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Competing Interests: Disclosures The authors have no conflicts of interest to disclose.

Background: Although coarctation of the aorta without concomitant intracardiac pathology is relatively common, there is lack of guidance regarding aspects of its management in neonates and infants., Methods: A panel of experienced congenital cardiac surgeons, cardiologists, and intensivists was created, and key questions related to the management of isolated coarctation in neonates and infants were formed using the PICO (Patients/Population, Intervention, Comparison/Control, Outcome) Framework. A literature search was then performed for each question. Practice guidelines were developed with classification of recommendation and level of evidence using a modified Delphi method., Results: For neonates and infants with isolated coarctation, surgery is indicated in the absence of obvious surgical contraindications. For patients with risk factors for surgery, medical management before intervention is reasonable. For those stable off prostaglandin E 1 , the threshold for intervention remains unclear. Thoracotomy is indicated when arch hypoplasia is not present. Sternotomy is preferable when arch hypoplasia is present that cannot be adequately addressed through a thoracotomy. Sternotomy may also be considered in the presence of a bovine aortic arch. Antegrade cerebral perfusion may be reasonable when the repair is performed through a sternotomy. Extended end-to-end, arch advancement, and patch augmentation are all reasonable techniques., Conclusions: Surgery remains the standard of care for the management of isolated coarctation in neonates and infants. Depending on degree and location, arch hypoplasia may require a sternotomy approach as opposed to a thoracotomy approach. Significant opportunities remain to better delineate management in these patients., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

In this invited expert review, we focus on evolving lifetime management strategies for adolescents and young adults with congenital aortic valve disease, acknowledging that these patients often require multiple interventions during their lifetime. Our goal is to preserve the native aortic valve when feasible. Leveraging advanced multimodality imaging, a detailed assessment of the aortic valve and root complex can be obtained, and a surgically approach tailored to an individual patient's anatomy and pathology can be used. In turn, aortic valve repair and reconstruction can be offered to a greater number of patients, either as a definitive strategy or as a component of a staged strategy to delay the need for aortic valve replacement until later in life when more options are available., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Although anomalous aortic origin of a coronary artery (AAOCA) is associated with risk of sudden cardiac arrest (SCA), there is a spectrum of disease, with the appropriate management for many remaining unclear. Increasing data warrant review for an updated perspective on management., Methods: A panel of congenital cardiac surgeons, cardiologists, and imaging practitioners reviewed the current literature related to AAOCA and its management. Survey of relevant publications from 2010 to the present in PubMed was performed., Results: The prevalence of AAOCA is 0.4% to 0.8%. Anomalous left coronary artery is 3 to 8 times less common than anomalous right coronary, but carries a much higher risk of SCA. Nevertheless, anomalous right coronary is not completely benign; 10% demonstrate ischemia, and it remains an important cause of SCA. Decision-making regarding which patients should be recommended for surgical intervention includes determining anatomic features associated with ischemia, evidence of ischemia on provocative testing, and concerning cardiovascular symptoms. Ischemia testing continues to prove challenging with low sensitivity and specificity, but the utility of new modalities is an active area of research. Surgical interventions focus on creating an unobstructed path for blood flow and choosing the appropriate surgical technique given the anatomy to accomplish this. Nontrivial morbidity has been reported with surgery, including new-onset ischemia., Conclusions: A proportion of patients with AAOCA demonstrate features and ischemia that warrant surgical intervention. Continued work remains to improve the ability to detect inducible ischemia, to risk stratify these patients, and to provide guidance in terms of which patients warrant surgical intervention., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Pulmonary artery banding (PAB) in isolation or combined with a congenital cardiac surgical procedure is common and has important mortality. We aimed to determine patient characteristics, clinical outcomes, variation in clinical outcomes by diagnoses, and center variation in PAB use., Methods: Using The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD), this study evaluated outcomes of patients undergoing PAB across diagnoses, participating centers, and additional procedures. Patients were identified by procedure and diagnosis codes from 2016 to 2019. We separated patients into groups of main and bilateral PAB and described their outcomes, focusing on patients with main PAB., Results: This study identified 3367 PAB procedures from 2016 to 2019 (3% of all STS CHSD cardiovascular cases during this period): 2677 main PAB, 690 bilateral PAB. Operative mortality was 8% after main PAB and 26% after bilateral PAB. There was significant variation in use of main PAB by center, with 115 centers performing at least 1 main PAB procedure (range, 1-134; Q1-Q3, 8-33). For patients with main PAB, there were substantial differences in mortality, depending on timing of main PAB relative to other procedures. The highest operative mortality (25%; P < .0001) was in patients who underwent main PAB after another separate procedure during their admission, with extracorporeal membrane oxygenation being the most frequent preceding procedure., Conclusions: PAB is a frequently