Familial Associations of Prevalence and Cause-Specific Mortality for Thoracic Aortic Disease and Bicuspid Aortic Valve in a Large-Population Database.

BACKGROUND: Thoracic aortic disease and bicuspid aortic valve (BAV) likely have a heritable component, but large population-based studies are lacking. This study characterizes familial associations of thoracic aortic disease and BAV, as well as cardiovascular and aortic-specific mortality, among relatives of these individuals in a large-population database. METHODS: In this observational case-control study of the Utah Population Database, we identified probands with a diagnosis of BAV, thoracic aortic aneurysm, or thoracic aortic dissection. Age- and sex-matched controls (10:1 ratio) were identified for each proband. First-degree relatives, second-degree relatives, and first cousins of probands and controls were identified through linked genealogical information. Cox proportional hazard models were used to quantify the familial associations for each diagnosis. We used a competing-risk model to determine the risk of cardiovascular-specific and aortic-specific mortality for relatives of probands. RESULTS: The study population included 3 812 588 unique individuals. Familial hazard risk of a concordant diagnosis was elevated in the following populations compared with controls: first-degree relatives of patients with BAV (hazard ratio [HR], 6.88 [95% CI, 5.62-8.43]); first-degree relatives of patients with thoracic aortic aneurysm (HR, 5.09 [95% CI, 3.80-6.82]); and first-degree relatives of patients with thoracic aortic dissection (HR, 4.15 [95% CI, 3.25-5.31]). In addition, the risk of aortic dissection was higher in first-degree relatives of patients with BAV (HR, 3.63 [95% CI, 2.68-4.91]) and in first-degree relatives of patients with thoracic aneurysm (HR, 3.89 [95% CI, 2.93-5.18]) compared with controls. Dissection risk was highest in first-degree relatives of patients who carried a diagnosis of both BAV and aneurysm (HR, 6.13 [95% CI, 2.82-13.33]). First-degree relatives of patients with BAV, thoracic aneurysm, or aortic dissection had a higher risk of aortic-specific mortality (HR, 2.83 [95% CI, 2.44-3.29]) compared with controls. CONCLUSIONS: Our results indicate that BAV and thoracic aortic disease carry a significant familial association for concordant disease and aortic dissection. The pattern of familiality is consistent with a genetic cause of disease. Furthermore, we observed higher risk of aortic-specific mortality in relatives of individuals with these diagnoses. This study provides supportive evidence for screening in relatives of patients with BAV, thoracic aneurysm, or dissection.

Longitudinal Fluctuations in Treatment Response After OnabotulinumToxinA and Sacral Neuromodulation for Refractory Urgency Incontinence.

PURPOSE: We compared fluctuations in treatment response after onabotulinumtoxinA and sacral neuromodulation for urgency incontinence using Markov models. MATERIALS AND METHODS: We fit data from a randomized trial to Markov models to compare transitions of success/failure over 6 months between 200 U onabotulinumtoxinA and sacral neuromodulation. Objective failure was <50% reduction in urgency incontinence episodes from baseline; subjective failure "strongly disagree" to "neutral" to the Patient Global Symptom Control questionnaire. RESULTS: Of the 357 participants (median baseline daily urgency incontinence episodes 4.7 [IQR 3.7-6.0]) 61% vs 51% and 3.2% vs 6.1% reported persistent states of objective success and failure over 6 months after onabotulinumtoxinA vs sacral neuromodulation. Participants receiving onabotulinumtoxinA vs sacral neuromodulation had lower 30-day transition probabilities from objective and subjective success to failure (10% vs 14%, ratio 0.75 [95% CI 0.55-0.95]; 14% vs 21%, ratio 0.70 [95% CI 0.51-0.89]). The 30-day transition probability from objective and subjective failure to success did not differ between onabotulinumtoxinA and sacral neuromodulation (40% vs 36%, ratio 1.11 [95% CI 0.73-1.50]; 18% vs 17%, ratio 1.14 [95% CI 0.65-1.64]). CONCLUSIONS: Over 6 months after treatment, 2 in 5 women's symptoms fluctuate. Within these initial 6 months, women receiving onabotulinumtoxinA transitioned from success to failure over 30 days less often than sacral neuromodulation. For both treatments, there was an almost 20%-40% probability over 30 days that women returned to subjective and objective success after failure. Markov models add important information to longitudinal models on how symptoms fluctuate after urgency incontinence treatment.

