OBJECTIVE:To assess associations between the presence of genetic diagnoses and survival and morbidity of patients with symptomatic tetralogy of Fallot (sTOF) requiring neonatal intervention. STUDY DESIGN:We performed an analysis of a multicenter, retrospective study of sTOF patients from 2005 to 2017 from the Congenital Cardiac Research Collaborative. The primary outcome was transplant-free survival, evaluated by Cox proportional hazards regression modeling, adjusted for center, repair strategy, anatomical diagnosis, prematurity, and invasive ventilation before intervention. Genetic diagnoses were retrospectively collected from hospital records. RESULTS:The study group included 572 neonates with sTOF, of whom 151 (26.4%) had an identifiable genetic diagnosis, including 22q11 deletion (n = 63, 41.7%), trisomy 21 (n = 28, 18.5%), and other genetic diagnoses (n = 60, 39.7%). At a median follow-up of 4.12 (1.53, 7.47) years, there was no significantly increased hazard ratio of death in patients with a genetic diagnosis (adjusted hazard ratio 1.71 [95% CI 0.96-3.07], P = .07). However, patients with a genetic diagnosis had longer median intensive care unit and total hospital stays ([13 vs 9 days, P < .001] and [32.5 vs 24 days, P < .001], respectively) and were more likely to be discharged with feeding tubes (OR 2.1 [95% CI 1.31-3.37], P = .002). CONCLUSIONS:Neonates with sTOF with a genetic diagnosis had no significant survival difference to those without but did have a higher risk for other hospital morbidities, including longer admissions and the need for feeding tubes. Genetic testing in this population can inform clinicians and families regarding these important considerations within this congenital heart disease population.

BACKGROUND:Current guidelines recommend surgical aortic valve replacement (SAVR) over transcatheter aortic valve replacement (TAVR) for patients ≤65 years of age. Recent state-specific data suggest that >50% of patients aged ≤65 years undergo TAVR. Given recent data of a potential survival benefit for mechanical SAVR in patients aged 60 years and younger, we sought to evaluate the national incidence of TAVR, bioprosthetic SAVR, and mechanical SAVR in young patients. METHODS:Using the Premier Healthcare Database, all patients aged 40 to 65 years undergoing isolated aortic valve replacement (2016-2024) were assessed. Premier is a nationally representative all-payer, all-age, inpatient and outpatient database accounting for 25% of the population of the United States. Diagnosis-related group and International Classification of Diseases, Tenth Revision procedure codes were used to define procedures and comorbidities and a validated frailty metric. RESULTS:A total of 18,694 patients receiving first-time isolated aortic valve replacement were analyzed (31.3% TAVR, 68.7% SAVR). Patients receiving TAVR were older and more often female and Black, with higher Kim frailty index (P < .0001). Young patients were more likely to receive TAVR at teaching hospitals and larger centers (>499 beds). TAVR increased from the beginning of the study period to a peak of 40.8% in 2020, followed by a decrease to 32.5% in 2024. Overall utilization of mechanical SAVR remained between 15% and 18% throughout. CONCLUSIONS:Nearly one-third of patients aged 40 to 65 years receive TAVR in contemporary practice. Real-time data highlight that evolving TAVR use outside of current guidelines is less than previously reported in regional data sets.

