Outcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus.

OBJECTIVE: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. RESULTS: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI:1.07,5.27; p=0.034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). CONCLUSIONS: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.

Increased utilization of the hybrid procedure is not associated with improved early survival for newborns with hypoplastic left heart syndrome: a single-centre experience.

OBJECTIVES: The primary objectives were to examine utilization of the Hybrid versus the Norwood procedure for patients with hypoplastic left heart syndrome or variants and the impact on hospital mortality. The Hybrid procedure was 1st used at our institution in 2004. METHODS: Review of all subjects undergoing the Norwood or Hybrid procedure between 1 January 1984 and 31 December 2022. The study period was divided into 8 eras: era 1, 1984-1988; era 2, 1989-1993; era 3, 1994-1998; era 4, 1999-2003; era 5, 2004-2008; era 6, 2009-2014; era 7, 2015-2018 and era 8, 2019-2022. The primary outcome was in-hospital mortality. Mortality rates were computed using standard binomial proportions with 95% confidence intervals. Rates across eras were compared using an ordered logistic regression model with and adjusted using the Tukey-Kramer post-hoc procedure for multiple comparisons. In the risk-modelling phase, logistic regression models were specified and tested. RESULTS: The Norwood procedure was performed in 1899 subjects, and the Hybrid procedure in 82 subjects. Use of the Hybrid procedure increased in each subsequent era, reaching 30% of subjects in era 8. After adjustment for multiple risk factors, use of the Hybrid procedure was significantly and positively associated with hospital mortality. CONCLUSIONS: Despite the increasing use of the Hybrid procedure, overall mortality for the entire cohort has plateaued. After adjustment for risk factors, use of the Hybrid procedure was significantly and positively associated with mortality compared to the Norwood procedure.

Surgical versus transcatheter aortic valve replacement in low-risk Medicare beneficiaries.

OBJECTIVE: Recent approval of transcatheter aortic valve replacement (TAVR) in patients at low surgical risk has resulted in a rapid real-world expansion of TAVR in patients not otherwise examined in recent low-risk trials. We sought to evaluate the outcomes of surgical aortic valve replacement (SAVR) versus TAVR in low-risk Medicare beneficiaries. METHODS: Using the US Centers for Medicare and Medicaid Services claims database, we evaluated all beneficiaries undergoing isolated SAVR (n = 33,210) or TAVR (n = 77,885) (2018-2020). International Classification of Diseases 10th revision codes were used to define variables and frailty was defined by the validated Kim index. Doubly robust risk adjustment was performed with inverse probability weighting and multilevel regression models, as well as competing-risk time to event analysis. A low-risk cohort was identified to simulate recent low-risk trials. RESULTS: A total of 15,749 low-risk patients (8144 SAVR and 7605 TAVR) were identified. Comparison was performed with doubly robust risk adjustment accounting for all factors. TAVR was associated with lower perioperative stroke (odds ratio, 0.62; P < .001) and hospital mortality (odds ratio, 0.16; P < .001) compared with SAVR. However, risk-adjusted longitudinal analysis demonstrated TAVR was associated with higher late risk of stroke (hazard ratio, 1.65; P < .001), readmission for valve reintervention (hazard ratio, 1.88; P < .001), and all-cause mortality (hazard ratio, 1.54; P < .001) compared with SAVR. CONCLUSIONS: Among low-risk Medicare beneficiaries younger than age 75 years undergoing isolated AVR, SAVR was associated with higher index morbidity and mortality but improved 3-year risk-adjusted stroke, valve reintervention, and survival compared with TAVR.

Atrial Fibrillation Management During Surgical vs Transcatheter Aortic Valve Replacement.