used congenital cardiac procedure with high mortality and variation in use across centers. Outcomes vary widely by banding type and patient diagnosis. Main PAB after cardiac surgical procedures, especially extracorporeal membrane oxygenation, is associated with very high operative mortality., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: In 2007, congenital cardiac surgery became a recognized fellowship by the American Council of Graduate Medical Education (ACGME). Beginning in 2023, the fellowship transitioned from a 1-year to a 2-year program. Our objective is to provide current benchmarks by surveying current training programs and assessing characteristics contributing to career success., Methods: This was a survey-based study in which tailored questionnaires were distributed to program directors (PDs) and graduates of the ACGME accredited training programs. Data collection included responses to multiple-choice and open-ended questions relevant to didactics, operative training, training center characteristics, mentorship, and employment characteristics. Results were analyzed using summary statistics and subgroup and multivariable analyses., Results: The survey yielded responses from 13 of 15 PDs (86%) and 41 of 101 graduates (41%) from ACGME accredited programs. Perceptions among PDs and graduates were somewhat discordant, with PDs more optimistic than graduates. Of PDs, 77% (n = 10) believed current training adequately prepares fellows and is successful in securing employment for graduates. The responses from graduates demonstrated 30% (n = 12) were dissatisfied with operative experience and 24% (n = 10) with overall training. Being supported during the first 5 years of practice was significantly associated with retention in congenital cardiac surgery and greater practicing case volumes., Conclusions: Dichotomous views exist between graduates and PDs regarding success in training. Mentorship during the early career was associated with increased case volumes, career satisfaction, and retention in the congenital cardiac surgery field. Educational bodies should incorporate these elements during training and after graduation., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Studies examining the volume-outcome relationship in congenital heart surgery (CHS) are more than a decade old. Since then, mortality has declined, and case-mix adjustment has evolved. We determined the current relationship between hospital CHS volume and outcomes., Methods: Patients aged ≤18 years undergoing index operations in The Society of Thoracic Surgeons-Congenital Heart Surgery Database (2017-2020) were included. Associations between annual hospital volume and case-mix-adjusted operative mortality, major complications, failure to rescue (FTR), and postoperative length of stay (PLOS) were assessed using Bayesian hierarchical models, overall, by The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category, and for the Norwood procedure., Results: Across 101 centers (76,714 index operations), median annual volume was 144 operations/y. Operative mortality was 2.7%. Lower-volume hospitals had higher mortality, with an apparent transition zone at ∼190 operations/y (95% credible interval [CrI], 115-450 operations/y), below which a sustained uptick in the estimated odds of death occurred. Odds of death compared with a 450 operations/y reference were 50 operations/y (odds ratio [OR], 1.84; 95% CrI, 1.41-2.37), 100 operations/y (OR, 1.37; 95% CrI, 1.08-1.71), 200 operations/y (OR, 0.92; 95% CrI, 0.1-1.18), 300 operations/y (OR, 0.89; 95% CrI, 0.76-1.04). The volume-outcome effect was more apparent for STAT 4 to 5 than STAT 1 to 3 operations. In the overall cohort, PLOS and complications were similar across hospital volumes, whereas FTR rates were higher at lower-volume hospitals. Lower-volume hospitals had worse outcomes after the Norwood procedure, most notably mortality and FTR., Conclusions: Hospital volume is associated with mortality and FTR after CHS. The relationship is strongest for high-risk operations. These data can inform ongoing initiatives to improve CHS care., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains., (Copyright © 2023 The Society of Thoracic Surgeons, The American Association For Thoracic Surgery, and World Society for Pediatric and Congenital Heart Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Relationships between pulmonary hypertension (PHTN), mitral valve intervention (MVI), and death among patients with Shone's syndrome (SS) are unclear. Our recent data suggested temporal progression of MV disease and early survival benefit with MVI at initial operation. In this expanded cohort, we characterized trajectory and impact of PHTN on survival, and its relationship to MVI(s)., Methods: Record review of 219 SS patients undergoing operation at our quaternary center between 1956-2022, 111 of whom were pediatric (< age 18 yrs.). Patients were determined to have PHTN using a time-defined hierarchical algorithm consisting of cardiac catheterization data, echocardiographic parameters, pulmonary vasodilator therapy, and record-verified PHTN diagnosis. Analysis includes risk-adjusted predicted survival and freedom from PHTN curves stratified by MVI timing., Results: Among 219 patients, 92 (43%) had mitral valve stenosis on presentation, with mean gradients of 3.5 (IQR 4.7). Sixty patients had at least one MVI (15 (6%) and 8 (3%) had 3 or more). Median follow-up was 19 years (IQR 29) with 18 (8%) deaths. Ninety-three (42%) patients developed PHTN, with 50% and 25% freedom from PHTN at 37 and 51 years from presentation. PHTN was strongly associated with death (p <0.001). In unadjusted analyses, MVI, even if successive, delayed the onset of PHTN at 10 years and improved survival (p<0.001). However, this protective effect was lost after risk-adjustment., Conclusions: PHTN development in SS is progressive and mirrors the survival curve, suggesting it is a major mortality risk-factor. Salutary effects of early and/or successive MVI are reduced after risk-adjustment., (Copyright © 2023. Published by Elsevier Inc.)

