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Objective: This study tested the hypothesis that phosphodiesterase 5 inhibition alone or in combination with ACE inhibition improves glucose homeostasis and fibrinolysis in individuals with metabolic syndrome.Research Design and Methods: Insulin sensitivity, beta-cell function, and fibrinolytic parameters were measured in 18 adults with metabolic syndrome on 4 separate days after a randomized, crossover, double-blind, 3-week treatment with placebo, ramipril (10 mg/day), tadalafil (10 mg o.d.), and ramipril plus tadalafil.Results: Ramipril decreased systolic and diastolic blood pressure, ACE activity, and angiotensin II and increased plasma renin activity. Ramipril did not affect insulin sensitivity or beta-cell function. In contrast, tadalafil improved beta-cell function (P = 0.01). This effect was observed in women (331.9 +/- 209.3 vs. 154.4 +/- 48.0 32 micro x mmol(-1) x l(-1), respectively, for tadalafil treatment vs. placebo; P = 0.01) but not in men. There was no effect of any treatment on fibrinolysis. CONCLUSIONS Phosphodiesterase 5 inhibition may represent a novel strategy for improving beta-cell function in metabolic syndrome. [ABSTRACT FROM AUTHOR]

Background: Prior investigations of the center-specific case volume on outcomes in hypoplastic left heart syndrome have conflicting results. This study utilized the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry to investigate the center volume-outcome relationship in patients following the Norwood procedure with consideration of pre-operative high-risk features., Methods: Between 2016 and 2023, centers were categorized by Norwood procedure volume into low (≤ 5 cases/year), medium (6 to 10 cases/year), and high-volume centers (> 10 cases/year). We compared pre-operative high-risk features between the center volume categories and assessed survival outcomes, focusing on 30-day and 1-year mortality. We further compared short-term perioperative morbidity outcomes., Results: We analyzed 3,397 patients from 69 institutions participating in NPC-QIC. Twenty-nine centers were classified as a low-, 20 as medium-, and 20 as high-volume centers. There was no difference in frequency of preoperative high-risk features among the center categories in the majority of considered variables. There was no association between the volume categories and 30-day mortality. Low-volume and medium-volume were associated with higher risk of 1-year mortality. This difference remained when adjusing for the presence of high-risk features (Low: OR (95%CI) 1.40 (1.03-1.60), P=0.020; Medium: OR (95%CI) 1.28 (1.05-1.86), P=0.025). Post-operative comorbidities were more frequent in low-, and medium-volume centers, including the need for diagnostic and interventional catheterization., Conclusions: Patients undergoing Norwood procedure in low-, and medium-volume centers have worse 1-year mortality. The outcome characteristics are potentiated when adjusted for high-risk features with significantly higher survival and lower morbidity in patients treated in high-volume centers., (Copyright © 2025. Published by Elsevier Inc.)

Background: Significant atrioventricular valve dysfunction can be associated with mortality or need for transplant in functionally univentricular heart patients undergoing staged palliation. The purposes of this study are to characterize the impact of concomitant atrioventricular valve intervention on outcomes at each stage of single ventricle palliation and to identify risk factors associated with poor outcomes in these patients. Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried for functionally univentricular heart patients undergoing single ventricle palliation from 2013 through 2022. Separate analyses were performed on cohorts corresponding to each stage of palliation (1: initial palliation; 2: superior cavopulmonary anastomosis; 3: Fontan procedure). Bivariate analysis of demographics, diagnoses, comorbidities, preoperative risk factors, operative characteristics, and outcomes with and without concomitant atrioventricular valve intervention was performed. Multiple logistic regression was used to identify predictors associated with operative mortality or major morbidity. Results: Concomitant atrioventricular valve intervention was associated with an increased risk of operative mortality or major morbidity for each cohort (cohort 1: 62% vs 46%, P  < .001; cohort 2: 37% vs 19%, P  < .001; cohort 3: 22% vs 14%, P  < .001). Black race in cohort 1 (odds ratio [OR] 3.151, 95% CI 1.181-9.649, P  = .03) and preterm birth in cohort 2 (OR 1.776, 95% CI 1.049-3.005, P  = .032) were notable predictors of worse morbidity or mortality. Conclusions: Concomitant atrioventricular valve intervention is a risk factor for operative mortality or major morbidity at each stage of single ventricle palliation. Several risk factors are associated with these outcomes and may be useful in guiding decision-making., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Although medical advancements have improved the mortality of CHD, morbidity still exists, impacting patient quality of life. Returning to baseline in the early surgical recovery phase is an area of potential improvement. This preliminary project aims to qualitatively understand CHD family perspectives concerning the immediate postoperative recovery phase. The participating patients enrolled in the Enhanced Recovery After Surgery program, a postsurgical symptom management tool utilised in adult centres and broadening into pediatrics. Twenty-three of 27 contacted families answered open-ended questions 1 to 3 months postoperatively regarding difficulties experienced during their first week home. They reviewed a list of symptoms including: difficulties with pain, nausea, activity, sleep, appetite, bowel or urinary systems, and taking medications. A qualitative thematic analysis was performed with the open responses, as well as a quantitative assessment of the types of issues that made recovery challenging. Participants struggled most with sleep (78%), returning to activity (70%), and pain (57%). Open-ended responses suggested that an inability to do daily activities, sleep (frequently impacted by pain), and inadequate resources most negatively impacted recovery. Given these findings, investigating postoperative sleep regimens and effective pain plan components may prove useful, in addition to the further development of early mobility programs. The positive and negative experiences highlighting the desire for readily available medical guidance enforce the need for open communication between families and team members, potentially aided by digital tools. Ultimately, further data could support the development of a standardised protocol to better the immediate postoperative quality of life for CHD families.