Impact of Social Risk Screening on Discharge Care Processes and Postdischarge Outcomes: A Pragmatic Mixed-Methods Clinical Trial During the COVID-19 Pandemic.

BACKGROUND: Social risk screening during inpatient care is required in new CMS regulations, yet its impact on inpatient care and patient outcomes is unknown. OBJECTIVES: To evaluate whether implementing a social risk screening protocol improves discharge processes, patient-reported outcomes, and 30-day service use. RESEARCH DESIGN: Pragmatic mixed-methods clinical trial. SUBJECTS: Overall, 4130 patient discharges (2383 preimplementation and 1747 postimplementation) from general medicine and surgical services at a 528-bed academic medical center in the Intermountain United States and 15 attending physicians. MEASURES: Documented family interaction, late discharge, patient-reported readiness for hospital discharge and postdischarge coping difficulties, readmission and emergency department visits within 30 days postdischarge, and coded interviews with inpatient physicians. RESULTS: A multivariable segmented regression model indicated a 19% decrease per month in odds of family interaction following intervention implementation (OR=0.81, 95% CI=0.76-0.86, P<0.001), and an additional model found a 32% decrease in odds of being discharged after 2 pm (OR=0.68, 95% CI=0.53-0.87, P=0.003). There were no postimplementation changes in patient-reported discharge readiness, postdischarge coping difficulties, or 30-day hospital readmissions, or ED visits. Physicians expressed concerns about the appropriateness, acceptability, and feasibility of the structured social risk assessment. CONCLUSIONS: Conducted in the immediate post-COVID timeframe, reduction in family interaction, earlier discharge, and provider concerns with structured social risk assessments likely contributed to the lack of intervention impact on patient outcomes. To be effective, social risk screening will require patient/family and care team codesign its structure and processes, and allocation of resources to assist in addressing identified social risk needs.

Management of undifferentiated embryonal sarcoma of the liver: A Pediatric Surgical Oncology Research Collaborative study.

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare tumor for which there are few evidence-based guidelines. The aim of this study was to define current management strategies and outcomes for these patients using a multi-institutional dataset curated by the Pediatric Surgical Oncology Research Collaborative. METHODS: Data were collected retrospectively for patients with UESL treated across 17 children's hospitals in North America from 1989 to 2019. Factors analyzed included patient and tumor characteristics, PRETEXT group, operative details, and neoadjuvant/adjuvant regimens. Event-free and overall survival (EFS, OS) were the primary and secondary outcomes, respectively. RESULTS: Seventy-eight patients were identified with a median age of 9.9 years [interquartile range [IQR): 7-12]. Twenty-seven patients underwent resection at diagnosis, and 47 patients underwent delayed resection, including eight liver transplants. Neoadjuvant chemotherapy led to a median change in maximum tumor diameter of 1.6 cm [IQR: 0.0-4.4] and greater than 90% tumor necrosis in 79% of the patients undergoing delayed resection. R0 resections were accomplished in 63 patients (81%). Univariate analysis found that metastatic disease impacted OS, and completeness of resection impacted both EFS and OS, while multivariate analysis revealed that R0 resection was associated with decreased expected hazards of experiencing an event [hazard ratio (HR): 0.14, 95% confidence interval (CI): 0.04-0.6]. At a median follow-up of 4 years [IQR: 2-8], the EFS was 70.0% [95% CI: 60%-82%] and OS was 83% [95% CI: 75%-93%]. CONCLUSION: Complete resection is associated with improved survival for patients with UESL. Neoadjuvant chemotherapy causes minimal radiographic response, but significant tumor necrosis.