Background: The Ozaki procedure using autologous pericardium is an interesting but complex alternative for aortic valve replacement. We present a standardized approach to minimize the learning curve and confirm reproducibility., Methods: After careful preparation, from May 2015 to February 2021, an Ozaki procedure was performed on 46 patients age 51 ± 14 years. Seven had unicuspid (15%), 29 bicuspid (63%), and 10 tricuspid (22%) aortic valves, and 2 patients had endocarditis. Endpoints were operative learning curves, perioperative outcomes, intermediate-term valve hemodynamics, reintervention, health-related quality of life (MacNew Heart Disease Health-Related Quality of Life questionnaire), and mortality., Results: Cardiopulmonary bypass and aortic clamp times decreased from 145 to 125 ​minutes and 120 to 100 ​minutes, respectively, over the first 20 cases, reflecting the learning curve. There was no major perioperative morbidity or mortality. Median postoperative stay was 6.9 days. Aortic regurgitation was mild or less in all but 2 patients who developed moderate aortic regurgitation. Mean aortic valve gradient was 7.9 mmHg postoperatively, 9.2 mmHg by 6 months, and constant thereafter. Left ventricular ejection fraction was 58% preoperatively, 60% at 6 months, and remained stable thereafter. One patient developed infective endocarditis 7 months postoperatively, failed medical management, and underwent valve replacement at 14 months. Two-year survival was 96%, with 1 noncardiac death at 16 months. Health-related quality of life in mental, physical, and emotional domains was better than matched norms, global 6.2 vs. 5.0 ( p < 0.0001)., Conclusions: Using a well-prepared standardized approach, the Ozaki procedure is reproducible with a short learning curve, excellent hemodynamic performance, and good quality of life., Competing Interests: Dr Blackstone had statistical consultation related to valve trials with Edwards Lifesciences and Abiomed. The other authors had no conflicts to declare., (© 2023 The Author(s).)

Competing Interests: Disclosures The author has no conflicts of interest to disclose.