In cases of uncontrollable hepatic hemorrhage or acute hepatic failure after trauma, liver transplantation can be a lifesaving procedure. Traumatic tricuspid valve injuries are rare, and symptoms can range from indolent to acute right heart failure. When concomitant, traumatic liver transplant and tricuspid injuries have significant physiologic interplay and management implications. We present a 14-year-old male injured in an all-terrain vehicle accident, who sustained a devastating disruption of the common bile duct and celiac artery injury, leading to acute hepatic failure, necessitating a two-stage liver transplantation. He was subsequently found to have a severe traumatic tricuspid injury, which required tricuspid valve replacement. At 4 years post-injury, he is without major complications. This is the first case presentation of the cooccurrence of these complex pathologies. Importantly, we demonstrate the complex decision-making surrounding traumatic liver transplantation and timing of subsequent tricuspid valve repair, weighing the complex interplay of these 2 pathologies., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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.

Background: Medical management, primarily a fat-modified diet (FMD), is the mainstay of treatment for most patients with chylothorax. Duration of FMD is traditionally reported as 6 weeks, but no studies have demonstrated the shortest effective duration that prevents recurrence of chylothorax. The aim of this study was to decrease FMD duration to 2 weeks in children with postoperative chylothorax without a significant increase in recurrence., Methods: This single-center study included pediatric (aged <18 years) patients in whom chylothorax developed within 30 days of cardiac surgery. Patients with cavopulmonary anastomoses were excluded. The preintervention cohort consisted of 19 patients with a diagnosis of chylothorax between February 2014 and June 2015, and the postintervention cohort comprised 98 patients from July 2015 to December 2019. FMD duration was decreased from 6 weeks to 4 weeks in May 2016 and to 2 weeks in June 2018. Recurrence was defined as a return of a chylous effusion requiring chest tube placement or hospital readmission within 30 days of resuming a regular diet., Results: The median duration of FMD decreased from 42 days (interquartile range, 30, 43 days) in the preintervention cohort to 26 days (interquartile range, 14, 29 days) in the postintervention cohort, with no recurrence of chylothorax in any group. Compliance with the FMD duration instruction in the 6-week, 4-week, and 2-week groups was 100%, 84%, and 67% respectively. Compared with the first 6 months, compliance with the 2-week FMD instruction during the final 12 months increased from 40% (6/15) to 79% (26/33)., Conclusions: At the study center, FMD duration decreased from 6 weeks to 2 weeks without any recurrence of chylothorax., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: There are no published data on left ventricular (LV) reverse remodeling after transcatheter aortic valve replacement (TAVR) in children. The aim of this study was to assess changes in LV echocardiographic parameters 6 months after TAVR in children., Methods: This single-center, retrospective study included all 22 patients (age < 21 years) who underwent TAVR. The median age was 14.7 years (interquartile range, 13.3-15.9 years), median weight was 57 kg (interquartile range, 46.0-66.3 kg), and 59% of patients were male. Demographics, type and duration of aortic valve dysfunction, symptom and treatment data, and preprocedural and 6-month follow-up echocardiographic data (LV volume, mass, end-diastolic dimension, end-systolic dimension, ejection fraction [EF], sphericity, and longitudinal strain) were collected. Failure to reverse remodel at 6 months was defined as meeting at least two of the following: Z score ≥ 2 that was unchanged or increased from baseline for LV volume, mass, end-diastolic dimension, or end-systolic dimension; abnormally high sphericity index that was unchanged or increased; and abnormally low EF or longitudinal strain. Median, interquartile range, and range are reported for continuous variables, and pre- and post-TAVR data were compared using the Wilcoxon signed rank test., Results: Eight patients (36%) had isolated aortic stenosis, four (18%) had isolated regurgitation, and 10 had (46%) mixed disease. Twelve (55%) had symptoms and 20 (91%) had prior surgical or catheter valve interventions. The primary complication was left bundle branch block, occurring in four children (18%). At 6 months, LV volume, mass, end-diastolic dimension, end-systolic dimension, and sphericity index improved. EF and strain were normal at baseline and at follow-up. Of three patients who failed to reverse remodel, two had left bundle branch block. Of three patients with persistent symptoms, one had failure of reverse remodeling., Conclusions: Most pediatric patients had evidence of reverse LV remodeling 6 months after TAVR, suggesting a possible alternative to surgical aortic valve replacement in this population. Functional parameters (EF and strain) were normal at baseline and follow-up. Future studies are needed to determine optimal timing of TAVR and to explore the association of postprocedural left bundle branch block on failed reverse remodeling and outcomes in this population., (Copyright © 2022 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Transcatheter aortic valve implantation (TAVI) is common in adults but rare in children and adolescents. Since 2014, our institution has incorporated a transcatheter approach as an option for aortic valve replacement in this population. The purpose of this study was to compare short-term outcomes of TAVI with surgical aortic valve replacement (SAVR). This single-center, retrospective study included patients aged 10 to 21 years who had a native SAVR or TAVI between January 2010 to April 2020. Comparative analysis of baseline characteristics and a composite outcome (stroke within 6 months, readmission within 30 days, death) between SAVR and TAVI were made using chi-square test or Wilcoxon rank sum test, as appropriate. Of the 77 patients who underwent native aortic valve implantation during the study period (60 SAVR, 17 TAVI), 46 were aged 10 to 21 years (30 SAVR, 16 TAVI). Median follow-up was 3.8 years (interquartile range 1.5 to 4.9) for the SAVR group and 1.5 years (interquartile range 1.1 to 1.2) for the TAVI group. There was no difference in the composite outcome between groups. Patients in the SAVR group were more likely to have undergone concomitant surgical intervention and have longer intensive care unit and hospital stays. In conclusion, our study suggests similar short-term outcomes between SAVR and TAVI in children and young adults aged 10 to 21 years. Longer-term studies are essential to understand the utility of TAVI and to better consider the option of a transcatheter approach as an alternative to SAVR in the pediatric population., (Published by Elsevier Inc.)

