Long-Term Outcomes Following the Ross Procedure in Neonates and Infants: A Multi-Institutional Analysis.

OBJECTIVES: For neonates and infants with aortic valve pathology, the Ross procedure has historically been associated with high rates of morbidity and mortality. Data regarding long-term durability are lacking. METHODS: The international, multi-institutional Ross Collaborative included six tertiary-care centers. Infants who received a Ross operation between 1996-2016 (allowing a minimum five years of follow-up) were retrospectively identified. Serial echocardiograms were examined to study evolution in neoaortic size and function. RESULTS: Primary diagnoses for the 133 patients (n=30 neonates) included isolated aortic stenosis (AS; 14%, n=19), Shone complex (14%, n=19), and AS+other (excluding Shone complex; n=95, 71%) including arch obstruction (n=55), left ventricular hypoplasia (n=9), and mitral disease (>moderate stenosis or regurgitation, n=31). At the time of Ross, median age was 96 (IQR 36-186) days and median weight was 4.4 (3.6-6.5) kg. In-hospital mortality occurred in 13/133 (10%) patients (4/30 [13%] neonates). Post-discharge mortality occurred in 10/120 (8%) patients at a median 298 days post-Ross. Post-Ross neoaortic dilatation occurred, peaking at 4-5 standard deviations above normal at 2-3 years before returning to near-baseline z-score at a median follow-up of 11.5 [6.4-17.4] years. Autograft/LVOT reintervention was required in 5/120 (4%) patients at a median 10.3 [4.1-12.8] years. Freedom from >moderate neoaortic regurgitation (AR) was 86% at 15 years. CONCLUSIONS: Neonates and infants experience excellent post-discharge survival and long-term freedom from autograft reintervention and AR following Ross. Neoaortic dilatation normalizes in this population in the long-term. Increased consideration should be given to Ross in neonates and infants with aortic valve disease.

Achieving an Optimal Outcome After Stage 1 Palliation for Hypoplastic Left Heart Syndrome and Variants: Frequency, Associated Factors, and Subsequent Outcomes.

BACKGROUND: We sought to measure frequency of achieving an optimal outcome after stage 1 palliation (S1P) for hypoplastic left heart syndrome and variants, determine factors associated with optimal outcomes, and compare outcomes after stage 2 palliation (S2P) using the National Pediatric Cardiology Quality Improvement Collaborative database (2008-2016). METHODS AND RESULTS: This is a retrospective cohort study with optimal outcome defined a priori as meeting all of the following: (1) discharge after S1P in <19 days (top quartile), (2) no red flag or major event readmissions before S2P, and (3) performing S2P between 90 and 240 days of age. Optimal outcome was achieved in 256 of 2182 patients (11.7%). Frequency varied among centers from 0% to 25%. Factors independently associated with an optimal outcome after S1P were higher gestational age (odds ratio [OR], 1.1 per week [95% CI, 1.0-1.2]; P=0.02); absence of a genetic syndrome (OR, 2.5 [95% CI, 1.2-5]; P=0.02); not requiring a post-S1P catheterization (OR, 2.7 [95% CI, 1.5-4.8]; P=0.01), intervention (OR, 1.5 [95% CI, 1.1-2]; P=0.006), or a procedure (OR, 4.5 [95% CI, 2.8-7.1]; P<0.001) before discharge; and not having a post-S1P complication (OR, 2.7 [95% CI, 1.9-3.7]; P<0.001). Those with an optimal outcome after S1P had improved S2P outcomes including shorter length of stay, less ventilator days, shorter bypass time, and fewer postoperative complications. CONCLUSIONS: Identifying patients at lowest risk for poor outcomes during the home interstage period could shift necessary resources to those at higher risk, alter S2P postoperative expectations, and improve quality of life for families at lower risk.

Preoperative Hemodynamics Impact the Benefit of Fenestration on Fontan Postoperative Length of Stay.

