysiology in patients with unilateral vocal fold immobility (UVFI). All individuals with UVFI completing a MBS during the prior 5 years were included. Demographic information, penetration-aspiration score, timing of aspiration and quantitative measures from the MBS were extracted from electronic medical records and compared across 1, 3, and 20 cc liquid bolus swallows. UVFI patient measures were compared to normal age-matched controls to identify swallowing pathophysiology associated with aspiration. The incidence of aspiration by UVFI etiology groups (i.e., central nervous system, idiopathic, iatrogenic, skull base tumor, or peripheral tumor) was also compared. Of the 61 patients who met inclusion criteria, aspiration was observed in 23%. Maximum pharyngeal constriction was abnormal in 79% of aspirators compared to 34% of non-aspirators (p = .003). Delay in airway closure was the most common swallowing abnormality identified in the study population (62%) but was not associated with aspiration. Among the 14 individuals who aspirated, the iatrogenic and skull base tumor etiology groups comprised the majority (i.e., 36% each). However, the incidence of aspiration for the iatrogenic group was 19% compared to 50% of the skull base tumor group. Aspiration in patients with UVFI was associated with abnormally reduced pharyngeal constriction. Delayed airway closure was common in both aspirators and non-aspirators.

Feeding outcomes in post-discharge feeding clinic for infants following cardiac surgery.

INTRODUCTION: The aim of this study was to describe the development and assess the usefulness of a feeding clinic to help infants with CHD tolerate the highest level of oral feeding while achieving growth velocity and supporting neurodevelopment. MATERIALS AND METHODS: This retrospective, cohort study assessed feeding outcomes for infants who underwent cardiac surgery at <30 days of age with cardiopulmonary bypass between February 2016 and April 2020. Diagnoses, age at surgery, hospitalisation variables, and feeding outcomes were compared between two cohorts, pre- and post-implementation of a specialised feeding clinic using Exact Wilcoxon signed-rank test, chi-squared, or Fisher's exact test. The association between time to full oral feed and risk factors was assessed using univariable and multivariable Cox regression model. RESULTS: Post-clinic infants (n = 116) surgery was performed at a median of 6 days of life (interquartile range: 4, 8) with median hospital length of stay of 19 days (interquartile range: 16, 26). Infants' median age at first clinic visit was at 30 days old (interquartile range: 24, 40) and took median 10 days (interquartile range: 7, 12) after hospital discharge to first clinic visit. In the post-clinic cohort, the median time to 100% oral feeding was 47 days (interquartile range: 27, 96) compared to the 60 days (interquartile range: 20, 84) in the pre-clinic cohort (n = 22), but the difference was not statistically significant. DISCUSSION: The cardiac feeding clinic was utilised by our neonatal surgery population and feasible in coordination with cardiology follow-up visits. Future assessment of cardiac feeding clinic impact should include additional measures of feeding and neurodevelopmental success.

Poor Weight Recovery Between Stage 1 Palliation and Hospital Discharge for Infants with Single Ventricle Physiology: An Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Phase II Dataset.

OBJECTIVE: To investigate change in weight-for-age z-scores (WAZ) and risk factors for impaired weight gain between stage 1 palliation (S1P) for single ventricle physiology and discharge. STUDY DESIGN: This was a secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative Phase II database. The primary outcome was change in WAZ between S1P and discharge. Risk factors were selected using multivariable mixed effects regression constructed by step-wise model selection, with adjustment for WAZ at S1P and a random effect for center. RESULTS: Of 730 infants who were discharged after S1P, WAZ decreased in 98.6% (-1.5 ± 0.7). WAZ at discharge was <-1 but >-2 (at risk) in 40% and <-2 (failure to thrive) in 35% of participants. Males, higher WAZ at S1P, non-S1P procedures (mostly noncardiac), increased length of stay, necrotizing enterocolitis, and angiotensin-converting enzyme inhibitor use at discharge were associated with a greater decrease in WAZ. Preoperative enteral feeding and respiratory medications were associated with a lesser decrease in the WAZ. CONCLUSIONS: Nearly all infants lose weight after S1P with little recovery by hospital discharge. At discharge, three-quarters of the infants in the cohort were at risk for impaired weight gain or had failure to thrive. Most risk factors associated with change in WAZ were unmodifiable or surrogates of disease severity. Novel interventions are needed to minimize the early catabolic effects and promote anabolic recovery after S1P.