Temporizing balloon aortic valvuloplasty (BAV) may be utilized in the management of decompensated severe aortic stenosis. BAV is occasionally used as a bridge to eventual transcatheter aortic valve replacement (TAVR). We sought to evaluate the outcomes of urgent inpatient TAVR versus BAV followed by TAVR (BAVTAV). The United States Medicare database was used to evaluate all beneficiaries undergoing TAVR (n = 227,145) or BAV (n = 16,643) from 2018 to 2022. Patients were stratified into 3 cohorts: urgent inpatient TAVR without BAV, urgent BAV followed by inpatient TAVR (urgent BAVTAV), and urgent BAV followed by elective outpatient TAVR (elective BAVTAV). To adjust for selection bias, doubly robust risk adjustment was performed using inverse probability weighting and multilevel regression to assess peri-procedural and 5-year outcomes. A total of 23,762 patients underwent urgent TAVR without BAV, while 4,404 patients received BAVTAV (1,503 urgent and 2,901 elective). Inpatient mortality of urgent TAVR, urgent BAVTAV, and elective BAVTAV was 3.0%, 5.3%, and 1.5%, respectively, with a similar association for acute stroke (2.1% vs 2.7% vs 2.0%) and new pacemaker implantation (8.5% vs 8.5% vs 6.0%). After risk adjustment, elective BAVTAV was associated with lower index mortality (odds ratios 0.61, p = 0.011), stroke (odds ratios 0.55, p < 0.0001), and longitudinal mortality (hazard ratio 0.67, p < 0.001) compared with urgent TAVR. Urgent BAVTAV was associated with a higher index and longitudinal mortality. In conclusion, among Medicare beneficiaries with acutely decompensated severe aortic stenosis, temporizing BAV as a bridge to future outpatient elective TAVR appears to be a viable treatment strategy when felt to be medically possible.

OBJECTIVES:Recent data suggest that 50% of patients ≤65 years of age undergo transcatheter aortic valve implantation (TAVI). It has also been suggested that these patients are typically high risk with poor life expectancy. We sought to evaluate predicted risk, procedural and longitudinal outcomes, and cost in patients ≤65 years who received TAVI in the Premier Healthcare Database. METHODS:All patients aged 65 and younger receiving TAVI (2017-2024) were assessed. A predicted risk of index surgical aortic valve mortality model, incorporating the Fried frailty index, was fitted to simulate the Society of Thoracic Surgeons risk model (AUC = 0.850). TAVI outcomes were stratified by predicted risk of mortality. RESULTS:A cohort of 6921 patients ≤65 years received TAVI. The median age was 60 years, 81.3% were elective, and 9.1% bicuspid. The average predicted risk was 4.3%, with 66.2% of patients having a predicted risk <4%. A total of 50 patients (0.7%) died within 30 days of the index procedure. Early complications included 41 (0.6%) emergent sternotomies, 182 (2.6%) unplanned coronary interventions, 32 (0.5%) femoral artery repairs, 77 (1.0%) strokes, and 485 (4.1%) new permanent pacemakers. With a median follow-up of 3.3 years, 5-year survival was 88% in low- and intermediate-risk patients and 78% in the high-risk cohort (>8% predicted). CONCLUSIONS:Most contemporary patients ≤65 years receiving TAVI outside of existing guidelines are of low surgical risk. These findings, in combination with the cumulative longitudinal risks of TAVI, highlight the need for careful heart team discussions for most young patients requiring AVR.

Objective Shared decision-making of prosthesis selection for aortic valve replacement (AVR) weighs patient-specific valve durability with oral anticoagulation requirements. Given evolving strategies of lifetime management, we evaluated contemporary longitudinal outcomes of patients aged 65 years or greater undergoing bioprosthetic versus mechanical AVR. Methods Patients aged 65-85 who underwent isolated surgical AVR (2018-2022) were identified in the United States Centers for Medicare and Medicaid Services database and stratified by valve type as bioprosthetic (bAVR) or mechanical (mAVR). Doubly robust risk adjustment of variables including frailty was performed using inverse probability weighting of propensity scores, multivariable logistic regression, and time-to-event analysis with competing risks. The primary outcome was the composite of all-cause mortality, valve reintervention, stroke, and bleeding. Results The study cohort included a total of 69,423 patients (62,925 bAVR and 6498 mAVR). After comprehensive risk adjustment was performed, bAVR versus mAVR was associated with superior freedom from the primary composite outcome over the 5-year study period (hazard ratio [HR], 0.82; P < .001), as well as reduction in longitudinal mortality (HR, 0.78; P < .001), all-cause readmissions (HR, 0.89; P < .001), and readmissions for bleeding (HR, 0.47, P < .001) and heart failure (HR, 0.86; P < .001). No difference in aortic valve reintervention was observed between groups (HR, 0.93; P = .65). Similar trends favoring bAVR were observed in subanalyses of patients aged 65 to 69 years. For patients with preoperative end-stage renal disease, there was no difference in the longitudinal primary outcome between bAVR or mAVR. Conclusions In Medicare beneficiaries, bAVR was associated with superior risk-adjusted survival, fewer bleeding complications, and fewer readmissions with no difference in valve reintervention compared with mAVR.