Background: Patients receiving a right ventricle to pulmonary artery conduit (PC) in infancy will require successive procedures or replacements, each with variable longevity. We sought to identify factors associated with time-related risk of a subsequent surgical replacement (PC3) or transcatheter pulmonary valve insertion (TPVI) after a second surgically placed PC (PC2)., Methods: From 2002 to 2016, 630 patients from 29 Congenital Heart Surgeons' Society member institutions survived to discharge after initial valved PC insertion (PC1) at age ≤ 2 years. Of those, 355 underwent surgical replacement (PC2) of that initial conduit. Competing risk methodology and multiphase parametric hazard analyses were used to identify factors associated with time-related risk of PC3 or TPVI., Results: Of 355 PC2 patients (median follow-up, 5.3 years), 65 underwent PC3 and 41 TPVI. Factors at PC2 associated with increased time-related risk of PC3 were smaller PC2 Z score (hazard ratio [HR] 1.6, P < .001), concomitant aortic valve intervention (HR 7.6, P = .009), aortic allograft (HR 2.2, P = .008), younger age (HR 1.4, P < .001), and larger Z score of PC1 (HR 1.2, P = .04). Factors at PC2 associated with increased time-related risk of TPVI were aortic allograft (HR: 3.3, P = .006), porcine unstented conduit (HR 4.7, P < .001), and older age (HR 2.3, P = .01)., Conclusions: Aortic allograft as PC2 was associated with increased time-related risk of both PC3 and TPVI. Surgeons may reduce risk of these subsequent procedures by not selecting an aortic homograft at PC2, and by oversizing the conduit when anatomically feasible., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Cardiac arrhythmias occur in 3-40% of patients with acute myocarditis and cause significant morbidity and mortality. Myocardial injury also results in abnormal myocardial deformation. The relationship between left ventricular (LV) deformation, measured by two-dimensional speckle tracking echocardiography (2D-STE), and arrhythmia in pediatric myocarditis is unknown. We evaluated the association between 2D-STE and arrhythmias in children hospitalized with acute myocarditis. We reviewed patients ≤ 18 years hospitalized for acute myocarditis from 2008 to 2018. Arrhythmias were defined as 1) non-sustained or sustained ventricular tachycardia or ventricular fibrillation, 2) sustained supraventricular tachycardia (SVT), 3) high-grade or complete heart block, and 4) any arrhythmia treated with an antiarrhythmic medication. Systolic LV strain values (including LV global longitudinal strain (GLS), global circumferential strain (GCS), and six segments of LV regional long axis strain) were obtained from initial echocardiograms during hospitalization. Of 66 patients hospitalized, 23 (35%) had arrhythmias. SVT was the predominant arrhythmia (74%). Global and regional strain indices were reduced in the arrhythmia group: LV GLS [-8.9 (IQR -13.6, -6.1) vs. -13.7 (IQR -16.9, -9.7), p = 0.038]; basal inferior/septal [-10.7 (IQR -15.5, -7.8) vs. -16.4 (IQR -18, -11.8), p = 0.009]; basal anterior/lateral [-7.1 (IQR -12.8, -4.7) vs. -9.4 (IQR -16.7, -7.4), p = 0.025]; and mid inferior/septal segments [-9 (IQR -13, -7.7) vs. -14.1 (IQR -22.5, -10.7), p = 0.007]. After controlling for age, reductions in GLS and segmental strain in the two basal and two mid-segments were associated with increased arrhythmia occurrence (p <0.05). Our findings suggest that echocardiographic LV deformation by 2D-STE may be useful in identifying pediatric patients with acute myocarditis at risk for arrhythmias., Competing Interests: Declaration of Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Infants with aortic coarctation may present with left ventricular (LV) dysfunction which may complicate the postoperative course and lead to increased healthcare costs. We aimed to define the prevalence of moderate to severe left ventricular (LV) systolic dysfunction, evaluate time to recovery, and compare health care costs. Single-center retrospective cohort study at a tertiary care hospital was conducted. Infants < 6 months old at diagnosis with aortic coarctation were identified using surgical codes for coarctation repair between January 2010 and May 2018. Moderate to severe dysfunction was defined as ejection fraction (EF) < 40%. Of 160 infants studied, 18 (11%) had moderate to severe LV dysfunction at presentation. Compared to those with better LV function, infants with moderate to severe LV dysfunction were older at presentation (12 vs. 6 days, p = 0.004), had more postoperative cardiac intensive care unit (ICU) days (5 vs. 3, p < 0.001), and more ventilator days (3.5 vs. 1, p < 0.001). The median time to normal LV EF (≥ 55%) was 6 days postoperatively (range 1-230 days). Infants presenting with moderate to severe LV dysfunction had higher index hospitalization costs ($90,560 vs. $59,968, p = 0.02), but no difference in cost of medical follow-up for the first year following discharge ($3,078 vs. $2,568, p = 0.46). In the current era, > 10% of infants with coarctation present with moderate to severe LV dysfunction that typically recovers. Those with moderate to severe dysfunction had longer duration of mechanical ventilation and postoperative cardiac ICU stays, likely driving higher costs of index hospitalization.