Background: Operative mortality risk models for adults with congenital heart disease (ACHD) undergoing cardiac operations are essential, given the growing population of these patients, yet they are currently unavailable. Existing adult Society of Thoracic Surgeons (STS) models exclude congenital procedures, whereas existing congenital models exclude operations for acquired disease. We aimed to develop an STS mortality risk model for ACHD patients undergoing cardiac operations., Methods: Leveraging a comprehensive list of diagnostic and procedure codes, ACHD patients who underwent cardiac operations were identified from the STS Adult Cardiac Surgery Database (versions: v2.73, v2.81, and v2.9) between 2011 and 2019. The model was developed and validated in the ACHD population using a 60/40 development/validation split. Univariate analyses and clinical expertise informed the addition of ACHD-relevant procedure and diagnosis variables to existing STS adult risk model variables. Model performance was assessed overall and in 38 subgroups based on patient demographics, procedures, and diagnoses., Results: Forty-seven procedure and diagnosis variables relevant to ACHD were added to existing STS adult risk model variables. The derived ACHD model for operative mortality was well calibrated within demographic, procedural, and diagnosis subgroups and the overall ACHD population, and discrimination in the validation cohort was excellent (C statistic, 0.815) compared with the model using only existing STS adult risk model variables (C statistic, 0.79; P < .0001)., Conclusions: A novel, high-performing STS ACHD mortality risk model has been developed on the basis of contemporary patient data. The ACHD risk model represents an important expansion of the STS portfolio. Implementation with an online risk calculator is planned., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We have previously described our novel surgical technique in addressing the anomalous aortic origin of the left coronary artery with a transseptal course behind the right ventricular outflow tract. By means of the Najm procedure, we performed complete unroofing of the transseptal anomalous aortic origin of the left coronary artery and elongated the right ventricular outflow tract posteriorly using an autologous pericardial patch. This report documents modifications to address challenging anatomic variants and highlights the importance of managing septal arteries and myocardial bridges. We also describe the excellent midterm outcomes of 14 patients who have undergone the Najm procedure., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: As a formative step toward development of adult congenital heart disease (ACHD) risk-adjusted metrics, we describe The Society of Thoracic Surgeons (STS) ACHD population, procedural frequency, and early mortality., Methods: Adults (≥18 years) with CHD (2014-2019) were identified in the STS Adult Cardiac Surgery (ASCD) and the Congenital Heart Surgery (CHSD) Databases. After deduplication, variable mapping, data concatenation, and harmonization of preprocedure factors, procedures were grouped, and unadjusted mortality was catalogued for the overall cohort and the cohort excluding patients with an isolated bicuspid aortic valve (BAV)., Results: Among 171 186 ACSD and 18 281 CHSD records, 152 731 unique records met inclusion criteria. Twenty-eight congenital diagnoses accounted for 86% of the overall cohort, and prevalence of ACHD increased over the study time frame. ACHD patients underwent operations to treat both acquired and CHD. Most common procedures overall and after excluding isolated BAV were aortic valve replacement (AVR), ascending aortic surgery, and coronary artery bypass grafting (CABG). After excluding isolated BAV, major procedure combinations occurred in 46% (31% had 2 major procedures and 3% had ≥4), and the most prevalent combinations included AVR + CABG (n = 2352), AVR + subaortic stenosis repair (n = 1481), and AVR + ascending aortic surgery (n = 1239). Unadjusted 30-day mortality was procedure dependent, 2.8% overall and 3.6% with isolated BAV excluded., Conclusions: The ACHD surgical population is heterogenous, and patients undergo surgery for CHD-related and adult/acquired procedure combinations. Early mortality is variable and influenced by surgical complexity. Excluding isolated BAV patients and developing procedure-based ACHD mortality risk models may be ideal but will require empirically derived grouping and collaboration., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.)