BACKGROUND: Societal guidelines support atrial fibrillation (AF) treatment during surgical aortic valve replacement (SAVR). Recently, many patients with AF at low to intermediate risk are managed by transcatheter aortic valve replacement (TAVR). Therefore, we evaluated longitudinal outcomes in these populations. METHODS: The United States Centers for Medicare and Medicaid Services inpatient claims database was evaluated for all beneficiaries with AF undergoing TAVR or SAVR with/without AF treatment (2018-2020). Treatment of AF included concomitant left atrial appendage obliteration, with/without surgical ablation, or endovascular appendage occlusion and/or catheter ablation at any time. Diagnosis-related group and International Classification of Diseases, 10th Revision, codes defined procedures with doubly robust risk adjustment across each group. RESULTS: A total of 24,902 patients were evaluated (17,453 TAVR; 7,449 SAVR). Of patients undergoing SAVR, 3176 (42.6%) underwent AF treatment (SAVR+AF). Only 656 TAVR patients (4.5%) received AF treatment. Comparing well-balanced SAVR+AF vs SAVR vs TAVR, there were no differences in the in-hospital incidence of renal failure, bleeding, or stroke, but increased pacemaker requirement (odds ratio [OR], 3.45; P < .0001) and vascular injury (OR, 9.09; P < .0001) were noted in TAVR and higher hospital mortality (OR, 4.02; P < .0001) in SAVR+AF. SAVR+AF was associated with lower readmission for stroke compared with SAVR alone (hazard ratio [HR], 0.87; P = .029) and TAVR (HR, 0.68; P < .0001) and with improved survival vs TAVR (HR, 0.79; P = .019). CONCLUSIONS: In Medicare beneficiaries with AF requiring aortic valve replacement, SAVR+AF was associated with improved longitudinal survival and freedom from stroke compared with TAVR. SAVR+AF treatment should be considered first-line therapy for patients with AF requiring aortic valve replacement.

Life-threatening pulmonary haemorrhage treated with coil embolisation followed by lobectomy in a patient with KCNT1 mutation.

KCNT1 mutations are associated with childhood epilepsy, developmental delay, and vascular malformations. We report a child with a likely pathogenic KCNT1 mutation (c.1885A>C, p.Lys629Glu) with recurrent pulmonary haemorrhage due to aortopulmonary collaterals successfully managed with coil embolisation followed by right upper lobectomy.

Volume Alone Does Not Predict Quality Outcomes in Hospitals Performing Pediatric Cardiac Surgery.

BACKGROUND: Lower institutional volume has been associated with inferior pediatric cardiac surgery outcomes. This study explored the variation in mortality rates among low-, mid-, and high-volume hospitals performing pediatric cardiac surgery in the United States. METHODS: The Kids' Inpatient Database was explored for the years 2016 and 2019. Hospitals performing only off-bypass coarctation and ventricular septal defect repair were omitted. The hospitals were divided into 3 groups by their annual case volume. Multivariable logistic regression models were fit to obtain risk-adjusted in-hospital mortality rates. RESULTS: A total of 25,749 operations performed by 235 hospitals were included in the study. The risk-adjusted mortality rate for the entire sample was 1.9%. There were 140 hospitals in the low-volume group, 64 hospitals in the mid-volume group, and 31 in the high-volume group. All groups had low-mortality (mortality <1.9%) and high-mortality (mortality >1.9%) hospitals. Among low-volume hospitals, 53% were low-mortality (n = 74) and 47% were high-mortality (n = 66) hospitals. Among mid-volume hospitals, 58% were low-mortality (n = 37) and 42% were high-mortality (n = 27) hospitals. Among high-volume hospitals, 68% were low-mortality (n = 21) and 32% were high-mortality (n = 10) hospitals. There was no statistically significant difference in risk-adjusted in-hospital mortality when comparing low-, mid-, and high-volume centers for 7 Society of Thoracic Surgeons benchmark procedures. CONCLUSIONS: This national, real-world, risk-adjusted volume outcome analysis highlights that volume alone may not be the sole arbiter to predict quality of pediatric cardiac surgery outcomes. Using case volume alone as a surrogate for quality may unfairly asperse high-performing, low-volume programs.

En Bloc Resection of a Giant Ganglioneuroma of the Chest Through Clamshell Thoracotomy.

Ganglioneuroma is a benign tumor requiring subtotal resection as a primary mode of treatment. There are several surgical approaches. A giant ganglioneuroma of the chest cavity may be approached via a clamshell thoracotomy. This manuscript presents a case of giant ganglioneuroma resected en bloc via clamshell thoracotomy in a seven-year-old child.

Concomitant Treatment of Atrial Fibrillation in Isolated Coronary Artery Bypass Grafting.