Objectives: We leveraged decomposition analysis, commonly used in labor economics, to understand determinants of cost differences related to location of admission in children undergoing neonatal congenital heart surgery., Design: A retrospective cohort study., Setting: Pediatric Health Information Systems database., Patients: Neonates (<30 d old) undergoing their index congenital heart surgery between 2004 and 2013., Measurements and Main Results: A decomposition analysis with bootstrapping determined characteristic (explainable by differing covariate levels) and structural effects (if covariates are held constant) related to cost differences. Covariates included center volume, age at admission, prematurity, sex, race, genetic or major noncardiac abnormality, Risk Adjustment for Congenital Heart Surgery-1 score, payor, admission year, cardiac arrest, infection, and delayed sternal closure.Of 19,984 infants included (10,491 [52%] to cardiac ICU/PICU and 9,493 [48%] to neonatal ICU), admission to the neonatal ICU had overall higher average costs ($24,959 ± $3,260; p < 0.001) versus cardiac ICU/PICU admission. Characteristic effects accounted for higher costs in the neonatal ICU ($28,958 ± $2,044; p < 0.001). Differing levels of prematurity, genetic syndromes, hospital volume, age at admission, and infection contributed to higher neonatal ICU costs, with infection rate providing the most significant contribution ($13,581; p < 0.001). Aggregate structural effects were not associated with cost differences for those admitted to the neonatal ICU versus cardiac ICU/PICU (p = 0.1). Individually, prematurity and age at admission were associated with higher costs due to structural effects for infants admitted to the neonatal ICU versus cardiac ICU/PICU., Conclusions: The difference in cost between neonatal ICU and cardiac ICU/PICU admissions is largely driven by differing prevalence of risk factors between these units. Infection rate was a modifiable factor that accounted for the largest difference in costs between admitting units.

Background/aims: Registry-based trials have emerged as a potentially cost-saving study methodology. Early estimates of cost savings, however, conflated the benefits associated with registry utilisation and those associated with other aspects of pragmatic trial designs, which might not all be as broadly applicable. In this study, we sought to build a practical tool that investigators could use across disciplines to estimate the ranges of potential cost differences associated with implementing registry-based trials versus standard clinical trials., Methods: We built simulation Markov models to compare unique costs associated with data acquisition, cleaning, and linkage under a registry-based trial design versus a standard clinical trial. We conducted one-way, two-way, and probabilistic sensitivity analyses, varying study characteristics over broad ranges, to determine thresholds at which investigators might optimally select each trial design., Results: Registry-based trials were more cost effective than standard clinical trials 98.6% of the time. Data-related cost savings ranged from $4300 to $600,000 with variation in study characteristics. Cost differences were most reactive to the number of patients in a study, the number of data elements per patient available in a registry, and the speed with which research coordinators could manually abstract data. Registry incorporation resulted in cost savings when as few as 3768 independent data elements were available and when manual data abstraction took as little as 3.4 seconds per data field., Conclusions: Registries offer important resources for investigators. When available, their broad incorporation may help the scientific community reduce the costs of clinical investigation. We offer here a practical tool for investigators to assess potential costs savings.

Neurodevelopmental (ND) impairment is common in children with congenital heart disease (CHD). While routine ND surveillance and evaluation of high-risk patients has become the standard-of-care, capture rate, barriers to referral, and potential patient benefits remain incompletely understood. Electronic data warehouse records from a single center were reviewed to identify all eligible and evaluated patients between July 2015 and December 2017 based on current guidelines for ND screening in CHD. Diagnoses, referring provider, and payor were considered. Potential benefit of the evaluation was defined as receipt of new diagnosis, referral for additional evaluation, or referral for a new service. Contingencies were assessed with Fisher's exact test. In this retrospective, cohort study, of 3434 children identified as eligible for ND evaluation, 135 were evaluated (4%). Appropriate evaluation was affected by diagnostic bias against coarctation of the aorta (CoArc) and favoring hypoplastic left heart syndrome (HLHS) (1.8 vs. 11.9%, p<0.01). Referrals were disproportionally made by a select group of cardiologists, and the rate of ND appointment non-compliance was higher in self-pay compared to insured patients (78% vs 27%, p<0.01). Potential benefit rate was 70-80% amongst individuals with the three most common diagnoses requiring neonatal surgery (CoArc, transposition of the great arteries, and HLHS). Appropriate ND evaluation in CHD is impacted by diagnosis, provider, and insurance status. Potential benefit of ND evaluation is high regardless of diagnosis. Strategies to improve access to ND evaluations and provider understanding of the at-risk population will likely improve longitudinal ND surveillance and clinical benefit.

Background: The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion., Methods: Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies across centres. Classification and regression tree analysis assessed the association of centre-level factors with length of stay and percentage of patients with prolonged pleural effusion (>7 days)., Results: The median Fontan age (14 centres, 320 patients) was 3.1 years (range from 1.7 to 3.9), and the weight-for-age z-score was -0.56 (-1.35 + 0.44). Extra-cardiac Fontans were performed in 79% (4-100%) of patients at the 13 centres performing this procedure; lateral tunnels were performed in 32% (3-100%) at the 11 centres performing it. Deep hypothermic circulatory arrest (nine centres) ranged from 6 to 100%. Major complications occurred in 17% (7-33%). The length of stay was 9.5 days (9-12); 15% (6-33%) had prolonged pleural effusion. Centres with fewer patients (<6%) with prolonged pleural effusion and fewer (<41%) complications had a shorter length of stay (<10 days; sensitivity 1.0; specificity 0.71; area under the curve 0.96). Avoiding deep hypothermic circulatory arrest and higher weight-for-age z-score were associated with a lower percentage of patients with prolonged effusions (<9.5%; sensitivity 1.0; specificity = 0.86; area under the curve 0.98)., Conclusions: Fontan perioperative practices varied widely among study centres. Strategies to decrease the duration of pleural effusion and minimise complications may decrease the length of stay. Further research regarding deep hypothermic circulatory arrest is needed to understand its association with prolonged pleural effusion.

