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.

Left Atrial Strain in Multisystem Inflammatory Syndrome in Children and Associations with Systemic Inflammation and Cardiac Injury.

Multisystem inflammatory syndrome in children (MIS-C) commonly involves cardiac injury with both systolic and diastolic dysfunction. Left atrial strain (LAS) detects subclinical diastolic dysfunction in adults but is infrequently used in children. We evaluated LAS in MIS-C and the associations with systemic inflammation and cardiac injury. In this retrospective cohort study, LAS parameters [reservoir (LAS-r), conduit (LAS-cd), and contractile (LAS-ct)] obtained from admission echocardiograms of MIS-C patients were compared to healthy controls and between MIS-C patients with and without cardiac injury (BNP > 500 pg/ml or troponin-I > 0.04 ng/ml). Correlation and logistic regression analyses were performed to assess LAS associations with admission inflammatory and cardiac biomarkers. Reliability testing was performed. We identified 118 patients with MIS-C and 20 healthy controls. Median LAS parameters were reduced in MIS-C patients compared to controls (LAS-r: 31.8 vs. 43.1%, p < 0.001; LAS-cd: - 28.8 vs. - 34.5%, p = 0.006; LAS-ct: - 5.2 vs. - 9.3%, p < 0.001) and reduced in MIS-C patients with cardiac injury (n = 59) compared to no injury (n = 59) (LAS-r: 29.6 vs. 35.8%, p = 0.001; LAS-cd: - 26.5 vs. - 30.4%, p = 0.036; LAS-ct: - 4.6 vs. - 9.3%, p = 0.008). A discrete LAS-ct peak was absent in 65 (55%) MIS-C patients but present in all controls (p < 0.001). Procalcitonin correlated strongly with averaged E/e' (r = 0.55, p = 0.001). Moderate correlations were found for ESR and LAS-ct (r = - 0.41, p = 0.007) as well as BNP and LAS-r (r = - 0.39, p < 0.001) and LAS-ct (r = 0.31, p = 0.023). Troponin-I had only weak correlations. Intra-rater reliability was good for all LAS parameters, and inter-rater reliability was good to excellent for LAS-r, and fair for LAS-cd and LAS-ct. LAS analysis, particularly the absence of a LAS-ct peak, was reproducible and may be superior to conventional echocardiographic parameters for detecting diastolic dysfunction in MIS-C. No strain parameters on admission were independently associated with cardiac injury.

Intracardiac echocardiography in paediatric and congenital cardiac ablation shortens procedure duration and improves success without complications.

AIMS: Common to adult electrophysiology studies (EPSs), intracardiac echocardiography (ICE) use in paediatric and congenital heart disease (CHD) EPS is limited. The purpose of this study was to assess the efficacy of ICE use and incidence of associated complications in paediatric and CHD EPS. METHODS AND RESULTS: This single-centre retrospective matched cohort study reviewed EPS between 2013 and 2022. Demographics, CHD type, and EPS data were collected. Intracardiac echocardiography cases were matched 1:1 to no ICE controls to assess differences in complications, ablation success, fluoroscopy exposure, procedure duration, and arrhythmia recurrence. Cases and controls with preceding EPS within 5 years were excluded. Intracardiac echocardiography cases without an appropriate match were excluded from comparative analyses but included in the descriptive cohort. We performed univariable and multivariable logistic regression to assess associations between variables and outcomes. A total of 335 EPS were reviewed, with ICE used in 196. The median age of ICE cases was 15 [interquartile range (IQR) 12-17; range 3-47] years, and median weight 57 [IQR 45-71; range 15-134] kg. There were no ICE-related acute or post-procedural complications. There were 139 ICE cases matched to no ICE controls. Baseline demographics and anthropometrics were similar between cases and controls. Fluoroscopy exposure (P = 0.02), procedure duration (P = 0.01), and arrhythmia recurrence (P = 0.01) were significantly lower in ICE cases. CONCLUSION: Intracardiac echocardiography in paediatric and CHD ablations is safe and reduces procedure duration, fluoroscopy exposure, and arrhythmia recurrence. However, not every arrhythmia substrate requires ICE use. Thoughtful selection will ensure the judicious and strategic application of ICE to enhance outcomes.