BACKGROUND: Societal guidelines support concomitant management of atrial fibrillation (AF) in patients undergoing cardiac surgery. To assess real-world adoption and outcomes, this study evaluated Medicare beneficiaries with AF who underwent isolated coronary artery bypass grafting (CABG) with surgical ablation (SA) or left atrial appendage obliteration (LAAO) or both procedures in combination (SA + LAAO). METHODS: The US Centers for Medicare & Medicaid Services inpatient claims database identified all patients with AF who underwent isolated CABG from 2018 to 2020. Diagnosis-related group and International Classification of Diseases-10th revision procedure codes defined covariates for doubly robust risk adjustment. RESULTS: A total of 19,524 patients with preoperative AF who underwent isolated CABG were stratified by SA + LAAO (3475 patients; 17.8%), LAAO only (4541 patients; 23.3%), or no AF treatment (11,508 patients; 58.9%). After doubly robust risk adjustment, longitudinal analysis highlighted that concomitant AF treatment with SA + LAAO (hazard ratio [HR], 0.74; P = .049) or LAAO alone (HR, 0.75; P = . 031) was associated with a significant reduction in readmission for stroke at 3 years compared with no AF treatment. Furthermore, SA + LAAO (HR, 0.86; P = .016) but not LAAO alone (HR, 0.97; P = .573) was associated with improved survival compared with no AF treatment. Finally, SA + LAAO was associated with a superior composite outcome of freedom from stroke or death at 3 years compared with LAAO alone (HR, 0.86;, P = .033) or no AF treatment (HR, 0.81; P = .001). CONCLUSIONS: In Medicare beneficiaries with AF who underwent isolated CABG, concomitant AF treatment was associated with reduced 3-year readmission for stroke. SA + LAAO was associated with superior reduction in stroke or death at 3 years compared with LAAO alone or no AF treatment.

Intra-Hisian Wenckebach after surgical closure of conoventricular ventricular septal defect.

We report a 5-year-old girl with transient complete atrioventricular (AV) block following surgical closure of a symptomatic conoventricular ventricular septal defect (VSD) which recovered on post-operative day 9. She later presented with exertional dizziness and fatigue. While congenital cardiac defect repairs are occasionally complicated by complete heart block, this patient was found to have intra-Hisian Wenckebach which is rare in the pediatric population and can be very difficult to discern from surface electrocardiograms and by Holter monitoring. Mechanisms of post-surgical AV block, including intra-Hisian Wenckebach, are not well characterized in the pediatric population.

Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot.

BACKGROUND: Neonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy. OBJECTIVES: The objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population. METHODS: Neonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <-2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth. RESULTS: We included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were -2.19 (25th-75th percentile: -2.55 to -1.94) and -2.23 (25th-75th percentile: -2.64 to -1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001). CONCLUSIONS: Despite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF.

Association of Interstage Monitoring Era and Likelihood of Hemodynamic Compromise at Intervention for Recoarctation Following the Norwood Operation.

Background Intensive monitoring has been associated with a lower death rate between the Norwood operation and superior cavopulmonary connection, possibly due to early identification and effective treatment of residual anatomic lesions like recoarctation before lasting harm occurs. Methods and Results Neonates undergoing a Norwood operation and receiving interstage care at a single center between January 1, 2005, and September 18, 2020, were studied. In those with recoarctation, we evaluated association of era ([1] preinterstage monitoring, [2] a transitional phase, [3] current era) and likelihood of hemodynamic compromise (progression to moderate or greater ventricular dysfunction/atrioventricular valve regurgitation, initiation/escalation of vasoactive/respiratory support, cardiac arrest preceding catheterization, or interstage death with recoarctation on autopsy). We also analyzed whether era was associated with technical success of transcatheter recoarctation interventions, major adverse events, and transplant-free survival. A total of 483 subjects were studied, with 22% (n=106) treated for recoarctation during the interstage period. Number of catheterizations per Norwood increased (P=0.005) over the interstage eras, with no significant change in the proportion of subjects with recoarctation (P=0.36). In parallel, there was a lower likelihood of hemodynamic compromise in subjects with recoarctation that was not statistically significant (P=0.06), with a significant difference in the proportion with ventricular dysfunction at intervention (P=0.002). Rates of technical success, procedural major adverse events, and transplant-free survival did not differ (P>0.05). Conclusions Periods with interstage monitoring were associated with increased referral for catheterization but also reduced likelihood of ventricular dysfunction (and a suggestion of lower likelihood of hemodynamic compromise) in subjects with recoarctation. Further study is needed to guide optimal interstage care of this vulnerable population.

Impact of Ventricular Dysfunction and Atrioventricular Valve Regurgitation on Pre-Fontan Attrition.