BACKGROUND: Utilization of Fontan fenestration varies considerably by center. OBJECTIVES: Using a multicenter Pediatric Heart Network dataset linking surgical and preoperative hemodynamic variables, the authors evaluated factors associated with use of Fontan fenestration and the impact of fenestration on post-Fontan length of stay (LOS). METHODS: Patients 2 to 6 years old at Fontan surgery from 2010 to 2020 with catheterization<1 year prior were included. Factors associated with fenestration were evaluated using multivariable logistic regression adjusting for key covariates. Restrictive cubic spline analysis was used to evaluate potential cut-points for hemodynamic variables associated with longer postoperative LOS stratified by fenestration with multivariable linear regression to evaluate the magnitude of effect. RESULTS: Fenestration was used in 465 of 702 patients (66.2%). Placement of a fenestration was associated with center (range 27%-93% use, P < 0.0001) and Fontan type (OR: 14.1 for lateral tunnel vs extracardiac conduit, P < 0.0001). No hemodynamic variable was independently associated with fenestration. In a multivariable linear model adjusting for center, a center-fenestration interaction, prematurity, preoperative mean pulmonary artery pressure (mPAP), and cardiac index, fenestration was associated with shorter hospital LOS after Fontan (P = 0.0024). The benefit was most pronounced at mPAP ≥13 mm Hg (median LOS: 9 vs 12 days, P = 0.001). CONCLUSIONS: There is wide center variability in use of Fontan fenestration that is not explained by preoperative hemodynamics. Fenestration is independently associated with shorter LOS, and those with mPAP ≥13 mm Hg at pre-Fontan catheterization benefit the most. We propose this threshold as minimal criteria for fenestration.

Precision in Norwood Shunt Sizing: Single Ventricle Reconstruction Trial Public Dataset Analysis.

BACKGROUND: Morbidity and mortality after the Norwood procedure remains high. Shunt size selection is not standardized and the impact of shunt size on outcomes is poorly understood. The Single Ventricle Reconstruction trial randomized infants to modified Blalock-Taussig-Thomas shunt (MBTTS) or right ventricle-to-pulmonary artery shunt at the Norwood procedure. We assessed shunt size distribution and its association with postoperative outcomes. METHODS: We included 544 patients, excluding 5 with ambiguous shunt crossover data. Normalized shunt diameter 1 and 2 were calculated as shunt diameter divided by patient's weight and body surface area, respectively. The primary outcome was 30-day mortality after Norwood. Secondary outcomes were intensive care and total length of stay, and survival to Glenn procedure. Logistic and ordinal regression models evaluated the association of normalized shunt diameter with outcomes. RESULTS: Thirty-day mortality after Norwood was 11.4% (n = 62), survival to Glenn procedure was 72.6% (n = 395), median length of stay was 14.0 (interquartile range, 9.0-27.7) days and 24.0 (interquartile range, 16.0-41.0) days in the intensive care and total, respectively. Normalized shunt diameters exhibited variation in both shunt types but were not associated with 30-day mortality. Right ventricle-to-pulmonary artery shunt size was not associated with secondary outcomes. However, a MBTTS diameter ≥1.5 mm/kg predicted longer Norwood (odds ratio, 4.89; 95% CI, 1.41-16.90) and intensive care (odds ratio, 4.11; 95% CI, 1.25-13.49]) duration. CONCLUSIONS: Shunt size selection was variable. Right ventricle-to-pulmonary artery shunt had a wider size range seen with favorable outcomes compared with MBTTS. A MBTTS either too large or too small is associated with worse postoperative outcomes. Refining shunt sizing practices can improve surgical outcomes after the Norwood procedure.

Deployment of Point-of-Care Echocardiography to Improve Cardiac Diagnostic Access Among American Indians.

