The complex heterogeneity inherent to reparative procedures for congenital heart defects and their relatively small volumes makes comprehensive analyses of outcomes particularly challenging. That said, an incisive understanding of the impact of patient- and disease-specific factors is crucial to improving overall outcomes. Datasets that reflect 'real world' contemporary practice of congenital cardiac care and provide collective outcomes data across age groups and institutions in various parts of the world play a vital role in filling this gap. Risk-adjusted benchmark outcomes data offer opportunities to improve the quality and value of care provided at the level of an individual patient, an institution or program, and the overall specialty. On-going enhancements to data collected and their analyses will ensure that the care we provide continues to evolve. Future efforts should be aimed at integrating multiple datasets to ensure access to longitudinal follow-up and effective analysis of long-term outcomes.

Since its inception in 1994, The Society of Thoracic Surgeons Congenital Heart Surgery Database (CHSD) has established itself as the largest congenital and pediatric cardiothoracic surgical clinical data registry in the world, containing data from 677,684 operations as of 2023. The CHSD collects detailed clinical data and undertakes risk-stratified and risk-adjusted analysis using statistically robust high-fidelity risk models. The 37th data harvest analysis in Spring 2024 demonstrated an aggregate mortality of 2.6% in the CHSD. Survival in all benchmark operation groups continues to improve. An educational audit mechanism ensures the validity and accuracy of data collected in the CHSD. CHSD data continue to be used in important research endeavors that shape the practice of congenital cardiothoracic surgery. CHSD data fields and analytics are constantly refined to ensure that they remain contemporary. These efforts will continue to support quality and process improvement, ultimately allowing us to provide better care for our patients.

BACKGROUND:Children who undergo cardiac surgery may require postcardiotomy extracorporeal membrane oxygenation (ECMO). Although morbidities are considerable, our understanding of outcome determinants is limited. We evaluated associations between patient and perioperative factors with outcomes. METHODS:The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried for patients aged <18 years old who underwent postcardiotomy ECMO from January 2016 through June 2021. The primary outcome was survival to hospital discharge. The secondary outcome was survival without neurologic injury. Logistic regression for binary outcomes and competing risk analysis for survival were used to identify the most important predictors. Variables were selected by stepwise procedure using entry level P = .35. Those with P ≤ .1 were kept in the final model. RESULTS:Postcardiotomy ECMO was used to support 3181 patients during the same hospitalization as cardiac surgery: (A) intraoperative initiation of ECMO, n = 1206; (B) early postoperative (≤48 hours), n = 936; and (C) late postoperative (>48 hours), n = 1039. The most common primary procedure of the index operation was the Norwood procedure. Of those with intraoperative ECMO, 57% survived to discharge vs 59% with early postoperative ECMO and 42% late postoperative ECMO (χ2(2) = 64, P < .0001, V = 0.14). In all groups, postoperative septicemia, cardiac arrest, and new neurologic injury had the strongest association with mortality, whereas postoperative reintubation and unplanned noncardiac reoperation were associated with higher survival. CONCLUSIONS:Multiple risk factors impact survival in children who undergo cardiac surgery and postcardiotomy ECMO. ECMO initiated >48 hours after surgery is associated with the poorest outcomes. This is the first step in creating a predictive tool to educate clinicians and families regarding expectations in this high-risk population.

The management of hypoplastic left heart syndrome (HLHS) has improved substantially over the past few decades; yet long-term outcomes are sub-optimal. Transplant-free survival at 6 years is around 60%.1 Complications of the Fontan circulation continue to plague late outcomes in this and other single ventricle patient populations.2 This has led to a renewed attempt to establish biventricular circulation3 or alternatively, identify transplant candidates early to improve overall outcomes. Prior studies indicate some success in recruiting the hypoplastic left ventricle (LV) for eventual incorporation as the systemic ventricle.

The discovery of human pluripotent stem cells (hiPSCs) and advances in DNA editing techniques have opened opportunities for personalized cell-based therapies for a wide spectrum of diseases. It has gained importance as a valuable tool to investigate genetic and functional variations in congenital heart defects (CHDs), enabling the customization of treatment strategies. The ability to understand the disease process specific to the individual patient of interest provides this technology with a significant advantage over generic animal models. However, its utility as a disease-in-a-dish model requires identifying effective and efficient differentiation protocols that accurately reproduce disease traits. Currently, iPSC-related research relies heavily on the quality of cells and the properties of the differentiation technique In this review, we discuss the utility of iPSCs in bench CHD research, the molecular pathways involved in the differentiation of cardiomyocytes, and their applications in CHD disease modeling, therapeutics, and drug application.