Background Advances in tetralogy of Fallot care have improved outcomes; however, sociodemographic disparities among infants with symptomatic tetralogy of Fallot requiring neonatal intervention (sTOF) remain incompletely characterized. Objectives This study aimed to investigate sociodemographic and socioeconomic factors associated with management strategy and clinical outcomes in sTOF. Methods We conducted a retrospective cohort study of neonates with sTOF undergoing intervention between January 2005 and November 2017 at 9 centers in the Congenital Cardiac Research Collaborative. The primary outcome was management strategy: primary complete repair vs staged repair. The primary predictor was the Childhood Opportunity Index, a neighborhood-level health composite measure. Secondary predictors included distance to cardiac center, maternal race-ethnicity, language, and insurance. Group differences were assessed using chi-square/Fisher tests with Holm adjustment, and multivariable models determined associations between predictors and outcomes. Results Among 417 neonates, 180 (43%) underwent primary repair and 237 (57%) staged repair. Most were prenatally diagnosed (59%), non-Hispanic White (63%), and publicly insured (54%). In univariate analyses, Childhood Opportunity Index and maternal race-ethnicity were associated with management strategy; these associations attenuated after adjustment. Infants living >100 miles from a cardiac center had a higher hazard for mortality compared with those <50 miles (HR: 2.38; 95% CI: 1.07-5.30; P = 0.033). Maternal race-ethnicity and public insurance were associated with longer hospital stays and increased in-hospital complications. Conclusions Sociodemographic factors, including geography, race-ethnicity, insurance, and neighborhood opportunity, are associated with management and outcomes in neonates with sTOF. Distance to care, maternal race-ethnicity, and insurance have notable impacts on these outcomes and warrant further assessment to ensure equitable decision-making management strategies.

BACKGROUND:As percutaneous therapeutic options expand, the optimal management of severe aortic stenosis (AS) and concomitant coronary artery disease (CAD) is being questioned between coronary artery bypass grafting with surgical aortic valve replacement (CABG+SAVR) and percutaneous coronary intervention with transcatheter aortic valve replacement (PCI+TAVR). This study sought to compare perioperative and longitudinal risk-adjusted outcomes between patients undergoing CABG+SAVR and patients undergoing PCI+TAVR. METHODS:Using the Centers for Medicare & Medicaid Services inpatient claims database, the study evaluated all patient aged 65 years and older with AS and CAD who were undergoing CABG+SAVR or PCI+TAVR (from 2018 to 2022). Comorbidities and frailty were accounted for using validated metrics with doubly robust risk adjustment using inverse probability weighting, multilevel regression, and competing-risk time to event analyses. The primary end point was a 5-year composite of stroke, myocardial infarction (MI), valve reintervention, or death. RESULTS:A total of 37,822 patients formed the study cohort (PCI+TAVR, n = 17,413; CABG+SAVR, n = 20,409). Accounting for age, comorbidities, frailty, and number of vessels revascularized, PCI+TAVR was associated with lower procedural mortality (1.1% vs 3.6%; odds ratio [OR], 0.29; P <.001) but higher vascular complications (OR, 6.02; P <.001) and new permanent pacemaker (OR, 1.92; P <.001). However, the longitudinal 5-year primary end point favored CABG+SAVR (20.4% vs 14.2%; OR, 1.44, P <.001). Subgroup analyses demonstrated a benefit in the use of arterial conduit in CABG+;AVR in patients with single-vessel CAD. CONCLUSIONS:Among Medicare beneficiaries with severe AS and CAD, CABG+SAVR was associated with higher procedural mortality than PCI+TAVR but lower 5-year risk-adjusted stroke, MI, valve reintervention, and death.