BACKGROUND: American Indians face significant barriers to diagnosis and management of cardiovascular disease. We sought to develop a real-world implementation model for improving access to echocardiography within the Indian Health Service, the American Indian Structural Heart Disease Partnership. METHODS AND RESULTS: The American Indian Structural Heart Disease Partnership was implemented and evaluated via a 4-step process of characterizing the system where it would be instituted, building point-of-care echocardiography capacity, deploying active case finding for structural heart disease, and evaluating the approach from the perspective of the clinician and patient. Data were collected and analyzed using a parallel convergent mixed methods approach. Twelve health care providers successfully completed training in point-of-care echocardiography. While there was perceived usefulness of echocardiography, providers found it difficult to integrate screening point-of-care echocardiography into their workday given competing demands. By the end of 12 months, 6 providers continued to actively utilize point-of-care echocardiography. Patients who participated in the study felt it was an acceptable and effective approach. They also identified access to transportation as a notable challenge to accessing echocardiograms. Over the 12-month period, a total of 639 patients were screened, of which 36 (5.6%) had a new clinically significant abnormal finding. CONCLUSIONS: The American Indian Structural Heart Disease Partnership model exhibited several promising strategies to improve access to screening echocardiography for American Indian populations. However, competing priorities for Indian Health Service providers' time limited the amount of integration of screening echocardiography into outpatient practice. Future endeavors should explore community-based solutions to develop a more sustainable model with greater impact on case detection, disease management, and improved outcomes.

Center-level factors associated with shorter length of stay following stage 1 palliation: An analysis of the national pediatric cardiology quality improvement collaborative registry.

BACKGROUND: Stage 1 single ventricle palliation (S1P) has the longest length of stay (LOS) of all benchmark congenital heart operations. Center-level factors contributing to prolonged hospitalization are poorly defined. METHODS: We analyzed data from infants status post S1P included in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry. Our primary outcome was patient-level LOS with days alive and out of hospital before stage 2 palliation (S2P) used as a balancing measure. We compared patient and center-level characteristics across quartiles for median center LOS, and used multivariable regression to calculate center-level factors associated with LOS after adjusting for case mix. RESULTS: Of 2,510 infants (65 sites), 2037 (47 sites) met study criteria (61% male, 61% white, 72% hypoplastic left heart syndrome). There was wide intercenter variation in LOS (first quartile centers: median 28 days [IQR 19, 46]; fourth quartile: 62 days [35, 95], P < .001). Mortality prior to S2P did not differ across quartiles. Shorter LOS correlated with more pre-S2P days alive and out of hospital, after accounting for readmissions (correlation coefficient -0.48, P < .001). In multivariable analysis, increased use of Norwood with a right ventricle to pulmonary artery conduit (aOR 2.65 [1.1, 6.37]), shorter bypass time (aOR 0.99 per minute [0.98,1.0]), fewer additional cardiac operations (aOR 0.46 [0.22, 0.93]), and increased use of NG tubes rather than G tubes (aOR 7.03 [1.95, 25.42]) were all associated with shorter LOS centers. CONCLUSIONS: Modifiable center-level practices may be targets to standardize practice and reduce overall LOS across centers.

Effects of a zero-gravity treadmill on body composition and cardiorespiratory fitness after Achilles surgery in a Masters runner.

Zero-gravity treadmills allow alterations in training load. Data are lacking on the utilization of this strategy to allow injured Masters-level athletes to return to activity and regain their fitness. A 6-week training program was designed for a 39-year-old male runner recovering from Achilles surgery using a zero-gravity treadmill. Three training sessions per week were performed with gradually increasing loads. Cardiopulmonary exercise testing and bioelectrical impedance analysis were performed before and after program completion. Following the training program, the athlete was able to return to full weight-bearing running. On cardiopulmonary exercise testing, there were improvements in peak oxygen consumption (42.9 vs 47.3 mL/min/kg; 118.6% vs 130.5% of predicted). On bioelectrical impedance analysis, there were small improvements in total weight, skeletal muscle mass, and adiposity felt to be within the standard of error for bioelectrical impedance analysis. In conclusion, load-altering exercise may be helpful for the Masters-level athlete recovering from Achilles tendon surgery.

Machine Learning to Predict Interstage Mortality Following Single Ventricle Palliation: A NPC-QIC Database Analysis.