Predicting success using response after lead implantation with sacral neuromodulation for urgency incontinence.

IMPORTANCE: Many women report inadequate symptom control after sacral neuromodulation (SNM), despite 50% reduction in urgency incontinence episodes (UUIE) after test stimulation. OBJECTIVE: To determine the ideal percent UUIE reduction after test stimulation that predicts 24-month success. STUDY DESIGN: Using data from a multicenter SNM trial, we constructed receiver operating characteristic curves to identify an ideal threshold of percent UUIE reduction after test stimulation. We defined 24-month success as Patient Global Impression of Improvement of "very much better" to "better." We compared predictive accuracy of two models predicting success: (1) percent UUIE reduction alone and (2) with baseline characteristics. RESULTS: Of 149 women (median [IQR] baseline daily UUIE 4.7 [3.7, 6.0]), the ideal threshold for 24-month success was 72% (95% confidence interval 64,76%) UUIE reduction with accuracy 0.54 (0.42, 0.66), sensitivity 0.71 (0.56, 0.86) and specificity 0.27 (0.05, 0.55). The accuracy of the 50% reduction threshold was 0.60 (0.49, 0.71), sensitivity 0.95 (0.88, 1.0) and specificity 0.04 (0.0, 0.12). Percent reduction in UUIE was not better than chance in predicting 24-month success (concordance index [c-index] 0.47 [0.46, 0.62]); adding age, body mass index, diabetes mellitus and visual or hearing impairment the c-index was 0.68 (0.61, 0.78). CONCLUSIONS: Among women who received an internal pulse generator (IPG) due to ≥50% UUIE reduction after test stimulation, we found no ideal threshold that better predicted 24-month success. Percent reduction in UUIE after test stimulation poorly predicts 24-month success with or without clinical factors. Given this, re-evaluating how we determine who should receive an IPG is needed.

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.

Tympanomastoidectomy versus parenteral antibiotic therapy for pediatric otorrhea.

OBJECTIVE: To evaluate the efficacy of tympanomastoidectomy versus parenteral antibiotic therapy for otorrhea as a result of chronic suppurative otitis media (CSOM) without cholesteatoma in the pediatric population. METHODS: A retrospective review of 221 patients treated for otorrhea at a tertiary academic pediatric hospital was performed to evaluate the impact of tympanomastoidectomy versus parenteral antibiotic therapy on resolution of otorrhea. Inclusion criteria were age 0-18 years, prior treatment with otic and/or oral antibiotic, prior history of tympanostomy tube placement for recurrent otitis media, history of otorrhea, treatment with tympanomastoidectomy or parenteral antibiotic therapy, and follow-up of at least 1 month after intervention. Time to resolution was compared between the two modalities adjusting for age, bilateral ear disease status, and comorbidities using a Cox proportional hazard model. RESULTS: Eighty-three ears from 58 children met the inclusion criteria. Ears that initially underwent tympanomastoidectomy had a significantly shorter time to resolution of symptoms (median time to resolution) 9 months (95 % confidence interval CI: 6.2-14.8) vs. 48.5 months (95 % lower CI 9.4, p = 0.006). On multivariate analysis, however, only bilateral ear disease status was independently associated with time to resolution of symptoms (hazard ratio 0.4, 95 % CI 0.2-0.9, p = 0.03). There was no statistically significant difference in the rate of treatment-related complications when comparing tympanomastoidectomy to parenteral antibiotic therapy (p = 0.37). CONCLUSION: When adjusting for age, bilateral ear disease status, and comorbidities, there does not appear to be a significant difference in time to resolution of symptoms when comparing parenteral antibiotic therapy to tympanomastoidectomy. An informed discussion regarding risks and benefits of each approach should be employed when deciding on the next step in management for patients with CSOM who have failed more conservative therapies.