To evaluate the relationship between center volume and inpatient mortality among patients transferred to another hospital for pediatric cardiac surgery using contemporary real-world data. The Kids’ Inpatient Database (KID) was queried for cardiopulmonary bypass (CPB) cases for the years 2016 and 2019. Hospitals were divided into three groups based on terciles of volume: low: ≤ 103 cases/year, mid: 104–194 cases/year, and high: > 194 cases/year. Multilevel regression models were created to evaluate the association between volume and inpatient mortality for transferred patients, both for the entire cohort and for high-complexity cases. (Risk Stratification for Congenital Heart Surgery (RACHS-2) categories 3,4, and 5). Of 25,749 patients undergoing cases on CPB, 3511 (13.6

Cardiac mortality in the population of patients with adult congenital heart disease (ACHD) is driven primarily by heart failure. The clinical trial data, high-quality evidence and consensus guidelines that inform medical management of the population with acquired heart disease is lacking for those with ACHD. These patients require special attention, and while the data can cautiously be extrapolated, in many instances, future population driven studies are necessary. This review summarizes the most up-to-date data and provides expert review of heart failure in the patient with ACHD focusing on epidemiology, staging and the available pharmacologic therapies.

OBJECTIVES:Patients with a single ventricle and systemic outflow obstruction undergo 1 of 3 stage 1 palliative operations: Norwood-Sano, Norwood-Modified Blalock-Taussig-Thomas shunt (mBTTs), or stage 1 hybrid palliation. We evaluated real-world outcomes for patients undergoing stage 1 palliation strategies in a national dataset. METHODS:The Kids' Inpatient Database (KID) was queried for patients undergoing Norwood and hybrid procedures for the years 2016, 2019, and 2019. Data were extracted using International Classification of Diseases (ICD-10) codes. Using survey weights, an estimated national cohort was derived. Multilevel, multivariable logistic regression models evaluated risk-adjusted mortality and post-operative extracorporeal membrane oxygenation (ECMO). Operations were plotted by hospital mortality for the entire sample. RESULTS:A total of 1918 patients underwent stage 1 palliation with Norwood-Sano (1046, 54.5%), Norwood-mBTT shunt (770, 40.1%), or hybrid procedure (102, 5.3%). Unadjusted inpatient mortality was 9.0% for Norwood-Sano, 11.5% for Norwood-mBTT shunt, and 18.7% for the hybrid procedure. After risk adjustment, there was no significant difference in risk-adjusted inpatient mortality for Norwood-mBTTs (OR 1.25, P = .248) or hybrid procedures (OR 2.01, P = .057) compared to Norwood-Sano. There was no difference in need for ECMO for Norwood-mBTT shunt (OR 0.90, P = .78), or hybrid procedure (OR 0.50, P = .37). Finally, compared to Norwood-Sano, the use of mBTTs or hybrid procedures was higher in high-mortality hospitals. CONCLUSIONS:Compared to historical data, outcomes for stage 1 palliative procedures are improving over historical norms. There was no difference in risk-adjusted inpatient mortality or ECMO comparing Norwood-Sano to Norwood-mBTTs. These data may aid the evolution of operative strategies.