There is high risk of mortality between stage I and stage II palliation of single ventricle heart disease. This study aimed to leverage advanced machine learning algorithms to optimize risk-prediction models and identify features most predictive of interstage mortality. This study utilized retrospective data from the National Pediatric Cardiology Quality Improvement Collaborative and included all patients who underwent stage I palliation and survived to hospital discharge (2008-2019). Multiple machine learning models were evaluated, including logistic regression, random forest, gradient boosting trees, extreme gradient boost trees, and light gradient boosting machines. A total of 3267 patients were included with 208 (6.4%) interstage deaths. Machine learning models were trained on 180 clinical features. Digoxin use at discharge was the most influential factor resulting in a lower risk of interstage mortality (p < 0.0001). Stage I surgery with Blalock-Taussig-Thomas shunt portended higher risk than Sano conduit (7.8% vs 4.4%, p = 0.0002). Non-modifiable risk factors identified with increased risk of interstage mortality included female sex, lower gestational age, and lower birth weight. Post-operative risk factors included the requirement of unplanned catheterization and more severe atrioventricular valve insufficiency at discharge. Light gradient boosting machines demonstrated the best performance with an area under the receiver operative characteristic curve of 0.642. Advanced machine learning algorithms highlight a number of modifiable and non-modifiable risk factors for interstage mortality following stage I palliation. However, model performance remains modest, suggesting the presence of unmeasured confounders that contribute to interstage risk.

Increasing Use of the Right Ventricle-to-Pulmonary Artery Shunt for Stage 1 Palliation.

BACKGROUND: Stage 1 palliation of hypoplastic left heart syndrome entails use of the Norwood operation with a modified Blalock-Taussig shunt or a right ventricle-to-pulmonary artery shunt, or the hybrid procedure. Use trends and factors influencing palliation selection remain unclear. We aimed to evaluate these questions and to compare outcomes between types of stage 1 palliation. METHODS: The National Pediatric Cardiology Quality Improvement Collaborative phase 1 (June 2008-August 2016) and phase 2 (August 2016-September 2019) databases were used. Procedure type was assessed by operation year. Baseline characteristics and annual hospital volume were evaluated. Postsurgical admission duration and outcomes were compared. RESULTS: A total of 3497 patients were included, 30.8% with modified Blalock-Taussig shunt, 59.7% with right ventricle-to-pulmonary artery shunt, and 9.5% with hybrid. Use of the right ventricle-to-pulmonary artery shunt increased over time (P = .02). This increase was similar among all hospital volumes. Higher hospital volume (odds ratio [OR], 1.2; 95% CI, 1.1-1.4; P = .003), male sex (OR, 1.3; 95% CI, 1.1-1.6; P = .01), and isolated cardiac disease (OR, 1.33; 95% CI, 1.01-1.55; P = .05) were associated with relatively higher likelihoods of a modified Blalock-Taussig shunt. Mortality/transplant rates before stage 2 palliation were higher with the modified Blalock-Taussig shunt than with the right ventricle-to-pulmonary artery shunt (12.3% vs 9.6%, P = .03). CONCLUSIONS: In stage 1 palliation, use of right ventricle-to-pulmonary artery shunts has increased over time, use of modified Blalock-Taussig shunts has decreased, and use of hybrids was unchanged. The modified Blalock-Taussig shunt has a higher likelihood of use in higher-volume centers, males, and less complex patients but is associated with longer hospitalizations and lower transplant-free survival to stage 2 palliation.

Cumulative comorbid conditions influence mortality risk after staged palliation for hypoplastic left heart syndrome and variants.