Linking QuickDASH and PROMIS Upper-Extremity Computer-Adaptive Test Scores in Hand Surgery: A Crosswalk Study.

PURPOSE: Assessment of patient-reported outcome measures (PROMs) for hand and upper-extremity surgery patients using measures such as the Quick Disabilities of the Arm, Shoulder, and Hand (qDASH), as well as general measures including the Patient-Reported Outcomes Measurement Information System Upper Extremity Physical Function domain via a Computer-Adaptive Test (PROMIS UE CAT), has become commonplace. The aim of this study was to link, for crosswalking, the qDASH measure to both versions of the PROMIS UE CAT (v1.2 and v2.0). METHODS: We included 18,944 hand and upper-extremity patients who completed both versions of the PROMIS UE CAT and the qDASH at the same clinical encounter. Shoulder pathology was excluded. Score linkage was performed using the R package equate, and multiple equating models (linear regression, identity, mean, linear, equipercentile, and circle-arc models) were used to establish crosswalk tables. RESULTS: Mean qDASH and PROMIS UE CAT v1.2 scores were 38.2 (SD = 23.1) and 36.6 (SD = 9.8), respectively. Mean qDASH and PROMIS UE CAT v2.0 scores were 37.3 (SD = 21.8) and 38.3 (SD = 10.4), respectively. Pearson correlations had very strong linear relationships between the qDASH and the PROMIS UE CAT v1.2 and PROMIS UE CAT v2.0 (r = -0.83 [-0.84, -0.92] and r = -0.80 [-0.81, -0.80], respectively). For the equipercentile equating models, the intraclass correlation coefficient (ICC) had very strong positive relationships to linking measures with ICC = 0.85 (0.84, 0.86) for the qDASH-UE CAT v1.2 crosswalk and ICC = 0.83 (0.82, 0.84) for the qDASH-UE CAT v2.0 crosswalk. CONCLUSIONS: The linkages establish crosswalk tables using equipercentile equating models to convert the PROMIS UE CAT v1.2 and v2.0 scores to the qDASH and vice versa. CLINICAL RELEVANCE: This study provides crosswalk tables for commonly collected PROMs in hand surgery, increasing the comparability of results between centers using different PROMs to study the same conditions or treatments.

Establishing the Patient-Acceptable Symptom State for the Numeric Rating Scale-Pain Score in a Postoperative Non-Shoulder Hand and Upper-Extremity Population.

PURPOSE: The patient-acceptable symptom state (PASS) is a threshold score on a patient-reported outcome measurement beyond which patients consider themselves "well." Our purpose was to establish the PASS for the numeric rating scale (NRS) for pain in a 1-year postoperative hand surgery population. METHODS: This retrospective study included adult patients undergoing non-shoulder upper-extremity surgery at a single, tertiary medical center identified over a 9-month period. At 1 year after surgery, NRS pain and responses to a pain-specific anchor question were collected. Patients were dichotomized based on achieving "PASS(+)" or failing to achieve "PASS(-)" an acceptable symptom state based upon their response to a pain-specific anchor question. Threshold values of a PASS(+) state were calculated for the NRS pain score using the following three methods: (1) mean score of PASS(+) patients, (2) Tubach method (75th percentile threshold for PASS(+) patients), and (3) the Youden index (receiver operating curve analysis to maximize sensitivity and specificity). RESULTS: Of 233 included patients, mean age was 54 years (±17), and 58% (n = 136) were women. Mean NRS pain scores differed between PASS(+) and PASS(-) patients (0.7 ± 1.2 vs 3.8 ± 2.7, respectively). PASS(-) patients were more likely to be non-White and have a diagnosed psychiatric comorbidity. Patient-acceptable symptom state estimates ranged from 0.73 to 2.1 for NRS pain, depending on the calculation method (0.73 for the mean score method, 1.0 for the Tubach method, and 2.1 for the Youden index). The area under the curve for the Youden index method was 0.86 consistent with excellent discrimination. CONCLUSIONS: We propose the value of 2.1 to represent the PASS threshold for the NRS pain score in this population. CLINICAL RELEVANCE: This PASS value should be used when interpreting NRS pain score outcomes at a population level. This threshold is expected to yield excellent discrimination for patient satisfaction when applied to a postoperative hand surgery population.