Baseline Echocardiography and Laboratory Findings in MIS-C and Associations with Clinical Illness Severity.

Children with COVID-associated multisystem inflammatory syndrome (MIS-C) may develop severe disease. We explored the association of admission echocardiographic and laboratory parameters with MIS-C disease severity. This retrospective, single center study of consecutive MIS-C patients (4/2020-12/2021) excluded those with preexisting cardiomyopathy, congenital heart disease, or prior cardiotoxic therapy. Our hypothesis was that worse admission echocardiographic and laboratory parameters were associated with more severe disease based on vasoactive medication use. Univariable and multivariable logistic regression models assessed the association between vasoactive medication use and baseline variables. Of 118 MIS-C patients, median age was 7.8 years (IQR 4.6, 11.8), 48% received vasoactive medication. Higher admission brain natriuretic peptide [OR 1.07 (95% CI 1.02,1.14), p = 0.019], C-reactive protein [OR 1.08 (1.03,1.14), p = 0.002], troponin [OR 1.05 (1.02,1.1), p = 0.015]; lower left ventricular ejection fraction [LVEF, OR 0.96 (0.92,1), p = 0.042], and worse left atrial reservoir strain [OR 0.96 (0.92,1), p = 0.04] were associated with vasoactive medication use. Only higher CRP [OR 1.07 (1.01, 1.11), p = 0.034] and lower LVEF [0.91 (0.84,0.98), p = 0.015] remained independently significant. Among those with normal admission LVEF (78%, 92/118), 43% received vasoactive medication and only higher BNP [OR 1.09 (1.02,1.19), p = 0.021 per 100 pg/mL] and higher CRP [OR 1.07 (1.02,1.14), p = 0.013] were associated with use of vasoactive medication. Nearly half of all children admitted for MIS-C subsequently received vasoactive medication, including those admitted with a normal LVEF. Similarly, admission strain parameters were not discriminatory. Laboratory markers of systemic inflammation and cardiac injury may better predict early MIS-C disease severity.

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.

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..

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.

Multicenter retrospective evaluation of magnetic resonance imaging in pediatric and congenital heart disease patients with cardiac implantable electronic devices.

BACKGROUND: Guidelines addressing magnetic resonance imaging (MRI) in patients with cardiac implantable electronic devices (CIEDs) provide algorithms for imaging pediatric and congenital heart disease (CHD) patients. Guideline acceptance varies by institution. Guidelines also do not support routine MRI scans in patients with epicardial or abandoned leads, common in pediatric and CHD patients. OBJECTIVE: The purpose of this study was to determine the incidence of MRI-related complications in pediatric and CHD patients with CIEDs, including epicardial and/or abandoned leads. METHODS: A multicenter retrospective review included patients with CIEDs who underwent any MRI between 2007 and 2022 at congenital cardiac centers. The primary outcome was any patient adverse event or clinically significant CIED change after MRI, defined as pacing lead capture threshold increase >0.5 V with output change, P- or R- wave amplitude decrease >50% with sensitivity change, or impedance change >50%. RESULTS: Across 14 institutions, 314 patients (median age 18.8 [1.3; 31.4] years) underwent 389 MRIs. There were 288 pacemakers (74%) and 87 implantable cardioverter-defibrillators (22%); 52% contained epicardial leads, and 14 (4%) were abandoned leads only. Symptoms or CIED changes occurred in 4.9% of MRI scans (6.1% of patients). On 9 occasions (2%), warmth or pain occurred. Pacing capture threshold or lead impedance changes occurred in 1.4% and 2.0% of CIEDs post-MRI and at follow-up. CONCLUSION: Our data provide evidence that MRIs can be performed in pediatric and CHD patients with CIEDs, including non-MRI-conditional CIEDs and epicardial and/or abandoned leads, with rare minor symptoms or CIED changes but no other complications.