OBJECTIVE: Prematurity, low birth weight, genetic syndromes, extracardiac conditions, and secondary cardiac lesions are considered high-risk conditions associated with mortality after stage 1 palliation. We report the impact of these conditions on outcomes from a prospective multicenter improvement collaborative. METHODS: The National Pediatric Cardiology Quality Improvement Collaborative Phase II registry was queried. Comorbid conditions were categorized and quantified to determine the cumulative burden of high-risk diagnoses on survival to the first birthday. Logistic regression was applied to evaluate factors associated with mortality. RESULTS: Of the 1421 participants, 40% (575) had at least 1 high-risk condition. The aggregate high-risk group had lower survival to the first birthday compared with standard risk (76.2% vs 88.1%, P < .001). Presence of a single high-risk diagnosis was not associated with reduced survival to the first birthday (odds ratio, 0.71; confidence interval, 0.49-1.02, P = .066). Incremental increases in high-risk diagnoses were associated with reduced survival to first birthday (odds ratio, 0.23; confidence interval, 0.15-0.36, P < .001) for 2 and 0.17 (confidence interval, 0.10-0.30, P < .001) for 3 to 5 high-risk diagnoses. Additional analysis that included prestage 1 palliation characteristics and stage 1 palliation perioperative variables identified multiple high-risk diagnoses, poststage 1 palliation extracorporeal membrane oxygenation support (odds ratio, 0.14; confidence interval, 0.10-0.22, P < .001), and cardiac reoperation (odds ratio, 0.66; confidence interval, 0.45-0.98, P = .037) to be associated with reduced survival odds to the first birthday. CONCLUSIONS: The presence of 1 high-risk diagnostic category was not associated with decreased survival at 1 year. Cumulative diagnoses across multiple high-risk diagnostic categories were associated with decreased odds of survival. Further patient accrual is needed to evaluate the impact of specific comorbid conditions within the broader high-risk categories.

Association Between Seasonal Fontan Timing, Viral Infection Burden, and Post-Operative Length of Stay.

The Fontan procedure (FP) is typically a semi-elective surgery performed between 2 and 5 years of age to complete staged single ventricle palliation. Optimal timing for the FP, particularly in relation to seasonal infectious burden, remains unclear. We queried the Pediatric Health Information System (PHIS) database for all admissions for viral respiratory infections (VRI) from January 2006 to September 2015 and separately for all admissions with a primary procedure code of FP. The PHIS query generated 2,767,142 admissions for VRI and 6349 admissions for the FP from 45 children's hospitals. Of all FP, 2124 (33.5%) were performed from October through March. The median length of stay after Fontan procedure was 9 days (IQR 7-15). Median length of stay after FP was correlated with VRI burden (correlation coefficient = 0.3, p = 0.03). April through August (weeks 18 through 35) had the lowest VRI admission burden and FP length of stay was significantly shorter during this time (13.6 ± 14.8 days vs 14.9 ± 20.3 days, p = 0.03). The FP is frequently performed during the viral respiratory season. This timing is associated with an increased post-operative length of stay after the FP. For elective FP, ideal timing that avoids the viral respiratory season and minimizes post-operative LOS is April through August.

Congenital Heart Surgery Program Variable Case Mix: Risk Associated With Adjusted Mortality Rate.

BACKGROUND: Congenital heart operations are categorized into risk categories based on The Society of Thoracic Surgeons-European Association of Cardio-Thoracic Surgery Congenital Heart Surgery (STAT) Mortality Categories. The adjusted mortality rate should adjust for case mix. METHODS: The Society of Thoracic Surgeons Congenital Heart Surgery Public Reporting data were extracted for the top 50 U.S. News & World Report Cardiology and Heart Surgery Programs in 2021 (operations from January 2015 to December 2018). Variability in STAT 1 as a percentage of total operations and as a ratio to STAT 4+5 operations was evaluated. RESULTS: STAT 1 cases varied between centers from 18% to 37% of total. The ratio of STAT 1 to STAT 4+5 varied from 0.52 to 1.97. There was an inverse relationship between the STAT 1:STAT 4+5 ratio and adjusted mortality rate that did not reach statistical significance (P = .12). When programs (n = 12) in the quartiles with the highest vs lowest STAT 1:STAT 4+5 ratio were compared, a significant difference was found in the median adjusted mortality rate (2.2% vs 2.95%, P = .03). CONCLUSIONS: There is a 4-fold difference in the ratio of STAT 1 to STAT 4+5 cases among congenital heart surgery programs, even when smaller programs are excluded, suggesting significant differences in the decision-making philosophy. Programs with the highest proportion of STAT 1 cases had lower adjusted mortality rate, suggesting that The Society of Thoracic Surgery Congenital Heart Surgery Database mortality risk model adjusts well but not completely for case-mix variability between programs.