BACKGROUND:Survival for hypoplastic left heart syndrome (HLHS) and variants has improved over the past 4 decades; however, survival remains low compared with other forms of congenital heart disease. There is a paucity of data concerning long-term outcomes. OBJECTIVES:This study aims to: 1) examine long-term survival and the impact of patient factors on survival for newborns with HLHS; and 2) examine functional and health outcomes, including quality-of-life (QOL) in adulthood. METHODS:The study cohort included patients with HLHS and variants undergoing the Norwood or hybrid procedure between January 1984 and December 2023. Data on patient characteristics and management were abstracted from medical records. Vital status was ascertained by direct subject and family contact, review of the medical record, and publicly available data. Functional outcomes and QOL in adults (≥18 years of age) were assessed by patient reports. The primary outcome was death or cardiac transplantation at last follow-up. RESULTS:In the study period, 2,012 neonates underwent staged reconstructive surgery for HLHS (Norwood, n = 1,921 and hybrid, n = 91). Transplant-free survival was 31.0% at 35 years. Transplant-free survival improved over time but is not different across recent eras. Most responders reported good to excellent general health. CONCLUSIONS:In this cohort of newborns undergoing staged reconstructive surgery for HLHS, fewer than one-third are alive without a transplant at 35 years of age. Survival has not improved in recent years. However, there is a group of survivors who report good to excellent outcomes and QOL, consistent with a "high-performing" Fontan phenotype.

Background Failure to rescue (FTR) is a validated quality metric for evaluating outcomes in pediatric cardiac surgery. Using a national administrative database, we sought to assess the relationship between annual hospital case volume and FTR. Methods Cardiopulmonary bypass (CPB) cases were extracted from the Kids’ Inpatient Database for 2016 and 2019. FTR was defined as inpatient mortality of patients with at least 1 postoperative complication. Hospitals were divided into high-, mid-, and low-volume hospitals on the basis of annual CPB case volume terciles. A mixed effects multivariable logistic model was created to evaluate the association of annual CPB case volume and FTR. By use of the mean adjusted FTR rate of the entire sample as the benchmark, hospitals were divided into underperformers and overperformers. Results A total of 10,528 patients had 1 or more complications during the same admission. There were 134 hospitals in the low-volume group, 64 in the mid-volume group, and 31 in the high-volume group. The risk-adjusted FTR rate was 4.04% for the entire cohort. Compared with low-volume hospitals at baseline, there was no statistically significant difference in FTR for mid-volume hospitals (odds ratio, 0.8; 95% CI, 0.61-1.1; P = .184) or high-volume hospitals (odds ratio, 0.8; 95% CI, 0.6-1.1; P = .201). There were underperforming and overperforming hospitals in all volume groups. Conclusions This real-world contemporary analysis highlights that hospital volume is not associated with FTR after pediatric cardiac surgery. Underperformers and overperformers exist in all volume groups. Volume may not be a reliable quality metric for comparing outcomes in pediatric cardiac surgery.

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.

BACKGROUND:In symptomatic neonates with tetralogy of Fallot (sTOF), the initial treatment strategy significantly affects early outcomes, but its long-term impact remains less well defined. OBJECTIVES:The aim of the study was to compare primary (PR) vs staged repair (SR) in sTOF with respect to reintervention (RI) rates and types, clinical and echocardiographic outcomes, and medication use. METHODS:Neonates with sTOF undergoing PR or SR and with >1 year of follow-up after complete repair were included. The primary outcome was cumulative RI incidence; secondary outcomes included mortality and late echocardiographic and clinical findings. Propensity scoring adjusted for baseline differences. Landmark analysis assessed RI risk at yearly intervals following complete repair. RESULTS:Of 441 neonates, 182 (41%) underwent PR, and 259 (59%) underwent SR. Groups differed in gestational age, intubation, and 22q11 status. Median follow-up postrepair was 5.26 (2.91, 8.21) years. RI burden was high in both groups, with a small, consistent but nonsignificant advantage to PR. The type of RI varied over time. PR was associated with greater pulmonary insufficiency and larger pulmonary arteries. RV pressure was ≤half systemic in 80%; 10% had ≥moderate tricuspid regurgitation, without between-group difference. Elevated RV pressure was associated with ≥moderate tricuspid regurgitation. CONCLUSIONS:Among sTOF survivors beyond the early perioperative period, late RI burden and residual hemodynamic lesions are common and largely unrelated to initial strategy. PR is associated with increased pulmonary insufficiency and pulmonary artery size.