Objective: The objective of this study was to determine if the implementation of a value-driven outcomes tool comprising modifiable quality and utilization metrics lowers cost and improves value of coronary artery bypass grafting (CABG) postoperative care., Methods: Ten metrics were defined for CABG patients in 2 temporally separated phases. Clinical care protocols were designed and implemented to increase compliance with these metrics. Clinical outcomes and cost data were harvested from the electronic medical record using a proprietary value-driven outcomes tool and verified by a data management team. "Perfect care" was defined as achieving all 10 metrics per patient episode., Results: Over a 45-month period, data of 467 consecutive patients who underwent isolated CABG were analyzed. "Perfect care" was successfully achieved in 304 patients (65.1%). There were no observed differences in mortality between patient groups. Linear regression analysis showed a negative correlation between percent compliance with "perfect care" and mean cost. When multivariate analysis was used to adjust for preoperative risk score, mean cost for patients with "perfect care" was 37.0% less than for those without "perfect care.", Conclusions: In the context of focused institution-specific interventions to target quality and utilization metrics for CABG care, clinical care pathways and protocols informed by innovative tools that link automated tracking of these metrics to cost data might simultaneously promote quality and decrease costs, thereby enhancing value. This descriptive study provides preliminary support for a systematic approach to define, measure, and modulate the drivers of value for cardiothoracic surgery patients., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Transcatheter pulmonary valve replacement (TPVR) is increasingly utilized to address residual or recurrent right ventricular outflow tract pathology in congenital heart disease patients. We aimed to evaluate clinical outcomes and resource utilization comparing TPVR to surgical pulmonary valve replacement (SPVR) for this patient population., Methods: From January 2010 until December 2015, 224 patients underwent pulmonary valve replacement (SPVR = 100 and TPVR = 124). These groups were assessed and compared for survival, reoperation, reintervention (surgical or catheter based), hospital costs, and hemodynamic performance., Results: There were no mortalities in either group. Despite SPVR patients being significantly younger (12 ± 7 years of age versus 19 ± 13 years of age; p < 0.001) and smaller (body surface area 1.2 ± 0.5 m 2 versus 1.4 ± 0.5 m 2 ; p < 0.001), they had similar median valve size implanted (23 mm [interquartile range, 21 to 27 mm) versus 22 mm [interquartile range, 20 to 22 mm]). There was no difference in the adjusted peak gradient (SPVR 24.1 ± 15.1 mm Hg versus TPVR 20.7 ± 11.4 mm Hg; p = 017) at last follow-up. Cumulative freedom from reintervention at 4 years was similar between groups (p = 0.98). TPVR could not be placed in 34 patients either due to coronary compression or excessive outflow tract diameter. SPVR patients had longer hospital length of stay (4.1 ± 1.8 days versus 1.2 ± 0.7 days; p < 0.001). TPVR patients had higher rates of infective endocarditis (4.8% versus 0%; p < 0.001) and overall hospital costs ($57,221 ± $13091 versus $44,366 ± $16,519; p < 0.001)., Conclusions: Hemodynamic performance is similar between SPVR and TPVR with similar rates of reintervention. While SPVR patients have a longer hospital length of stay, TPVR was associated with higher rates of infective endocarditis, hospital costs, and failure to implant., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Our primary goal was to decrease time to resolution of postoperative chylothorax as demonstrated by total days of chest tube utilisation through development and implementation of a management protocol., Methods: A chylothorax management protocol was implemented as a quality improvement project at a tertiary-care paediatric hospital in July, 2015. Retrospective analysis was completed on patients aged 0-17 years diagnosed with chylothorax within 30 days of cardiac surgery in a pre-protocol cohort (February, 2014 to June, 2015, n=20) and a post-protocol cohort (July, 2015 to March, 2016, n=22).Measurements and resultsPatient characteristics were similar before and after protocol implementation. Duration of mechanical ventilation and cardiac ICU and hospital lengths of stay were unchanged between cohorts. Following protocol implementation, total duration of chest tube utilisation decreased from 12 to 7 days (p=0.047) with a decrease in maximum days of chest tube utilisation from 44 to 13 days. Duration of medium-chain triglyceride feeds decreased from 42 days to 31 days (p=0.01). In total, three patients in the post-protocol cohort underwent additional surgical procedures to treat chylothorax with subsequent resolution of chylothorax within 24 hours. There were no chest tube re-insertions or re-admissions related to chylothorax in either the pre- or post-protocol cohorts. Protocol compliance was 81%., Conclusions: Adoption of a chylothorax management protocol is feasible, and in our small cohort of patients implementation led to a significant decrease in the duration of chest tube utilisation, while eliminating practice variability among providers.