BACKGROUND: There is a significant incidence of pre-Fontan attrition-defined as failure to undergo Fontan completion-after superior cavopulmonary connection. This study investigated the impact of at least moderate ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) on pre-Fontan attrition. METHODS: This single-center retrospective cohort study included all infants who underwent Norwood palliation from 2008 to 2020 and subsequently underwent superior cavopulmonary connection. Pre-Fontan attrition was defined as death, listing for heart transplantation before Fontan completion, or unsuitability for Fontan completion. The study's secondary outcome was transplant-free survival. RESULTS: Pre-Fontan attrition occurred in 34 of 267 patients (12.7%). Isolated VD was not associated with attrition. However, patients with isolated AVVR had 5 times the odds of attrition (odds ratio, 5.4; 95% CI 1.8-16.2), and patients with both VD and AVVR had 20 times the odds of attrition (odds ratio, 20.1; 95% CI 7.7-52.8) compared with patients without VD or AVVR. Only patients with both VD and AVVR had significantly worse transplant-free survival compared with patients without VD or AVVR (hazard ratio, 7.7; 95% CI 2.8-21.6). CONCLUSIONS: The additive effect of VD and AVVR is a powerful contributor to pre-Fontan attrition. Future research investigating therapies that can mitigate the degree of AVVR may help improve Fontan completion rates and long-term outcomes.

Use of aortic wall patches as leaflet replacement material during aortic valve repair.

Objectives: Aortic valve repair can be limited by inadequate leaflet tissue for proper coaptation. Various kinds of pericardium have been used for cusp augmentation, but most have failed because of tissue degeneration. A more durable leaflet substitute is needed. Methods: In this report, 8 consecutive cases are presented in which autologous ascending aortic tissue was used to augment inadequate native cusps during aortic valve repair. Biologically, aortic wall is a living autologous tissue that could have exceptional durability as a leaflet substitute. Techniques for insertion are described in detail, along with procedural videos. Results: Early surgical outcomes were excellent, with no operative mortalities or complications, and all valves were competent with low valve gradients. Patient follow-up and echocardiograms to a maximum of 8 months' postrepair remain excellent. Conclusions: Because of superior biologic characteristics, aortic wall has the potential to provide a better leaflet substitute during aortic valve repair and to expand patient categories amenable to autologous reconstruction. More experience and follow-up should be generated.

Contemporary Coronary Artery Bypass Grafting vs Multivessel Percutaneous Coronary Intervention.

BACKGROUND: Interpretation of recent alterations to the guideline priority of coronary artery bypass grafting (CABG) and percutaneous coronary intervention (PCI) for multivessel disease contests historical data and practice. To reevaluate contemporary outcomes, a large contemporary analysis was conducted comparing CABG with multivessel PCI among Medicare beneficiaries. METHODS: The United States Centers for Medicare and Medicaid Services database was evaluated all beneficiaries with acute coronary syndrome undergoing isolated CABG or multivessel PCI (2018-2020). Risk adjustment was performed using multilevel regression analysis, Cox proportional hazards time to event models, and inverse probability of treatment weighting propensity scores. RESULTS: A total of 104,127 beneficiaries were identified undergoing CABG (n = 51,389) or multivessel PCI (n = 52,738). Before risk adjustment, compared with PCI, CABG patients were associated with younger age (72.9 vs 75.2 years, P < .001), higher Elixhauser Comorbidity Index (5.0 vs 4.2, P < .001), more diabetes (48.5% vs 42.2%, P < .001), higher cost ($54,154 vs $33,484, P < .001), and longer length of stay (11.9 vs 5.8 days, P < .001). After inverse probability of treatment weighting propensity score adjustment, compared with PCI, CABG was associated with lower hospital mortality (odds ratio, 0.74; P < .001), fewer hospital readmissions at 3 years (odds ratio, 0.85; P < .001), fewer coronary reinterventions (hazard ratio, 0.37; P < .001), and improved 3-year survival (hazard ratio, 0.51; P < .001). CONCLUSIONS: Contemporary real-world data from Medicare patients with multivessel disease reveal that CABG outcomes were superior to PCI, providing important longitudinal data to guide patient care and policy development.

Surgical ablation of atrial fibrillation is associated with improved survival compared with appendage obliteration alone: An analysis of 100,000 Medicare beneficiaries.