The effect of lateralization and distalization after Grammont-style reverse total shoulder arthroplasty.

BACKGROUND: The purpose of this study was to evaluate the relationship between multiple radiographic measures of lateralization and distalization and clinical outcome scores after a reverse total shoulder arthroplasty (RTSA). METHODS: We retrospectively evaluated all RTSAs performed by the senior author between January 1, 2007, and November 1, 2017. We then evaluated the visual analog scale for pain (VAS pain), Simple Shoulder Test (SST), and American Shoulder and Elbow Surgeons (ASES) scores and complication and reoperation rates at a minimum of 2-year follow-up. We measured preoperative and postoperative (2-week) radiographs for the lateralization shoulder angle (LSA), the distalization shoulder angle (DSA), lateral humeral offset, and the distance from the glenoid to the lateral aspect of the greater tuberosity. A multivariable analysis was performed to evaluate the effect of the postoperative radiographic measurements on final patient-reported outcomes (ASES scores, SST, and VAS pain). RESULTS: The cohort included 216 shoulders from unique patients who had patient-reported outcome scores available at a minimum of 2-year follow-up (average, 4.0 ± 1.9 years) for a total follow-up rate of 70%. In the multivariable models, more lateralization (LSA) was associated with worse final ASES scores -0.52 (95% confidence interval [CI]: -0.88, -0.17; P = .004), and more distalization (DSA) was associated with better final ASES scores 0.40 (95% CI: 0.11, 0.69; P = .007). More lateralization (LSA) was associated with worse final SST scores -0.06 (95% CI: -0.11, -0.003; P = .039). Finally, greater distalization (DSA) was associated with lower final VAS pain scores, ratio = 0.98 (95% CI: 0.96, 1.00; P = .021). CONCLUSIONS: Greater distalization and less lateralization are associated with better function and less pain after a Grammont-style RTSA. When using a Grammont-style implant, remaining consistent with Grammont's principles of implant placement will afford better final clinical outcomes.

Screw Diameter and Use of a Washer Affect Symptomatic Implant Removal Rates in Pediatric Medial Epicondyle Fractures.

OBJECTIVE: Medial epicondyle fractures are a common pediatric injury. When operative, cannulated partially threaded screws, with or without a washer, are commonly utilized. These implants may need to be removed after full healing if symptomatic. There is mixed evidence regarding the influence of a washer on rates of implant removal, and the influence of screw size has not been studied. We aim to determine the rate of symptomatic deep implant removal for each fixation type and identify factors associated with the need for removal. METHODS: This was an IRB-reviewed, retrospective, case-cohort study. Patients treated at our institution between January 1, 2004 and December 31, 2019, age 18 years old or younger, with a medial epicondyle fracture managed operatively with 4.0 or 4.5 mm cannulated screws with or without washers were included. Patients with multiple operative ipsilateral elbow fractures and those who underwent implant removal for reasons other than pain or irritation were excluded. Removal rates were compared between screw sizes (4.0 vs 4.5 mm), as well as with and without a washer using a Cox proportional hazards model. RESULTS: In total, 151 patients met the inclusion criteria, 54 with symptomatic hardware removed and 97 without symptomatic hardware removed. A significantly higher number of patients treated with 4.5 mm screws compared with 4.0 mm screws underwent removal of symptomatic deep implants (50% vs 30%, P = 0.033). In a multivariable Cox regression model adjusting for age and ulnar nerve status, when no washer was used, the hazard ratio (HR) for symptomatic hardware removal for 4.5 mm screws was 2.92 times the HR for 4.0 mm screws (95% CI: 1.35-6.29). When a 4.0 mm screw was used, the HR for symptomatic hardware removal for a washer was 3.24 times the HR without a washer (95% CI: 1.53-6.84). CONCLUSION: Implant removal rates are influenced by screw size and the use of a washer. These results may help guide implant choice and counsel families regarding the rate of symptomatic implant removal. LEVEL OF EVIDENCE: Level III-therapeutic level, case-control study.