Pediatric heart transplantation (HT) is resource intensive. Event-driven pediatric databases do not capture data on resource use. The objective of this study was to evaluate resource utilization and identify associated factors during initial hospitalization for pediatric HT. This multicenter retrospective cohort study utilized the Pediatric Health Information Systems database (43 children's hospitals in the United States) of children ≤19 years of age who underwent transplant between January 2007 and July 2013. Demographic variables including site, payer, distance and time to center, clinical pre- and post-transplant variables, mortality, cost, and charge were the data collected. Total length of stay (LOS) and charge for the initial hospitalization were used as surrogates for resource use. Charges were inflation adjusted to 2013 dollars. Of 1,629 subjects, 54% were male, and the median age at HT was 5 years (IQR [interquartile range] 0 to 13). The median total and intensive care unit LOS were 51 (IQR 23 to 98) and 23 (IQR 9 to 58) days, respectively. Total charge and cost for hospitalization were $852,713 ($464,900 to $1,609,300) and $383,600 ($214,900 to $681,000) respectively. Younger age, lower volume center, southern region, and co-morbidities before transplant were associated with higher resource use. In later years, charges increased despite shorter LOS. In conclusion, this large multicenter study provides novel insight into factors associated with resource use in pediatric patients having HT. Peritransplant morbidities are associated with increased cost and LOS. Reducing costs in line with LOS will improve health-care value. Regional and center volume differences need further investigation for optimizing value-based care and efficient use of scarce resources., (Copyright © 2018 Elsevier Inc. All rights reserved.)

We report the case of a 4-year-old boy with Ehlers-Danlos syndrome undergoing single-ventricle palliation for an unbalanced atrioventricular canal defect. No reports of single-ventricle palliation in the setting of connective tissue disorders exist in the current literature. Unique findings on the patient's preoperative imaging included a disproportionately large neoaortic root and a regurgitant atrioventricular valve, which may foretell the need for future intervention., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: This study evaluates the morbidity, mortality, and cost differences between patients who underwent either a simple or a complex arterial switch operation., Methods: A retrospective study of patients undergoing an arterial switch operation at a single institution was performed. Simple cases were defined as patients with d-transposition of the great arteries with usual coronary anatomy or circumflex artery originating from the right with either intact ventricular septum or ventricular septal defect. Complex cases included all other forms of coronary anatomy, aortic coarctation or arch hypoplasia, and Taussig-Bing anomalies. Costs were acquired using an institutional activity-based accounting system., Results: A total of 98 patients were identified, 68 patients in the simple group and 30 in the complex group. The mortality rate was 2% for the simple and 7% for the complex group, p=0.23. Major morbidities including cardiac arrest, extracorporeal membrane oxygenation, a major coronary event, surgical or catheter-based re-intervention, stroke, or permanent pacemaker placement, non-cardiac surgical procedures, mediastinitis, and sepsis did not differ between the simple and complex groups (16 versus 27%, p=0.16). The complex group had increased bleeding requiring re-exploration (0 versus 10%, p=0.04). Hospital and ICU length of stay did not differ. Complex patients had higher overall hospital costs (simple $80,749 versus complex $97,387, p=0.01) and higher postoperative costs (simple $60,192 versus complex $70,132, p=0.02). The operating room and supplies accounted for the majority of the cost difference., Conclusion: Complex arterial switches can be safely performed with low rates of morbidity and mortality but at an increased cost.

Background: Circulatory failure necessitating cardiac transplantation will ultimately develop in many patients with functional single-ventricle physiology. Interest in the use of mechanical circulatory support (MCS) in this population is growing., Methods: This was a retrospective case series of patients with functional single-ventricle physiology who underwent MCS with a ventricular assist device or a total artificial heart as a bridge to cardiac transplantation between January 2006 and December 2014. Baseline demographics, intraoperative data, postoperative complications, and outcome data were collected from the medical record., Results: MCS was used in 5 patients: HeartWare ventricular assist device (HeartWare International, Framingham, MA) in 1 patient, SynCardia total artificial heart (SynCardia Systems, Tucson, AZ) in 1, Thoratec Paracorporeal ventricular assist device (Thoratec Corp, Pleasanton, CA) in 1, and the Berlin Heart EXCOR (Berlin Heart Inc, The Woodlands, TX) in 2. The mean age at MCS was 12 ± 8 years. There were 2 early deaths at 12 and 28 days after MCS: 1 patient died of multiorgan system failure and 1 of neurologic injury. Overall, neurologic complications occurred in 3 patients (60%), and 1 patient (20%) required renal replacement therapy. Three patients (60%) underwent successful cardiac transplantation. The median time on the waiting list was 59 days (interquartile range, 18 to 126 days), and the median duration of MCS was 60 days (interquartile range, 28 to 93 days). At the time of transplant, all 3 patients were ambulatory, without the need for mechanical ventilation, and end-organ dysfunction had resolved. The 3 patients who received transplants were discharged from the hospital and were alive at an average follow-up of 9 ± 14 months., Conclusions: MCS can be successfully used as a bridge to transplantation in patients with a failing single-ventricle circulation. Use of MCS can allow for resolution of end-organ dysfunction and rehabilitation, leading to improved outcomes in this difficult population., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Based on outcome data, surgery is recommended for asymptomatic adults with chronic mitral regurgitation (MR) and systolic dysfunction, marked left ventricular (LV) dilation, pulmonary hypertension, atrial fibrillation, or high likelihood of successful repair; but indications for children are poorly defined. We sought to determine predictors of postoperative LV dysfunction in asymptomatic children with chronic MR. The surgical database was searched for all children who underwent mitral valve surgery for chronic MR (2000-2012). Exclusion criteria were preoperative symptoms, acute MR, cardiomyopathy, or other defects affecting LV size. Preoperative and latest follow-up clinical and echocardiographic data were obtained. LV dysfunction was defined as ejection fraction (EF) ≤55% or shortening fraction (SF) ≤28%. Associations between preoperative factors and late LV dysfunction were determined using univariate Poisson regression. For the 25 children who met criteria, preoperative median LV end systolic Z score (LVESZ) was 5.3, EF was 65%, and SF was 34%. At follow-up (median 3.9 years), nine patients (36%) had LV dysfunction. Lower preoperative SF (OR 0.6, p < 0.001) and higher LVESZ (OR 1.7, p < 0.01) were associated with late LV dysfunction. LVESZ ≥ 5 combined with SF ≤ 33% had a sensitivity of 89%, specificity of 88%, and negative predictive value of 93% for late LV dysfunction. Only 1/14 patients with preoperative SF > 33% had late LV dysfunction. For asymptomatic children with chronic MR, surgery should be considered before LVESZ exceeds five and SF falls below 33%. Patients with SF > 33% may be followed with serial echocardiographic measurements.