Multiple databases are available related to pediatric and congenital cardiac care. The ability to utilize databases to compare risk-stratified and risk-adjusted outcomes has facilitated improved survival after surgery over the past decade. This chapter reviews eight key aspects of databases for pediatric and congenital cardiac care and reflects on the role artificial intelligence (AI) may play in each.

Objective: Pulmonary arterioplasty (PA plasty) at bidirectional cavopulmonary anastomosis (BDCA) is associated with increased morbidity, but outcomes to final stage palliation are unknown. We sought to determine the influence of PA plasty on pulmonary artery growth and hemodyamics at Fontan. Methods: We retrospectively reviewed clinical data and outcomes for BDCA patients from 2006 to 2018. PA plasty was categorized by extent (type 1-4), as previously described. Outcomes included pulmonary artery reintervention and mortality before final palliation. Results: Five hundred eighty-eight patients underwent BDCA. One hundred seventy-nine patients (30.0%) underwent concomitant PA plasty. Five hundred seventy (97%) patients (169 [94%] PA plasty) survived to BDCA discharge. One hundred forty out of 570 survivors (25%) required PA/Glenn reintervention before final stage palliation (59 out of 169 [35%]) PA plasty; 81 out of 401 (20%) non-PA plasty; P < .001). Twelve-, 24-, and 36-month freedom from reintervention after BDCA was 80% (95% CI, 74-86%), 75% (95% CI, 69-82%), and 64% (95% CI, 57-73%) for PA plasty, and 95% (95% CI, 93-97%), 91% (95% CI, 88-94%), and 81% (95% CI, 76-85%) for non-PA plasty (P < .001). Prefinal stage mortality was 37 (6.3%) (14 out of 169 PA plasty; 23 out of 401 non-PA plasty; P = .4). Five hundred four (144 PA plasty and 360 non-PA plasty) patients reached final stage palliation (471 Fontan, 26 1.5-ventricle, and 7 2-ventricular repair). Pre-Fontan PA pressure and pulmonary vascular resistance were 10 mm Hg (range, 9-12 mm Hg) and 1.6 mm Hg (range, 1.3-1.9 mm Hg) in PA plasty and 10 mm Hg (range, 8-12 mm Hg) and 1.5 mm Hg (range, 1.3-1.9 mm Hg) in non-PA plasty patients, respectively (P = .29, .6). Fontan hospital mortality, length of stay, and morbidity were similar. Conclusions: PA plasty at BDCA does not confer additional mortality risk leading to final palliation. Despite increased pulmonary artery reintervention, there was reliable pulmonary artery growth and favorable pulmonary hemodynamics at final stage palliation.

BACKGROUND:Chronic limb-threatening ischemia (CLTI) is associated with increased risk of major adverse cardiac and limb events (MACLE). In patients with peripheral arterial disease (PAD), evolocumab is associated with decreased MACLE, improved maximal walking time, increased vascular flow-mediated dilation (FMD), and decreased carotid intima-media thickness (IMT). We investigated the additive effect of evolocumab in patients with CLTI on maximally tolerated lipid lowering therapy after an index revascularization for non-healing wounds. METHODS:This double-blind, prospective, randomized, single-center, placebo-controlled study investigated the effect of monthly evolocumab for 6 months compared to placebo in patients with CLTI, with recent revascularization, and on maximally tolerated statin therapy. The primary endpoint was the reduction of low-density lipoprotein cholesterol (LDL-C) at 6 months of therapy. Secondary endpoints included evaluation of FMD and carotid IMT. RESULTS:N = 13 in the evolocumab arm and N = 14 in the placebo arm. The evolocumab arm had a lower mean LDL-C level at 3 months and 6 months compared to baseline (mean 82 mg/dL at baseline, 26 mg/dL at 3 months, and 34 mg/dL at 6 months, p = 0.017). The placebo arm had no significant difference in LDL-C at 3 and 6 months compared to baseline. Treatment with evolocumab resulted in increased FMD of the brachial artery and decrease in carotid IMT at 6 months. There was no deleterious effect on wound healing. CONCLUSION:Evolocumab was associated with a decrease in LDL-C, decreased carotid IMT, and improved FMD in CLTI patients who were on evolocumab therapy in addition to maximally tolerated statin. There was no adverse effect on wound healing with further reduction in LDL-C.