Patent Ductus Arteriosus Stent Versus Surgical Aortopulmonary Shunt for Initial Palliation of Cyanotic Congenital Heart Disease with Ductal-Dependent Pulmonary Blood Flow: A Systematic Review and Meta-Analysis.

Background In patients with ductal-dependent pulmonary blood flow, initial palliation includes catheter-based patent ductus arteriosus (PDA) stent or surgical aortopulmonary shunt (APS). This meta-analysis aimed to compare outcomes between PDA stent and APS. Methods and Results A comprehensive literature search yielded six retrospective observational studies. Pooled adjusted hazard ratios (HR) were included to control for covariates and assess time to event analysis. Of 757 patients, 243 (32.1%) underwent PDA stent and 514 (67.9%) underwent APS. Pulmonary atresia with intact ventricular septum and expected biventricular repair were more common with PDA stent compared with APS (39.6% versus 21.2%, P<0.001 and 57.9% versus 46.6%, P=0.007, respectively). There was no statistically significant difference in mortality between PDA stent and APS (HR, 0.71; [95% CI, 0.26-1.93]; P=0.50). PDA stent was associated with lower risk of postprocedural complications (odds ratio [OR], 0.45; [95% CI, 0.25-0.81]; P=0.008), mechanical circulatory support (OR, 0.27; [95% CI, 0.09-0.79]; P=0.02), and shorter intensive care unit length of stay (-4.03 days; [95% CI, -5.99 to -2.07]; P<0.001), hospital length of stay (-5.54 days; [95% CI, -9.20 to -1.88]; P=0.003), and duration of mechanical ventilation (-3.41 days; [95% CI, -5.29 to -1.52]; P<0.001). There was no difference in pulmonary artery growth or hazard of unplanned reintereventions. Conclusions PDA stent has a similar hazard of mortality compared with APS. Benefits to PDA stent include shorter duration of mechanical ventilation, shorter hospital length of stay, and fewer complications. Differences in patient characteristics exist with more patients with pulmonary atresia with intact ventricular septum and expected biventricular repair undergoing PDA stent.

Evaluation of an Outpatient and Telehealth Initiative to Reduce Tube Dependency in Infants with Complex Congenital Heart Disease.

Infants with congenital heart disease (CHD) often require supplemental nutrition via tube feeding, even after corrective surgical repair. The need for tube feeding can persist months after discharge home, and outpatient weaning from the tube can be a slow and difficult process. Lack of consensus exists in the literature as to the best approach to this challenge. We describe a single institution's initiative to decrease tube dependency after discharge through an intensive, telehealth-centric program for children with CHD. Of 16 children aged 1-26 months, 94% were able to be successfully tube weaned at home, with median time to tube-free of 12 days. No significant weight loss was noted during or after the wean process. Longer tube wean time was associated with older age at onset and comorbid genetic syndrome. Our telehealth-based intensive tube weaning program was successful but required a multidisciplinary team, targeted pre-participation patient evaluation, and frequent communication.

Quality Improvement Methodology Facilitates Adherence to Echocardiogram Protocol Measurements.

INTRODUCTION: Local institutional echocardiogram protocols reflect standard measurements as per national guidelines, but adherence to measurements was inconsistent. This inconsistency led to variability in reporting and impacted the use of serial measurements for clinical decision-making. Therefore, we aimed to improve complete adherence to universal and protocol-specific measures for echocardiograms performed for first-time or cardiomyopathy studies from 60% to 90% from July 2019 to February 2020. METHODS: We included all sonographer-performed echocardiograms for first-time or cardiomyopathy protocol studies. We reviewed universal measures and protocol-specific measures for all included studies. We created a scoring system reflecting measurement completion. We used a control chart to measure compliance and established a baseline over 2 months. PDSA cycles over 5 months included interventions such as sonographer education, technical improvements to the measurement toolbar, and group and individual performance feedback. RESULTS: We reviewed over 4000 studies-the reporting of complete universal measures improved significantly from a median score of 60% to 93%. Protocol-specific measures for first-time studies also showed significant improvement from 62% to 90% adherence. Cardiomyopathy-specific measures demonstrated 87% adherence at baseline, which improved to 95% but then returned to baseline. Sonographer education and toolbar adjustment prompted special cause variation with further improvement following performance feedback. The universal and first-time protocol measures reached 90% adherence with sustained improvement for over 9 months. CONCLUSIONS: We employed quality improvement methodology to improve complete adherence to echocardiographic protocol measurements, thereby facilitating echocardiographic quality and reporting consistency. We plan to spread these interventions to improve adherence to other protocols.