Neonates born with borderline left heart hypoplasia, or hypoplastic left heart complex, can undergo biventricular repair while those with severe left heart hypoplasia require single ventricle palliation. Deciding which patients are candidates for biventricular repair may be very difficult since there are no scoring systems to predict biventricular repair in these patients. The purpose of this study is to develop an echocardiographic scoring system capable of predicting successful biventricular repair in neonatal hypoplastic left heart complex. The study cohort consisted of twenty consecutive neonates with hypoplastic left heart complex presenting between 9/2008 and 5/2013. Multiple retrospective echocardiographic measurements of the right and left heart were performed. Six patients with significant LH hypoplasia (patent mitral and aortic valves, small left ventricle) who had undergone single ventricle repair were used to validate the scoring system. Seventeen patients underwent biventricular repair and three underwent single ventricle repair. A scoring system (2V-Score) was developed using the equation {[(MV4C/AVPSLA) ÷ (LV4C/RV4C)] + MPA}/BSA. Using a cutoff value of ≤ 16.2, a biventricular repair would have been predicted with a sensitivity of 1.0, specificity 1.0, positive predictive value 1.0, negative predictive value 1.0, area under the ROC curve 1.0, and the p value was 0.0004. The 2V-Score was more accurate than the Rhodes, CHSS, or Discriminant scores in retrospectively predicting biventricular repair in this cohort. The 2V-Score shows promise in being able to predict a successful biventricular repair in patients with hypoplastic left heart complex but requires prospective validation prior to widespread clinical application.

With surgical palliation of hypoplastic left heart syndrome (HLHS), the tricuspid valve (TV) becomes the systemic atrioventricular valve and moderate/severe TV insufficiency (TVI), an adverse risk factor for survival to Fontan, has been reported in up to 35% of patients prior to stage I palliation. Precise echocardiographic identification of the mechanism of TVI cannot be determined by two-dimensional echocardiography. Three-dimensional echocardiography (3DE) can provide significant insight into the mechanisms of TVI. It is the intent of this report to propose a systematic method on how to evaluate and display 3DE images of the TV in HLHS which has not been done previously. TV anatomy, function, and the known mechanisms of insufficiency are reviewed. We defined three regions of the TV (anterior, posterior, septal) that can help define valve "leaflets" that incorporates the many variations of TV anatomy. To determine how the surgeon views the TV, a picture of a pathologic specimen of the TV was placed on a computer screen and rotated until it was oriented as it appears during surgery, the "surgeons view." We have proposed a systematic method for evaluating and displaying the TV using 3DE which can provide significant insight into the mechanisms causing TVI in HLHS. This has the potential to improve both the surgical approach to repairing the valve and, ultimately, patient outcomes.

Decompression of the left heart in patients supported with extracorporeal membrane oxygenation (ECMO) is often warranted to protect the myocardium and facilitate recovery. We studied the ability of standard echocardiographic parameters to predict left atrial hypertension by reviewing 3 cardiac patients supported with ECMO who subsequently underwent left atrial decompression. We found that standard echocardiographic parameters poorly predict the need for left atrial decompression on ECMO. Following a more specific diagnostic algorithm to estimate left-sided filling pressure in patients with depressed ejection fraction may significantly improve the ability of echocardiography to accurately predict left atrial hypertension and the need for decompression.

Hypoplastic left ventricle with scimitar syndrome is a rare combination of anatomic lesions. Five patients with this anatomy have been followed up at our institution. Four of these patients are alive: 2 after heart transplant, 1 underwent a Norwood operation, and 1 has survived staged palliation, culminating in a Fontan operation., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Blunt traumatic injuries to the innominate artery are rare but potentially devastating injuries. Patients with an innominate injury who survive typically have an isolated intimal tear with an intact adventitia. There are multiple case reports and series describing off-pump repair of innominate injuries using synthetic grafts, and even reports of successful endovascular stenting. We report the first successful case of innominate artery disruption and repair using CorMatrix (CorMatrix Alpharetta, GA) extracellular matrix., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Roux-en-Y gastric bypass (RYGB) surgery has become an accepted treatment for excessive obesity. We conducted a longitudinal study to assess regional body composition, muscle proteolysis, and energy expenditure before RYGB, and 6 and 12 months after RYGB. Whole-body and regional fat mass (FM) and lean mass (LM) were assessed via dual energy X-ray absorptiometry (DXA), and myofibrillar protein degradation was estimated by urinary 3-methylhistidine (3-MeH) in 29 subjects. Energy expenditure and substrate oxidation were also determined using a whole-room, indirect calorimeter in 12 of these subjects. LM loss constituted 27.8 +/- 10.2% of total weight loss achieved 12 months postoperatively, with the majority of LM loss (18 +/- 6% of initial LM) occurring in the first 6 months following RYGB. During this period, the trunk region contributed 66% of whole-body LM loss. LM loss occurred in the first 6 months after RYGB despite decreased muscle protein breakdown, as indicated by a decrease in 3-MeH concentrations and muscle fractional breakdown rates. Sleep energy expenditure (SEE) decreased from 2,092 +/- 342 kcal/d at baseline to 1,495 +/- 190 kcal/day at 6 months after RYGB (P < 0.0001). Changes in both LM and FM had an effect on the reduction in SEE (P < 0.001 and P = 0.005, respectively). These studies suggest that loss of LM after RYGB is significant and strategies to maintain LM after surgery should be explored.