Background: Socioeconomic and racial (SER) disparities among patients with congenital heart disease (CHD) may limit access to high-quality care. We characterized the national SER landscape and its relationship to early outcomes and identified interactions among determinants mitigating adverse outcome., Methods: The Pediatric Health Information System (PHIS) database was queried for patients (age <26 years) with CHD between 2016 and 2018. International Classification of Diseases, 10th Revision, codes were mapped to diagnostic categories for complexity adjustment. Correlational and hierarchical regression analyses identified risk factors and characterized interactions., Results: We identified 166,599 unique admissions from 52 hospitals, with 58,395 having interventions. Median age was 0 years (interquartile range [IQR], 4 years). Race/ethnicity was predominantly White (59%), Hispanic (20%), and Black (16%). Median neighborhood household income (NHI) was $41,082 and varied among hospitals. Patient NHI had a parabolic relationship with mortality, with both higher and lower values having increased risk. Black patients had significantly higher death, and this relationship was potentiated by lower NHI and complexity. Hospital length of stay was longer among Black neonates (median, 51 days; IQR, 93 days) compared with neonates of other ethnic groups (median, 32 days; IQR, 71 days; P < .0001. Care pathways, including permanent feeding tubes, were also more prevalent among Black neonates (17.8%) compared with White neonates (15%; P = .02)., Conclusions: Interactions among SER disparities modify CHD outcomes. Specific hospitals have more SER fragile patients but may have developed care pathways that prolong length of stay to mitigate risk among Black neonates. Adverse outcomes among SER-disadvantaged patients are magnified in complex CHD, suggesting tangible benefits to targeted resource allocation and population health initiatives., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The Physician Payments Sunshine Act was enacted to understand financial relationships with industry that might influence provider decisions. We investigated how industry payments within the congenital heart community relate to experience and reputation., Methods: Congenital cardiothoracic surgeons and pediatric cardiologists were identified from the Open Payments Database. All payments from 2013 through 2017 were matched to affiliated hospitals' U.S. News & World Report (USNWR) rankings, The Society of Thoracic Surgeons-Congenital Heart Surgery Public Reporting Star Ratings, and Optum Center of Excellence (COE) designation. Surgeon payments were linked to years since terminal training. Univariable analyses were conducted., Results: The median payment amount per surgeon ($71; interquartile range [IQR], $41-$99) was nearly double the median payment amount per cardiologist ($41; IQR, $18-$84; P < .05). For surgeons, median individual payment was 56% higher to payees at USNWR top 10 children's hospitals ($100; IQR, $28-$203) vs all others ($64; IQR, $23-$140; P < .001). For cardiologists, median individual payment was 26% higher to payees at USNWR top 10 children's hospitals ($73; IQR, $28-$197) vs all others ($58; IQR, $19-$140; P < .001). Findings were similar across The Society of Thoracic Surgeons-Congenital Heart Surgery star rankings and Optum Center of Excellence groups. By surgeon experience, surgeons 0 to 6 years posttraining (first quartile) received the highest number of median payments per surgeon (17 payments; IQR, 6.5-28 payments; P < .001). Surgeons 21 to 44 years posttraining (fourth quartile) received the lowest median individual payment ($51; IQR, $20-132; P < .001)., Conclusions: Industry payments vary by hospital reputation and provider experience. Such biases must be understood for self-governance and the delineation of conflict of interest policies that balance industry relationships with clinical innovation., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden death in youth. However, its significance and optimal management in adults is poorly understood. Our objective is to characterize AAOCA in a large single-center adult cohort based on coronary anatomic variants and surgical management strategies., Methods: We reviewed imaging, clinic, and operative reports for 645 adults with an encounter diagnosis code of congenital coronary anomaly from July 2015 to July 2017. After excluding other congenital heart defects, we characterized 167 patients with AAOCAs by anatomic variant, symptoms at diagnosis, indication for advanced imaging, and if performed, surgical repair. To describe the anatomic variant, we classified the origin and course by following the atomization scheme developed by the Congenital Heart Surgeon's Society's AAOCA registry., Results: Among adults with AAOCA, the anomalous origin involved the right coronary artery in 57% (96 of 167), left main coronary artery in 23% (39 of 167), left anterior descending in 2% (4 of 167), circumflex in 16% (26 of 167), and multiple coronaries in 1% (2 of 167). Anomalous right coronary arteries were diagnosed at an older median age than anomalous left main coronary arteries (55 vs 51 years, respectively; P = .026). Surgical repair of AAOCA occurred in 22% (36 of 167) of patients. Concomitant cardiac surgical procedures accompanied 36% (13 of 36) of them. No deaths occurred over a median follow-up of 2.5 years., Conclusions: Most patients in our single-center AAOCA registry were diagnosed in the presence of cardiac symptoms. Concomitant aortic valve disease and coronary atherosclerotic burden complicate both the evaluation and surgical approach to adult AAOCA repair., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Anomalous aortic origin of the left coronary artery (AAOCLA) with an extended transseptal course behind the right ventricular outflow tract (RVOT) is a rare variant that poses challenges not addressed by current surgical techniques. We utilized a novel transconal approach in 7 consecutive patients., Methods: A retrospective review was made of a prospectively collected database for consecutive patients undergoing transconal