Cardiac mortality in the population of patients with adult congenital heart disease (ACHD) is driven primarily by heart failure. Unifying consensus guidelines with high level of evidence is often lacking in this patient population. In practice, clinicians often cautiously extrapolate the evidence from acquired heart disease patients but one must not overlook that the ACHD patient has specific characteristics that make them unique. One of the key concepts in treating a patient with ACHD is to evaluate for and treat any reversible cause of heart failure prior to or concomitant with medical therapy. This review summarizes the most up-to-date data and provides expert review of heart failure in the patient with ACHD focusing on electrophysiologic, interventional, and advanced therapies on the road to heart or multi-organ transplantation.

The Fontan procedure, employed in the management of children with single ventricle congenital heart disease, continues to present long-term complications. Notably, certain complications associated with this procedure are linked to imbalances in the distribution of hepatic blood flow. One promising strategy to address this challenge involves employing a digital twin to simulate diverse Fontan configurations. The objective is to identify an optimal design that ensures balanced hepatic blood flow and minimizes power losses. However, successful implementation depends on accurate, patient-specific estimates of pulmonary vascular resistance (PVR) for each lung at the pre-Fontan (Glenn) stage. In clinical practice, only the total PVR is typically measured, via catheterization using the Fick principle, but individual lung resistances can be derived by combining pressure data from catheterization (Cath) with flow data from cardiac magnetic resonance imaging (CMR). Still, notable discrepancies exist: Fick-based total PVR often differs significantly from Cath-CMR-based PVR due to differences in flow quantification, and neither method can distinguish between proximal and distal resistances within the Glenn pathway. An alternative method for estimating PVR was previously developed using a computational fluid dynamics (CFD) optimization framework. This method demonstrated a favorable correlation with PVR estimates derived from Cath-CMR, although it was not directly compared to clinical PVR values derived using the Fick principle. In this study, we compare three methods for calculating PVR, namely Fick-based, Cath-CMR-based, and CFD-based, using patient data from three independent institutions. Our results show that Fick-based PVR values are, on average, significantly lower than those obtained via the Cath-CMR and CFD methods. The CFD-based total PVR estimates show good agreement with the total Cath-CMR-based PVR. However, the elevated left proximal resistance present in the CFD method leads to a significant underestimation of the left lung resistance by the Cath-CMR method. This underscores the significance of incorporating proximal resistance in PVR estimation and supports the potential utility of the CFD-based method for preoperative planning in single ventricle patients.

OBJECTIVE:Bivalirudin is a direct thrombin inhibitor used off-label for systemic anticoagulation in extracorporeal membrane oxygenation (ECMO). There are limited data available in pediatric patients, specifically regarding optimal dosing in this heterogeneous population of patients. This study aimed to characterize bivalirudin use in pediatric patients on ECMO at a single center. METHODS:A retrospective chart review was conducted for consecutive patients undergoing ECMO at a quaternary center between January 2021 and June 2023 who received bivalirudin as the primary anticoagulation agent. The primary outcome was the dose of bivalirudin required to achieve activated partial thromboplastin time (aPTT) in the goal range. Additionally, time in therapeutic range, time to initial aPTT goal, dose adjustments required in patients receiving renal replacement therapy, amount of blood and blood products received, incidence of major bleeding, and complete ECMO circuit changes were evaluated. RESULTS:Fourteen patients, including 6 neonates, 5 children, and 3 adolescents, were included in the study. Eleven patients were initially placed on venoarterial ECMO, and 3 were initially placed on venovenous ECMO. The median ECMO duration was 6 days. The median dose of bivalirudin required to achieve the initial goal aPTT level varied between neonates, children, and adolescents (0.1 mg/kg/hr, 0.2 mg/kg/hr, 0.05 mg/kg/hr, respectively). CONCLUSIONS:Multiple patient factors including age, indication for ECMO, renal function, and hepatic function must be taken into consideration when determining a starting dose of bivalirudin for these pediatric patients.