Congenital Heart Surgery Machine Learning-Derived In-Depth Benchmarking ToolThe Annals of Thoracic SurgeryPreviewWe previously showed that machine learning-based methodologies of optimal classification trees (OCTs) can accurately predict risk after congenital heart surgery and assess case-mix–adjusted performance after benchmark procedures. We extend this methodology to provide interpretable, easily accessible, and actionable hospital performance analysis across all procedures. Full-Text PDF

The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database (CHSD) continues to be the most comprehensive database of congenital and pediatric cardiothoracic surgical procedures in the world and contains information on 664,210 operations as of June 30, 2023. The 35th harvest of the STS CHSD data was undertaken in Spring 2023, spanning the 4 -year period January 1, 2019, through December 31, 2022, and included 144,919 operations performed at 114 participating sites in North America. The harvest analysis was successfully executed by the STS Research and Analytic Center. The overall unadjusted mortality rate was 2.68% and has remained stable over the 4 years included in the current harvest window. Mortality is highest in neonates (7.4%) and lowest in children (1.1%). As in prior analyses, observed mortality and postoperative length of stay in the database increase with an increase in STS-European Association for Cardio-Thoracic Surgery (STAT) Congenital Heart Surgery Mortality Categories. This quality report summarizes contemporary outcomes, provides the odds ratios for the CHSD risk model variables based on this analysis, and describes on -going efforts to improve data collection and augment analytical approaches. Lastly, 5 research publications completed in the last year using data from the CHSD are also summarized. (Ann Thorac Surg 2024;117:904-14) (c) 2024 by The Society of Thoracic Surgeons. Published by Elsevier Inc.

BACKGROUND We sought to evaluate whether the anatomic and physiologic stratification system (ACAP score), released as part of the American College of Cardiology/American Heart Association updated guidelines for management of adult congenital heart disease (ACHD) in 2018, better estimated mortality and morbidity after cardiac operations for ACHD. METHODS The ACAP score was determined for 318 patients (age double dagger 18 years) with ACHD undergoing heart surgery at our institution between December 2001 and August 2019. The primary end point was perioperative mortality. The secondary aim was to evaluate the performance of the ACAP, The Society of Thoracic Surgeons -European Association for Cardio-Thoracic Surgery (STAT) Congenital Heart Surgery Mortality Categories, and ACHS mortality scores/categories at predicting a composite adverse outcome of perioperative mortality, prolonged ventilation, and renal failure requiring replacement therapy. Logistic regression models were built to estimate mortality and the composite outcome using anatomic and physiologic components independently and together. Receiver operating characteristic curves were created, and area under the curves were compared using the Delong test. RESULTS The median age was 37 years (interquartile range, 26.3-50.0 years). There were 9 perioperative mortalities (2.8%). With respect to perioperative mortality, the area under the curve using the anatomic component only was 0.74, which improved to 0.81 after including physiologic severity ( P = .05). When physiologic severity was added to the model for the composite outcome, the discriminatory abilities of the ACHS mortality score and the STAT categories increased significantly to 0.83 (95% CI, 0.75-0.91; P = .02) and 0.82 (95% CI, 0.73-0.90; P = .04), comparable to the predictive power of ACAP. CONCLUSIONS Physiologic severity augments ability to predict mortality and morbidity after cardiac surgery for ACHD. There is need for more robust ACHD-specific risk models. (Ann Thorac Surg 2024;117:804-12) (c) 2024 by The Society of Thoracic Surgeons. Published by Elsevier Inc.