unroofing of transseptal AAOLCA. Surgical repair entails transection of the RVOT, unroofing the septal course of the AAOLCA, followed by RVOT extension with a rectangular strip of autologous pericardium. Preoperative characteristics, operative details, and postoperative course were abstracted., Results: All 7 patients identified were symptomatic. Median age was 48 years (range, 12 to 62). The AAOLCA with transseptal course was confirmed by computed tomography angiography. Three patients had provocative testing demonstrating anterolateral ischemia. Four patients underwent cardiac catheterization with intravascular ultrasound and indexed fractional flow reserve, confirming flow-limiting lesion. Importantly, 3 patients had negative provocative noninvasive testing for ischemia. Median postoperative hospital length of stay was 6 days (range, 4 to 12). No mortality or major complications occurred during a median follow-up of 0.75 years. Postoperative evaluation demonstrated anatomically patent unroofed AAOLCA with improved indexed fractional flow reserve compared with preoperative (0.59 ± 0.16 vs 0.90 ± 0.03, P = .05)., Conclusions: Complete unroofing of AAOLCA with transseptal course repaired with posterior extension of RVOT is an effective technique with excellent early outcome. Multimodality provocative testing is critical to evaluate these lesions as individual studies may be misleading. Intravascular ultrasound with indexed fractional flow reserve is clinically useful to confirm the hemodynamic significance of specific lesions., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Although outcomes for pediatric cardiomyopathy (CMP) patients have improved, an understanding of outcomes by CMP phenotype is essential. This study assessed changes in waitlist and post-transplant survival in nondilated cardiomyopathy (DCM) patients over 2 decades, explored ventricular assist device (VAD) utilization in this cohort, and identified risk factors for waitlist and posttransplant mortality in the current era., Methods: Pediatric patients with a diagnosis of CMP listed for heart transplantation during three eras: Era 1: March 5, 1999 to December 31, 2004; Era 2: January 1, 2005 to December 15, 2011; and Era 3 (current era): December 16, 2011 to February 28, 2018 were included. Multivariable Cox proportional hazards regression was performed to assess waitlist and posttransplant survival., Results: Compared with patients with DCM, those with hypertrophic and restrictive cardiomyopathy in the current era are less likely to be on VAD (23.4% vs 2.7% vs 4.5%); listed United Network for Organ Sharing Status 1A (75.6% vs 39.8% vs 34.8%), and more likely to have longer waitlist times (P < .01 for all). Only 3.3% hypertrophic and 2.4% restrictive cardiomyopathy patients had VAD implantation, although VAD use did not adversely impact waitlist survival in weighted non-DCM patients. Significant improvements have occurred in waitlist survival of hypertrophic and posttransplant survival of both types of non-DCM patients., Conclusions: Currently, waitlist and posttransplant survival is similar for all CMP phenotypes. VAD use is low in patients with non-DCM, although this did not increase waitlist mortality in adjusted analysis. Further studies in patients with non-DCM are needed to determine optimal timing and anatomic characteristics most likely to benefit from VAD implantation during the waitlist period., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Contemporary practice patterns and outcomes for aortic valve replacement (AVR) among young and middle-aged adults are unknown given guideline modifications for surgical AVR (SAVR) and increasing transcatheter AVR (TAVR) acceptance. This study describes SAVR and TAVR use and outcomes using The Society of Thoracic Surgeons (STS) National Databases., Methods: Adults 18 to 55 years of age in the Congenital Heart Surgery Database (CHSD) and the Adult Cardiac Surgery Database (ACSD) who underwent SAVR or TAVR from 2013 to 2018 were included. Perioperative characteristics and early outcomes were described by valve type. Multivariable regression identified determinants of death, length of hospital stay, and a composite end point of renal failure, persistent neurologic deficit, readmission, and reoperation., Results: The study analyzed 1580 unique CHSD and 44,173 ACSD operations, 16% of which were performed in patients with congenital heart disease. Valve use included the following: TAVR, 1%; mechanical, 42%; bioprosthetic, 55%; autograft, 0.6%; homograft, 1.2%; and Ozaki, 0.4%. Over time, TAVR volumes increased by 167%. The 30-day mortality was as follows: TAVR, 3.8%; mechanical, 3.2%; bioprosthetic, 3.7%; autograft, 0.6%; homograft, 9%; and Ozaki, 3.4%. Stroke rate was lower for isolated SAVR vs isolated TAVR (0.9% vs 2.4%; P = .002). In multivariable analyses, mortality risk was lower with mechanical valves, congenital morbidity risk was higher with TAVR, and length of stay was shorter with TAVR., Conclusions: TAVR is increasingly used for adults younger than 55 years of age. Given the uniformly excellent results with SAVR, including both mortality and morbidity-particularly regarding stroke, our data favor SAVR in this population, but a prospective trial is needed. Ongoing efforts to harmonize variables and outcomes definitions between the ACSD and CHSD are