Sensory hypersensitivities are associated with post-traumatic headache-related disability.

OBJECTIVE: To examine whether sensory hypersensitivity contributes to headache-related disability in a secondary analysis of patients with post-traumatic headache. BACKGROUND: Up to one-third of individuals with traumatic brain injuries report persistent headache 3 months post-injury. High rates of allodynia and photophobia have been observed in clinical studies and animal models of post-traumatic headache, but we do not fully understand how sensory amplifications impact post-traumatic headache-related disability. METHODS: We identified a cross-sectional sample of patients from the American Registry for Migraine Research database with new or worsening headaches post-head injury from 2016 to 2020 and performed a secondary analysis of those data. We modeled the relationship between sensory sensitivity and Migraine Disability Assessment scores using questionnaires. Candidate variables included data collection features (study site and year), headache-related and general clinical features (headache frequency, migraine diagnosis, abuse history, sex, age, cognitive and affective symptom scores), and sensory symptoms (related to light, sound, and touch sensitivity). RESULTS: The final sample included 193 patients (median age 46, IQR 22; 161/193, 83.4% female). Migraine Disability Assessment scores ranged from 0 to 260 (median 47, IQR 87). The final model included allodynia, hyperacusis, photosensitivity, headache days per month, abuse history, anxiety and depression, cognitive dysfunction, and age (R2 = 0.43). An increase of one point in allodynia score corresponded to a 3% increase in headache disability (95% CI: 0%-7%; p = 0.027), an increase of one-tenth of a point in the photosensitivity score corresponded to a 12% increase (95% CI: 3%-25%; p = 0.002), and an increase of one point in the hyperacusis score corresponded to a 2% increase (95% CI: 0%-4%; p = 0.016). CONCLUSIONS: Increased photosensitivity, allodynia, and hyperacusis were associated with increased headache-related disability in this sample of patients with post-traumatic headache. Symptoms of sensory amplification likely contribute to post-traumatic headache-related disability and merit an ongoing investigation into their potential as disease markers and treatment targets.

Validation of the Utah Photophobia Symptom Impact Scale (version 2) as a headache-specific photophobia assessment tool.

OBJECTIVE: To present an updated version of the Utah Photophobia Symptom Impact Scale version 2 (UPSIS2), providing robust clinical and psychometric validation, to improve headache-specific evaluation of light sensitivity and headache-related photophobia. BACKGROUND: The original UPSIS filled a gap in available tools for assessment of headache-associated light sensitivity by providing patient-reported evaluation of the impact of light sensitivity on activities of daily living (ADLs). We have since revised the original questionnaire to provide a more robust item construct and refined validation approach. METHODS: We conducted a psychometric validation of the UPSIS2 through a primary analysis of an online survey of volunteers with recurrent headaches recruited from the University of Utah clinics and surrounding community. Volunteers completed the original UPSIS and UPSIS2 questionnaire versions in addition to measures of headache impact, disability, and frequency. The UPSIS2 now includes a pre-defined recall period and a 1-4 Likert scale with standardized response anchors to improve clarity. Internal construct validity, external construct validity, and test-retest reliability, were evaluated. RESULTS: Responses were obtained from 163 volunteers, with UPSIS2 scores ranging from 15 to 57 (out of a possible 15-60) with a mean (standard deviation) of 32.4 (8.80). Construct validity was satisfactory, as evidenced by sufficient unidimensionality, monotonicity, and local independence. Reliability was excellent, with Rasch test reliability = 0.90 and Cronbach's alpha = 0.92, and an intraclass correlation of 0.79 (95% confidence interval 0.65-0.88) for participants who took the test twice. UPSIS2 correlates well with other headache measures (Spearman's correlations >0.50), as well as the original UPSIS (Spearman's correlation = 0.87), indicating good convergent validity. UPSIS2 scores differ significantly across International Classification of Headache Disorders (third edition) groups, indicating good known group validity. CONCLUSION: The UPSIS2 provides a well-validated headache-specific outcome measure for the assessment of photophobia impact on ADLs.