Background The Society of Thoracic Surgeons Workforce on Congenital Surgery performed a practice survey to analyze contemporary data. Methods An electronic survey was sent to congenital heart surgeons in North America. Details on demographics, training paradigm, clinical practice, and work satisfaction were queried, tabulated, and analyzed. Results Of 312 unique contacts, 201 (64.4%) responded. Of these, 178 (89%) were practicing. The median age was 52 years (interquartile range, 43, 59 years), and 157 (88%) were male. The number of female respondents increased from 12 (7%) in 2015 to 18 (11%) at present. Practice composition was predominantly mixed pediatric and adult (141; 79%), although 15 (8%) surgeons practiced exclusively pediatric surgery. Most surgeons (154; 87%) reported performing the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category 4 and 5 procedures. One-fourth (42; 24%) reported performing fewer than 50 pediatric cases per year, and 18 (10%) stated that their primary role was as a surgical assistant. Individual surgeon case volume was most commonly 100 to 149 total cases (29%). Although one-half (91; 51%) reported their volume as being “just right,” 74 (42%) reported that their case volume was “too small.” Seventy-six (43%) reported too many surgeons in their region. Of the 201 practicing surgeons, 30 (14.9%) plan retirement in the next 5 years. Most described career satisfaction, with 102 (57%) being very satisfied and 48 (27%) somewhat satisfied. Conclusions Although most congenital heart surgeons in North America are satisfied with their careers, more than 40% believe that their caseload is inadequate and that there are too many surgeons in their region. Further analysis is warranted regarding career dissatisfaction and diversity.

Objective: To test the hypothesis that in patients with peripheral arterial disease (PAD) and claudication, treated with maximal tolerated statin therapy, the addition of a monthly subcutaneous injection of evolocumab for 6 months improves treadmill walking performance. Background: Lipid lowering therapy improves walking parameters in patients with PAD and claudication. Evolocumab decreases cardiac and limb adverse events in patients with PAD; however, the effect of evolocumab on walking performance is not known. Methods: We performed a double-blind, randomized, placebo-controlled study to compare maximal walking time (MWT) and pain free walking time (PFWT) in patients with PAD and claudication treated with monthly subcutaneous injections of evolocumab 420 mg (n = 35) or placebo (n = 35). We also performed measurements of lower limb perfusion, brachial flow mediated dilatation (FMD), carotid intima media thickness (IMT), and serum biomarkers of PAD disease severity. Results: After six-months of treatment with evolocumab MWT increased by 37.7 % (87.5 +/- 24 s) compared to 1.4 % (-21.7 +/- 22.9 s) in the placebo group, p = 0.01. PFWT increased by 55.3 % (67.3 +/- 21.2 s) in the evolocumab group compared to 20.3 % (8.5 +/- 20.3 s) in the placebo group, p = 0.051. There was no difference in lower extremity arterial perfusion measurements. FMD increased by 42.0 +/- 73.9 % (1.01 +/- 0.7 %) in the evolocumab group and decreased by 16.29 +/- 20.06 % (0.99 +/- 0.68 %) in the placebo group (p < 0.001). IMT decreased by 7.16 +/- 4.6 % (0.06 +/- 0.04 mm) in the evolocumab group and increased by 6.68 +/- 4.9 % (0.05 +/- 0.03 mm) in the placebo group, (p < 0.001). Conclusions: The addition of evolocumab to maximal tolerated statin therapy improves maximal walking time in patients with PAD and claudication, increases FMD, and decreases IMT. Condensed abstract: Peripheral arterial disease (PAD) impairs quality of life by causing lower extremity intermittent claudication, rest pain, or amputation. Evolocumab is a monthly injectable monoclonal antibody medication that reduces cholesterol. In this study, we randomly treated patients with PAD and claudication, and on background statin therapy, with evolocumab or placebo, and found that evolocumab improves walking performance on a treadmill test by increasing maximal walking time. We also found that evolocumab decreases plasma MRP-14 levels, a marker of PAD severity. (c) 2023 Published by Elsevier Inc.

We congratulate Goyal and colleagues1 for their cohort study of 647 patients undergoing single-stage truncus arteriosus communis (TAC) repair between 1982 and 2011 reported to the Pediatric Cardiac Care Consortium registry. The goal of their study was “to describe in-hospital and long-term mortality following single-stage repair of TAC and explore factors associated with these outcomes.” Long-term mortality was obtained by matching with the National Death Index (NDI). Of 647 patients who underwent single-stage TAC repair (median age, 18 days), 486 (75%) survived to hospital discharge, and 215 (operated on before April 15, 2003) had identifiers for tracking long-term outcomes; 30-year survival was 78%.