OBJECTIVE: Societal guidelines support the concomitant surgical ablation of atrial fibrillation in patients undergoing cardiac surgery. Recent evidence has highlighted the stroke reduction of left atrial appendage obliteration with or without surgical ablation in similar populations. To inform clinical decision-making, we evaluated real-world outcomes of patients with atrial fibrillation undergoing cardiac surgery by comparing no atrial fibrillation management with left atrial appendage obliteration alone versus surgical ablation + left atrial appendage obliteration. METHODS: By using the US Centers for Medicare and Medicaid Services inpatient claims database, we evaluated all beneficiaries aged 65 years and older with a diagnosis of atrial fibrillation undergoing coronary artery bypass grafting or mitral/aortic/tricuspid valve repair or replacement between January 2018 and December 2020. Diagnosis-related group and International Classification of Diseases, 10th Revision procedure codes were used to define variables. Risk adjustment was performed with regression analysis using inverse probability weighting of propensity scores and Cox proportional hazards models. Subgroup analyses stratified patients by primary operation and paroxysmal or persistent atrial fibrillation. RESULTS: A total of 103,382 patients with preoperative atrial fibrillation were stratified by surgical ablation + left atrial appendage obliteration (10,437; 10.1%), left atrial appendage obliteration alone (12,901; 12.5%), or no atrial fibrillation management (80,044; 77.4%). Patients with persistent atrial fibrillation (21,076; 20.4%) received the highest proportion of surgical ablation + left atrial appendage obliteration (4661 19.4%) and left atrial appendage obliteration alone (3%724%; 15.4%) versus no atrial fibrillation management (15,688; 65.2%). Likewise, patients undergoing open atrial operations (mitral/tricuspid; 17,204; 16.6%) had higher proportions of atrial fibrillation treatment (surgical ablation + left atrial appendage obliteration 5267 30.6%; left atrial appendage obliteration alone 4259 24.8%; no atrial fibrillation management 7678 44.6%). After robust risk adjustment, surgical ablation + left atrial appendage obliteration was independently associated with reduced 3-year mortality compared with no atrial fibrillation treatment (hazard ratio, 0.68, P < .001) and left atrial appendage obliteration alone (hazard ratio, 0.90, P < .001). Compared with no atrial fibrillation treatment, readmissions for embolic stroke were lower with both surgical ablation + left atrial appendage obliteration (hazard ratio, 0.77, P = .009) and left atrial appendage obliteration alone (hazard ratio, 0.73, P < .001). Reduction in 3-year composite mortality or stroke after surgical ablation + left atrial appendage obliteration was superior to left atrial appendage alone (hazard ratio, 0.90, P = .035). CONCLUSIONS: In Medicare beneficiaries with atrial fibrillation undergoing cardiac surgery, the surgical management of atrial fibrillation was associated with lower 3-year mortality and readmission for stroke, with surgical ablation + left atrial appendage obliteration being associated with higher survival compared with left atrial appendage obliteration alone.

Impaired Maternal-Fetal Environment and Risk for Preoperative Focal White Matter Injury in Neonates With Complex Congenital Heart Disease.

Background Infants with congenital heart disease (CHD) are at risk for white matter injury (WMI) before neonatal heart surgery. Better knowledge of the causes of preoperative WMI may provide insights into interventions that improve neurodevelopmental outcomes in these patients. Methods and Results A prospective single-center study of preoperative WMI in neonates with CHD recorded data on primary cardiac diagnosis, maternal-fetal environment (MFE), delivery type, subject anthropometrics, and preoperative care. Total maturation score and WMI were assessed, and stepwise logistic regression modeling selected risk factors for WMI. Among subjects with severe CHD (n=183) who received a preoperative brain magnetic resonance imaging, WMI occurred in 40 (21.9%) patients. WMI prevalence (21.4%-22.1%) and mean volumes (119.7-160.4 mm3) were similar across CHD diagnoses. Stepwise logistic regression selected impaired MFE (odds ratio [OR], 2.85 [95% CI, 1.29-6.30]), male sex (OR, 2.27 [95% CI, 1.03-5.36]), and older age at surgery/magnetic resonance imaging (OR, 1.20 per day [95% CI, 1.03-1.41]) as risk factors for preoperative WMI and higher total maturation score values (OR, 0.65 per unit increase [95% CI, 0.43-0.95]) as protective. A quarter (24.6%; n=45) of subjects had ≥1 components of impaired MFE (gestational diabetes [n=12; 6.6%], gestational hypertension [n=11; 6.0%], preeclampsia [n=2; 1.1%], tobacco use [n=9; 4.9%], hypothyroidism [n=6; 3.3%], and other [n=16; 8.7%]). In a subset of 138 subjects, an exploratory analysis of additional MFE-related factors disclosed other potential risk factors for WMI. Conclusions This study is the first to identify impaired MFE as an important risk factor for preoperative WMI. Vulnerability to preoperative WMI was shared across CHD diagnoses.