Left atrial strain in multisystem inflammatory syndrome in children (MIS-C) and associations with systemic inflammation and cardiac injury.

Background: Multisystem inflammatory syndrome in children (MIS-C) commonly involves cardiac injury with both systolic and diastolic dysfunction. Left atrial strain (LAS) detects subclinical diastolic dysfunction in adults but is infrequently used in children. We evaluated LAS in MIS-C and the associations with systemic inflammation and cardiac injury. Methods: In this retrospective cohort study, conventional parameters and LAS (reservoir [LAS-r], conduit [LAS-cd], and contractile [LAS-ct]) obtained from admission echocardiograms of MIS-C patients were compared to healthy controls and between MIS-C patients with and without cardiac injury (BNP >500 pg/ml or troponin-I >0.04 ng/ml). Correlation and logistic regression analyses were performed to assess LAS associations with admission inflammatory and cardiac biomarkers. Reliability testing was performed. Results: Median LAS components were reduced in MIS-C patients (n=118) compared to controls (n=20) (LAS-r: 31.8 vs. 43.1%, p<0.001; LAS-cd: -28.8 vs. -34.5%, p=0.006; LAS-ct: -5.2 vs. -9.3%, p<0.001) and reduced in MIS-C patients with cardiac injury (n=59) compared to no injury (n=59) (LAS-r: 29.6 vs. 35.8%, p=0.001; LAS-cd: -26.5 vs. -30.4%, p=0.036; LAS-ct: -4.6 vs. -9.3%, p=0.008). An LAS-ct peak was absent in 65 (55%) MIS-C patients but present in all controls (p<0.001). Procalcitonin had strong correlation with averaged E/e' (r=0.55, p=0.001); ESR had moderate correlation with LAS-ct (r=-0.41, p=0.007); BNP had moderate correlation with LAS-r (r=-0.39, p<0.001) and LAS-ct (r=0.31, p=0.023), and troponin-I had only weak correlations. No strain indices were independently associated with cardiac injury on regression analysis. Intra-rater reliability was good for all LAS components; and inter-rater reliability was good to excellent for LAS-r, and fair for LAS-cd and LAS-ct. Conclusions: LAS analysis, particularly the absence of a LAS-ct peak, was reproducible and may be superior to conventional echocardiographic parameters for detecting diastolic dysfunction in MIS-C. No strain parameters on admission were independently associated with cardiac injury.

Coronary artery dilation associated with bicuspid and unicuspid aortic valve disease in children: a series of 17 patients.

INTRODUCTION: Coronary artery dilation associated with bicuspid/unicuspid aortic valves is described in adults with limited data in children. We aimed to describe the clinical course of children with bicuspid/unicuspid aortic valves and coronary dilation including coronary Z-score changes over time, association of coronary changes with aortic valve anatomy/function, and complications. MATERIALS AND METHODS: Institutional databases were searched for children ≤18 years with both bicuspid/unicuspid aortic valves and coronary dilation (1/2006-6/2021). Kawasaki disease and isolated supra-/subvalvar aortic stenosis were excluded. Statistics were descriptive with associations measured by Fisher's exact test and overlapping 83.7% confidence intervals. RESULTS: Of 17 children, bicuspid/unicuspid aortic valve was diagnosed at birth in 14 (82%). Median age at coronary dilation diagnosis was 6.4 years (range: 0-17.0). Aortic stenosis was present in 14 (82%) [2 (14%) moderate, 8 (57%) severe]; 10 (59%) had aortic regurgitation; 8 (47%) had aortic dilation. The right coronary was dilated in 15 (88%), left main in 6 (35%), and left anterior descending in 1 (6%) with no relationship between leaflet fusion pattern or severity of aortic regurgitation/stenosis on coronary Z-score. Follow-up evaluations were available for 11 (mean 9.3 years, range 1.1-14.8) with coronary Z-scores increasing in 9/11 (82%). Aspirin was used in 10 (59%). There were no deaths or coronary artery thrombosis. DISCUSSION: In children with bicuspid/unicuspid aortic valves and coronary dilation, the right coronary artery was most frequently involved. Coronary dilation was observed in early childhood and frequently progressed. Antiplatelet medication use was inconsistent, but no child died nor developed thrombosis.