valuable., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Over 150 hospitals perform congenital heart surgery (CHS) in the United States. Many hospitals are close together, with a median patient travel distance of 38.5 miles. We began with a theoretical blank slate and used objective methodology guided by population density and volume thresholds to estimate the optimal number and locations of hospitals to provide CHS in the United States., Methods: Guided by published data, we estimated the number of CHS operations in the United States in to be 32,500 per year. We distributed patients geographically based on population density. Metropolitan Statistical Areas (population centers and surrounding areas with close economic/social ties) were used as potential hospital locations. Patients were assigned to the closest hospital location such that all hospitals had a CHS volume of ≥300 operations., Results: We estimated 57 hospitals could serve the contiguous United States. Median theoretical hospital volume after regionalization was 451 operations (interquartile range, 366-648). Median patient travel distance was 35.1 miles. Some patients (6396/31,895, 20%) traveled more than 100 miles., Conclusions: Our model suggests the United States could be served by approximately 100 fewer CHS hospitals than currently exist. With hospitals optimally placed, patient travel burden would decrease. This model serves as a platform to improve care delivery by regionalization of CHS., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Repair of anomalous pulmonary venous return (APVR) when veins are remote from the left atrium (LA) is challenging and may eventuate in a higher prevalence of pulmonary vein stenosis, superior vena cava stenosis, or intracardiac baffle obstruction. We describe our experience in 6 patients with a technique, using both anterior and posterior in situ pericardial roll repairs, which reduces these complications., Methods: Six patients underwent in situ pericardial roll repair of APVR at Cleveland Clinic between 2018 and 2019. Median age was 40 years (range, 0.25-65 years). Three patients had partial APVR of right upper and middle veins into superior vena cava high above the right pulmonary artery without atrial septal defect; 2 had scimitar syndrome, and the infant had heterotaxy with unbalanced atrioventricular canal and mixed obstructed total APVR. The anomalous pulmonary vein drained into the respective cava far from the LA, which was not ideal for traditional repair techniques. In situ pericardial roll directed anomalous pulmonary vein to the LA. Most patients had concomitant complex cardiac procedures., Results: There was no mortality. Median hospital stay was 23 days (range, 4-60 days) and median follow-up was 20 months (range, 1-36 months). The infant required percutaneous dilatation and stenting of LA anastomosis but since underwent ventricular switch. At last follow-up, pulmonary veins were unobstructed and adult patients were asymptomatic with excellent functional status., Conclusions: In situ autologous pericardial roll is a useful technique that abrogates the need for mobilization of distant anomalous pulmonary vein with direct anastomosis or complex intracardiac baffles. It is suitable for multiple anatomic configurations and can be used in infants and adults., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Hospital readmission after pediatric cardiac surgery is incompletely understood. This study analyzed data from The Society of Thoracic Surgeons Congenital Heart Surgery Database to determine prevalence, to describe patient characteristics, and to evaluate risk factors for readmission., Methods: Readmission was defined by the "readmission within 30 days after discharge" field. Routine variables were summarized. Regression analysis was used to identify factors associated with readmission., Results: The study cohort included 56,429 patient records from 100 centers. Overall, 6,208 (11%) patients were readmitted. The most common reasons for readmission were respiratory or airway complications (14.2%), septic or infectious complications (11.4%), and reasons not related to the preceding surgical procedure (20.2%). Primary reason for readmission varied across benchmark operation groups. In multivariable analysis, factors associated with increased odds of readmission included the presence of noncardiac abnormalities (odds ratio [OR], 1.24), chromosomal abnormalities or genetic syndromes (OR, 1.24), preoperative mechanical circulatory support (OR, 1.36), other preoperative factors (OR, 1.21), prior cardiac surgery (OR, 1.31), Hispanic ethnicity (OR, 1.13), higher STAT procedural complexity (Society of Thoracic Surgeons/European Association for Cardio-Thoracic Surgery) (STAT level 3 vs 1, OR, 1.22; STAT 4 vs 1, OR, 1.48; STAT 5 vs 1, OR, 2.62), prolonged postoperative length of stay (OR, 1.07 per day from 0 to 14 days; OR, 1.01 per week >14 days), any major complication (OR, 1.27), any other postoperative complications (OR, 2.00), and discharge on a weekday (OR, 1.07)., Conclusions: Readmission is common after congenital heart surgery, mostly for noncardiovascular reasons. Process improvement initiatives targeted at high-risk patients could minimize its impact., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Several countries have regionalized congenital heart surgery (CHS). Before