Decreased Global Myocardial Work Efficiency Correlates with Coronary Vasculopathy in Pediatric Heart Transplant Patients.

Coronary angiography remains the standard for diagnosis of cardiac transplant vasculopathy (CAV), but it is invasive. Non-invasively derived left ventricle (LV) global myocardial work (GMW) indices have not been evaluated. We aimed to assess for correlations between LV GMW and the presence of CAV in a pediatric population. 24 heart transplant patients and 24 normal controls were prospectively enrolled. Patients were age-matched into groups with: orthotopic heart transplant and CAV (OHT-CAV; 6 patients, 33% male, mean age 13.5 years [SD 4.2]), orthotopic heart transplant without CAV (OHT; 18 patients, 67% male, mean age 11.1 years [SD 4.8]), and normal healthy controls (42% male, mean age 12.8 years [SD 5.0]). Transplant patients underwent cardiac catheterization with coronary angiography within 3 months of echocardiogram. Post-processing of echocardiograms with speckle-tracking echocardiography and derivation of GMW indices was performed. OHT-CAV patients had decreased global work efficiency (GWE) compared to OHT (mean difference = 7.01 [1.76, 12.25], adjusted p < 0.01). LV global longitudinal strain (GLS) and LV ejection fraction were not different between groups. Both global work index and GWE were decreased in OHT-CAV and OHT when compared to normal controls (OHT-CAV 1311.23 mmHg% vs OHT 1426.22 mmHg% vs controls 1802.81 mmHg%, adjusted p < 0.01; OHT-CAV 83.87% vs. OHT 90.87% vs. controls 95.41%, adjusted p < 0.01). GWE correlated negatively with the presence of CAV (r = - 0.44 [- 0.72, - 0.05]). This pilot study demonstrates decreased GWE correlates with pediatric CAV. This supports the need for further investigation of this promising diagnostic tool.

Diastolic Function and Ambulatory Hypertension in Children With Chronic Kidney Disease.

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Impact of Digoxin Use on Interstage Outcomes of Single Ventricle Heart Disease (From a NPC-QIC Registry Analysis).

Digoxin has been associated with lower interstage mortality (ISM) following stage 1 palliation (S1P). Despite a substantial increase in digoxin use nationally, ISM has not declined. We aimed to determine the impact of digoxin on ISM in the current era. This study analyzed data from the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry. All patients who survived to hospital discharge following S1P were included. Comparisons were made between pre-specified eras (1: 2010-2015, 2: 2016-2019) based on digoxin use. ISM risk was estimated using the previously published NEONATE score (excluding digoxin). Multivariable Cox proportional hazard models assessed the impact of digoxin on ISM and freedom from unplanned readmission in era 2. A total of 1400 (46.8%) patients were included from era 1 and 1589 (53.2%) from era 2. Digoxin use (22.4% vs 61.7%, p < 0.001) and the proportion of high-risk patients (9.1% vs 20.3%, p < 0.001) increased across eras. There was no difference in predicted ISM risk between those who did vs did not receive digoxin in era 2 (p = 0.82). In era 2, digoxin use was independently associated with lower ISM (AHR 0.60, 95%CI 0.36 to 0.98, p = 0.043) and greater freedom from unplanned readmission (AHR 0.44, 95%CI 0.32 - 0.59, p < 0.001). In conclusion, digoxin is independently associated with lower ISM and greater freedom from interstage readmission. The lack of improvement in overall ISM in the current era may be secondary to a greater proportion of high-risk patients and/or disproportionately higher digoxin use in lower risk patients, who may not derive the same benefit.