Comparison of treatment strategies for neonates with tetralogy of Fallot and pulmonary atresia.

OBJECTIVE: Neonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) but no major aorta-pulmonary collaterals are dependent on the arterial duct for pulmonary blood flow and require early intervention, either by primary (PR) or staged repair (SR) with initial palliation (IP) followed by complete repair (CR). The optimal approach has not been established. METHODS: Neonates with TOF/PA who underwent PR or SR were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Outcomes were compared between PR and SR (IP + CR) strategies. Propensity scoring was used to adjust for baseline differences. The primary outcome was mortality. Secondary outcomes included complications, length of stay, cardiopulmonary bypass and anesthesia times, reintervention (RI), and pulmonary artery (PA) growth. RESULTS: Of 282 neonates, 106 underwent PR and 176 underwent SR (IP: 144 surgical, 32 transcatheter). Patients who underwent SR were more likely to have DiGeorge syndrome and greater rates of mechanical ventilation before the initial intervention. Mortality was not significantly different. Duration of mechanical ventilation, inotrope use, and complication rates were similar. Cumulative length of stay, cardiopulmonary bypass, and anesthesia times favored PR (P ≤ .001). Early RI was more common in patients who underwent SR (rate ratio, 1.42; P = .003) but was similar after CR (P = .837). Conduit size at the time of CR was larger with SR. Right PA growth was greater with PR. CONCLUSIONS: In neonates with TOF/PA, SR is more common in greater-risk patients. Accounting for this, SR and PR strategies have similar mortality. Perioperative morbidities, RI, and right PA growth generally favor PR, whereas SR allows for larger initial conduit implantation.

Variation in Case-Mix Across Hospitals: Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

BACKGROUND: The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database was queried to document variation of patient characteristics, procedure types, and programmatic case-mix. METHODS: All index cardiac operations in patients less than 18 years of age in the STS Congenital Heart Surgery Database (July 2016 to June 2020) were eligible for inclusion except patients weighing ≤2.5 kg undergoing isolated patent ductus arteriosus closure. At the hospital level, we describe variations in patient and procedural characteristics known from previous analyses to be associated with outcomes. We also report variations across hospitals of programmatic case-mix. RESULTS: Data were analyzed from 117 sites (90 322 total operations, 87 296 total index cardiac operations eligible for STAT [STS-European Association for Cardio-Thoracic Surgery] 2020 Mortality Score). The median annual total index cardiac operations eligible for STAT 2020 Mortality Score per hospital was 157 (interquartile range [IQR], 94-276). Wide variability was documented in total annual index cardiac operations eligible for STAT 2020 Mortality Score per hospital (ratio 90th/10th percentile = 9.01), operations in neonates weighing <2.5 kg (ratio 90th/10th percentile = 4.09), operations in patients with noncardiac anatomic abnormalities (ratio 90th/10th percentile = 3.46), and operations in patients with preoperative mechanical ventilation (ratio 90th/10th percentile = 3.97). At the hospital level, the median percentage of all index cardiac operations in STAT 2020 Mortality Category 5 was 3.7% (IQR, 1.7%-4.9%), the median percentage of all index cardiac operations in STAT 2020 Mortality Category 4 or 5 was 24.4% (IQR, 19.0%-28.4%), the median hospital-specific mean STAT Mortality Category was 2.39 (IQR, 2.20-2.47), and the median hospital-specific mean STAT Mortality Score was 0.86 (IQR, 0.73-0.91). CONCLUSIONS: Substantial variation of patient characteristics, procedure types, and case-mix exists across pediatric and congenital cardiac surgical programs. Knowledge about programmatic case-mix augments data about indirectly standardized programmatic observed-to-expected (O/E) mortality. Indirectly standardized O/E ratios do not provide a complete description of a given pediatric and congenital cardiac surgical program. The indirectly standardized programmatic O/E ratios associated with a given program apply only to its specific case-mix of patients and may represent a quite different case-mix than that of another program.