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.

Ventricular arrhythmias following balloon-expandable transcatheter pulmonary valve replacement in the native right ventricular outflow tract.

BACKGROUND: Ventricular arrhythmia incidence in children and adolescents undergoing transcatheter pulmonary valve replacement (TPVR) within the native right ventricular outflow tract (nRVOT) is unknown. We sought to describe the incidence, severity, and duration of ventricular arrhythmias and identify associated risk factors in this population. METHODS: This was a retrospective cohort study of 78 patients <21 years of age who underwent TPVR within the nRVOT. Patients were excluded for pre-existing ventricular arrhythmia or antiarrhythmic use. Study variables included surgical history, valve replacement indication, valve type/size, and ventricular arrhythmia. Univariable logistic regression models were used to evaluate factors associated with ventricular arrhythmias, followed by subset analyses. RESULTS: Nonsustained ventricular arrhythmia occurred in 26/78 patients (33.3%). The median age at the procedure was 10.3 years (interquartle range [IQR]: 6.5, 12.8). Compared with other nRVOT types, surgical repair with transannular patch was protective against ventricular arrhythmia incidence: odds ratio (OR): 0.35 (95% confidence interval [CI], 0.13-0.95). Patient weight, valve type/size, number of prestents, and degree of stent extension into the RVOT were not associated with ventricular arrhythmia occurrence. Beta blocker was started in 16/26 (61.5%) patients with ventricular arrhythmia. One additional patient was lost to follow-up. The median beta blocker duration was 46 days (IQR 42, 102). Beta blocker was discontinued in 10 patients by 8-week follow-up and in the remaining four by 9 months. CONCLUSIONS: Though common after balloon-expandable TPVR within the nRVOT, ventricular arrhythmias were benign and transient. Antiarrhythmic medications were successfully discontinued in the majority at 6- to 8-week follow-up, and in all patients by 20 months.

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.

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.

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

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

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.

Can mild traumatic brain injury alter cognition chronically? A LIMBIC-CENC multicenter study.

OBJECTIVE: While outcome from mild traumatic brain injury (mTBI) is generally favorable, concern remains over potential negative long-term effects, including impaired cognition. This study examined the link between cognitive performance and remote mTBIs within the Long-term Impact of Military-relevant Brain Injury Consortium-Chronic Effects of Neurotrauma Consortium (LIMBIC-CENC) multicenter, observational study of Veterans and service members (SMs) with combat exposure. METHOD: Baseline data of the participants passing all cognitive performance validity tests (n = 1,310) were used to conduct a cross-sectional analysis. Using multivariable regression models that adjusted for covariates, including age and estimated preexposure intellectual function, positive mTBI history groups, 1-2 lifetime mTBIs (nonrepetitive, n = 614), and 3 + lifetime mTBIs (repetitive; n = 440) were compared to TBI negative controls (n = 256) on each of the seven cognitive domains computed by averaging Z scores of prespecified component tests. Significance levels were adjusted for multiple comparisons. RESULTS: Neither of the mTBI positive groups differed from the mTBI negative control group on any of the cognitive domains in multivariable analyses. Findings were also consistently negative across sensitivity analyses (e.g., mTBIs as a continuous variable, number of blast-related mTBIs, or years since the first and last mTBI). CONCLUSIONS: Our findings demonstrate that the average veteran or SM who experienced one or more mTBIs does not have postacute objective cognitive deficits due to mTBIs alone. A holistic health care approach including comorbidity assessment is indicated for patients reporting chronic cognitive difficulties after mTBI(s), and strategies for addressing misattribution may be beneficial. Future study is recommended with longitudinal designs to assess within-subjects decline from potential neurodegeneration. (PsycInfo Database Record (c) 2023 APA, all rights reserved).