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.

Thermoregulation-Focused Implementation of Delayed Cord Clamping among <34 Weeks' Gestational Age Neonates.

OBJECTIVE: Delayed cord clamping (DCC) is recommended for all neonates; however, adapting such practice can be slow or unsustainable, especially among preterm neonates. During DCC neonates are exposed to a cool environment, raising concerns for neonatal hypothermia. Moderate hypothermia may induce morbidities that counteract the potential benefits of DCC. A quality improvement project on a thermoregulation-focused DCC protocol was implemented for neonates less than 34 weeks' gestational age (GA). The aim was to increase the compliance rate of DCC while maintaining normothermia. STUDY DESIGN: The DCC protocol was implemented on October 1, 2020 in a large Level III neonatal intensive care unit. The thermoregulation measures included increasing delivery room temperature and using heat conservation supplies (sterile polyethylene suit, warm towels, and thermal pads). Baseline characteristics, the compliance rate of DCC, and admission temperatures were compared 4 months' preimplementation and 26 months' postimplementation RESULTS: The rate of DCC increased from 20% (11/54) in preimplementation to 57% (240/425) in postimplementation (p < 0.001). The balancing measure of admission normothermia remained unchanged. In a postimplementation subgroup analysis, the DCC cohort had less tendency to experience admission moderate hypothermia (<36°C; 9.2 vs. 14.1%, p = 0.11). The DCC cohort had more favorable secondary outcomes including higher admission hematocrit, less blood transfusions, less intraventricular hemorrhage, and lower mortality. Improving the process measure of accurate documentation could help to identify implementation barriers. CONCLUSION: Performing DCC in preterm neonates was feasible and beneficial without increasing admission hypothermia. KEY POINTS: · Thermoregulation-focused DCC protocol was implemented to increase DCC while maintaining normothermia.. · DCC rate increased from 20 to 57% while admission normothermia rate remained the same.. · DCC practice on preterm neonates is safe and feasible while maintaining normothermia..

Abnormal infant neurobehavior and later neurodevelopmental delays in children with critical CHD.

Infants with critical CHD have abnormal neurobehavior assessed by the Neonatal ICU Network Neurobehavioral Scales. This retrospective cohort study hypothesized associations between abnormal infant neurobehavior in the first month of life and later neurodevelopmental outcomes at 1-2 years of age. Associations between abnormal infant attention (orienting to and tracking stimuli) on the Neonatal ICU Network Neurobehavioral Scales and later motor, cognitive, and language neurodevelopmental outcomes on the Bayley Scales of Infant Development-III at follow-up were examined with descriptive statistics and univariable and multivariable regression. Multiple imputation was used to account for missing outcome data. 189 infants with critical CHD were included, and 69% had abnormal neurobehavioral attention scores. 58 (31%) returned as toddlers for neurodevelopmental follow-up, of which 23% had motor delay. Abnormal infant attention had high sensitivity (92%, 95% CI 60-100%) but low specificity (36%, 95% CI 23-52%) for later motor delay. Higher infant attention scores were associated with higher later motor scores in univariable analysis (coefficient 3.49, 95% CI 0.52,6.46, p = 0.025), but not in multivariable analyses. Neither cognitive nor language scores were associated with infant attention scores. Lower birth weight and male sex were significantly associated with lower motor scores in multivariable analysis (p = 0.048, 0.007). Although impaired infant attention is interdependent with other clinical and demographic risk factors, it may be a sensitive clinical marker of risk for later motor delay. In children with critical CHD, impaired infant attention may be capturing early signs of abnormal visual-motor neurodevelopment.