considering regionalization in the US, the current landscape must be understood. This investigation characterized the network of US hospitals providing CHS, including hospital locations and patient travel patterns., Methods: Patients ≤18 years undergoing CHS were identified in 2012 State Inpatient Databases from 39 states. Cases were stratified by the RACHS-1 method (high-risk defined as RACHS-1 categoris 4 to 6). Hospital and patient locations were identified. Patients were mapped to hospitals where they underwent surgery., Results: A total of 153 hospitals across 36 states performed ≥1 RACHS-1 case (19,064 operations). Of these, 101 hospitals (66%) were located within 25 miles of another hospital. Median annual RACHS-1 case volume was 90 (range, 1 to 797), with 55 hospitals performing ≤50 cases. A total of 111 hospitals (73%) performed ≥1 high-risk case. Of these, 39 (35%) performed ≤10 high-risk cases/year. Overall mortality rate was 3.5% (n = 666), with risk-adjusted mortality being lowest at hospitals in the highest-volume quartile (≥150 cases/year). About 25% of patients (n = 4,012) traveled >100 miles, with most traveling to hospitals within the highest-volume quartile; 53% of patients (n = 8,376) bypassed the nearest CHS hospital. Mortality was not associated with travel distance., Conclusions: We identified more US hospitals performing CHS than has been previously described. Many are small-volume and are in close proximity to one another. Patients are already traveling long distances to hospitals within the highest-volume quartile. These data help define the current landscape of CHS and associated considerations regarding regionalization., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The comprehensive stage 2 procedure (CS2) follows initial hybrid stage 1 palliation for patients with single-ventricle physiology. The goal of this study was to describe CS2 outcomes and differences between survivors of CS2 (s-CS2) and nonsurvivors (nons-CS2)., Method: All patients in The Society of Thoracic Surgeons Congenital Heart Surgery Database who underwent CS2 as index operation from 2010 to 2016 were included. Preoperative, operative, and postoperative data were analyzed, stratified by operative mortality (in-hospital or within 30 days of the operation), with univariate comparisons using χ 2 , Fisher exact, or Wilcoxon rank sum tests., Results: Of 209 patients (49 centers) who met inclusion criteria, 141 patients had the diagnosis of hypoplastic left heart syndrome. Overall operative mortality was 12.4% (26 of 209). s-CS2 had a lower prevalence of preoperative extracorporeal membrane oxygenation use (0.0% vs 7.7%, p = 0.02) and less frequently underwent concomitant tricuspid valve procedures at the time of the operation (1.1% vs 11.5%, p = 0.01) than nons-CS2. Postoperatively, the prevalence of any of six The Society of Thoracic Surgeons Congenital Heart Surgery Database major complications was higher in nons-CS2 than in s-CS2 (53.9% vs 23.0%, p < 0.01), including a higher prevalence of renal failure (7.7% vs 0.6%, p = 0.04) and postoperative extracorporeal membrane oxygenation use (46.2% vs 2.7%, p < 0.01). There were no other preoperative, operative, or postoperative differences between the two groups., Conclusions: Operative mortality associated with the CS2 procedure is substantial, especially for patients receiving extracorporeal membrane oxygenation support after CS2. No obvious modifiable variables were noted between patients who died and those who survived., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The effect of the timing of stage-2-palliation (S2P) on survival through single ventricle palliation remains unknown. This study investigated the optimal timing of S2P that minimizes pre-S2P attrition and maximizes post-S2P survival., Methods: The Congenital Heart Surgeons' Society's critical left ventricular outflow tract obstruction cohort was used. Survival analysis was performed using multiphase parametric hazard analysis. Separate risk factors for death after the Norwood and after S2P were identified. Based on the multivariable models, infants were stratified as low, intermediate, or high risk. Cumulative 2-year, post-Norwood survival was predicted. Optimal timing was determined using conditional survival analysis and plotted as 2-year, post-Norwood survival versus age at S2P., Results: A Norwood operation was performed in 534 neonates from 21 institutions. The S2P was performed in 71%, at a median age of 5.1 months (IQR: 4.3 to 6.0), and 22% died after Norwood. By 5 years after S2P, 10% of infants had died. For low- and intermediate-risk infants, performing S2P after age 3 months was associated with 89% ± 3% and 82% ± 3% 2-year survival, respectively. Undergoing an interval cardiac reoperation or moderate-severe right ventricular dysfunction before S2P were high-risk features. Among high-risk infants, 2-year survival was 63% ± 5%, and even lower when S2P was performed before age 6 months., Conclusions: Performing S2P after age 3 months may optimize survival of low- and intermediate-risk infants. High-risk infants are unlikely to complete three-stage palliation, and early S2P may increase their risk of mortality. We infer that early referral for cardiac transplantation may increase their chance of survival., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)