Background & Aims: Visceral adipose tissue (VAT) is an important risk factor for the metabolic complications associated with obesity. Therefore, a reduction in VAT is considered an important target of obesity therapy. We evaluated whether reducing VAT mass by surgical removal of the omentum improves insulin sensitivity and metabolic function in obese patients., Methods: We conducted a 12-month randomized controlled trial to determine whether reducing VAT by omentectomy in 22 obese subjects increased their improvement following Roux-en-Y gastric bypass (RYGB) surgery in hepatic and skeletal muscle sensitivity to insulin study 1. Improvement was assessed by using the hyperinsulinemic-euglycemic clamp technique. We also performed a 3-month, longitudinal, single-arm study to determine whether laparoscopic omentectomy alone, in 7 obese subjects with type 2 diabetes mellitus (T2DM), improved insulin sensitivity study 2. Improvement was assessed by using the Frequently Sampled Intravenous Glucose Tolerance Test., Results: The greater omentum, which weighed 0.82 kg (95% confidence interval: 0.67-0.97), was removed from subjects who had omentectomy in both studies. In study 1, there was an approximate 2-fold increase in muscle insulin sensitivity (relative increase in glucose disposal during insulin infusion) and a 4-fold increase in hepatic insulin sensitivity 12 months after RYGB alone and RYGB plus omentectomy, compared with baseline values (P<.001). There were no significant differences between groups (P>.87) or group x time interactions (P>.36). In study 2, surgery had no effect on insulin sensitivity (P=.844) or use of diabetes medications., Conclusions: These results demonstrate that decreasing VAT through omentectomy, alone or in combination with RYGB surgery, does not improve metabolic function in obese patients., (Copyright (c) 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.)

BACKGROUND/OBJECTIVES: Monitoring changes in total fat mass and abdominal adiposity are important in understanding the impact of different types of weight loss interventions on health risks. Our objective was to assess the usefulness of anthropometry and bioelectrical impedance analysis (BIA) in predicting fat mass changes during moderate weight loss. SUBJECTS/METHODS: Fat mass changes were assessed in 34 overweight adults (24 females, 10 males) after a 12-week supervised weight loss induced by caloric restriction (-30% of requirement) using BIA and DXA. Agreement between BIA and DXA measurements were assessed by Bland-Altman plots. Linear regression modeling was used to predict body and truncal fat mass from anthropometric measures. RESULTS: Diet intervention resulted in a significant decrease in body weight (- 7.86 ± 2.87 kg), body mass index (BMI - 2.69 ± 0.98 kg/m(2)), total body fat (- 5.22 ± 2.32 kg), truncal fat (- 2.80 ± 1.94 kg) and waist circumference (- 5.52 ± 3.57 cm). BMI and body weight were highly correlated with body fat (0.83 and 0.92 in females and 0.94 and 0.92 in males respectively) and truncal fat (0.75 and 0.87 in females; 0.90 and 0.84 in males respectively) during weight loss. Waist circumference was more correlated with truncal fat in males than females (0.94 vs. 0.85 in females). Compared to DXA, BIA underestimated total body fat changes in males (- 8.8 kg, p<0.001) and overestimated total body fat changes in females (+ 2.1 kg, p< 0.001). CONCLUSIONS: Body mass index, body weight, and waist circumference provide simple and more accurate than BIA estimates of relative changes in total and truncal fat during moderate weight loss in adults.

Background: Individuals with adjustable gastric bands experience plateaus in weight loss. Patients commonly attribute this to a "loosening" of their band with time. We sought to elucidate a physiologic mechanism for this pattern in patient behavior and describe the feasibility of a pressure-based adjustment algorithm for adjustable gastric bands., Methods: Following IRB protocol, 100 consecutive patients undergoing placement of the Lap-Band (Inamed) were enrolled and followed prospectively for 12 months. Intraband pressure measurements at band volumes 0 to 4 mL were recorded intraoperatively and at each subsequent band adjustment. Band adjustments were made using the currently accepted volume-based postoperative protocol., Results: Seventy-nine patients were included in analysis. Mean percent excess weight loss for the study cohort was 36 +/- 17% at a median followup of 347 days. During the time between adjustments, there was a statistically significant decrease (p < 0.001) in intraband pressure without a corresponding decrease in band volume. This was a result of a substantial change in the pressure-volume relationship of the Lap-Band. As time progressed, the Lap-Band developed less intraband pressure per unit volume. This change was not a result of changes in the elastic properties of the band material itself., Conclusions: Between adjustments, Lap-Band patients experience gradual loss of satiety and a loosening of their band, despite stable band volume. Their experience is substantiated by degradation in their intraband pressures with time. We have demonstrated that intraband pressures correlate with the patient's clinical history and have thereby established the foundation for a pressure-based adjustment protocol.