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.

Variation in provider compliance with sports restriction guidelines in children with an isolated bicuspid aortic valve.

BACKGROUND: Published guidelines for sports restriction for children with a bicuspid aortic valve remain controversial. We sought to describe practice variation and factors influencing sports restrictions in these children. METHODS: This retrospective single-centre study included children (7-18 years old) with an isolated bicuspid aortic valve at baseline from 1 January, 2005 to 31 December, 2014. Sports restrictions, factors potentially influencing decision-making, and outcomes were collected. Descriptive statistics and multivariable mixed-effects logistic regression models were performed with providers and patients as random effects. Provider variation was estimated using intraclass correlation coefficients. Odds ratios, 95% confidence intervals, and p-values were reported from the models. RESULTS: In 565 encounters (253 children; 34 providers), 41% recommended no sports restrictions, 40% recommended high-static and high-dynamic restrictions, and 19% had no documented recommendations. Based on published guidelines, 22% of children were inappropriately restricted while 30% were not appropriately restricted. The paediatric cardiology provider contributed to 37% of observed practice variation (p < 0.001). Sports restriction was associated with older age, males, greater ascending aorta z-score, and shorter follow-up interval. There were no aortic dissections or deaths and one cardiac intervention. CONCLUSION: Physicians frequently fail to document sports restrictions for children with a bicuspid aortic valve, and documented recommendations often conflict with published guidelines. Despite this, no adverse outcomes occurred. Providers accounted for a significant proportion of the variation in sports restrictions. Further research to provide evidence-based guidelines may improve provider compliance with activity recommendations in this population.

Evaluating the Performance of an Automated Patient-Reported Outcomes Collection Platform in a Postoperative Hand Surgery Population.

PURPOSE: Reliable collection of postoperative patient-reported outcome measures (PROMs) is critical to understanding surgical outcomes and the value of care. Automated PROMs collection, triggered by the electronic medical record at the 1-year postoperative anniversary, may provide a simple way to acquire outcomes for patients who have been discharged from clinic. The purposes of this study were to (1) evaluate the percentage of responses with an automated PROMs collection platform and (2) identify whether such a system may introduce selection bias by comparing responders with nonresponders. METHODS: Adult patients (aged ≥18 years) undergoing hand and upper-extremity surgeries between August 2017 and January 2019 were included. Preoperative Quick Disabilities of the Arm, Shoulder, and Hand (QuickDASH) scores were collected using tablet computer. Postoperative QuickDASH scores were collected using a single electronic survey request 1 year after surgery via an email with a personalized REDCap link. Univariate and multivariable regression analyses were performed to identify factors that differed between responders and nonresponders. RESULTS: A response rate of 27% (269 of 1010) was observed for the eligible postoperative patients. On multivariable analysis, the following were associated with greater odds of response: older age, Caucasian race (vs unknown), longer surgery duration, attending the first postoperative visit, and responding to the preoperative QuickDASH. CONCLUSIONS: The poor response rate that was observed highlights that an automated single email postoperative contact for PROMs collection is insufficient-active follow-up via reminder emails and/or telephone calls is needed. Outcome researchers and clinicians must be aware of potential selection biases, such as age and race, that may exist with automated PROMs collection. CLINICAL RELEVANCE: Single email postoperative contact to obtain postoperative PROMs is insufficient.

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.