AIM The "2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease" provides recommendations to guide clinicians on the evaluation and treatment of adult patients with congenital heart disease. It incorporates new evidence to replace the "2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease." METHODS A comprehensive literature search was conducted with a focus on literature published from 2017 to 2024; in some instances, older literature was also collected and reviewed. Clinical studies, systematic reviews and meta-analyses, and other evidence conducted on human participants and published in English were identified from MEDLINE (via PubMed), EMBASE, the Cochrane Library, Agency for Healthcare Research and Quality, and CINAHL for selected searches. STRUCTURE Recommendations from the "2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease" have been updated with new evidence to guide clinicians.

BACKGROUND:Atrioventricular valve regurgitation (AVVR) predicts long-term post-Fontan adverse outcomes. Literature on the measurement and prognostic value of quantitative AVVR assessment using cardiovascular magnetic resonance imaging (CMR) is limited. This study compared echocardiographic AVVR measurements with measurements by CMR, and investigated their association with clinical outcomes using the multi-center Fontan Outcome Registry using Clinical Examination (FORCE). METHODS:Patients with an examiner-reported AVVR severity category from CMR (AVVRReport-Cat) and an AVVR from matched echocardiography (AVVREcho) were included. Three quantitative CMR AVVR regurgitant fractions were used as follows: (1) stroke volume and antegrade aortic flow (AVVRStandard), (2) atrioventricular valve flow and antegrade aortic flow (AVVRAVV-AO), and (3) examiner-reported regurgitation fraction (AVVRReport-RF). Moderate AVVR was defined as regurgitant fraction of 20%-40% and severe >40%. The concordance across methods was calculated using Kappa statistics, and associations with outcomes of mortality or listing for/receiving heart transplantation were analyzed using Cox proportional hazard models. C-statistic was used to compare model performance. RESULTS:Exactly 2309 Fontan patients were included, with a median age at CMR of 15.7 years and (971/2309) 42.1% female. Categorical AVVR severity reported by CMR was significantly associated with adverse outcomes (adjusted HR 2.10, 95% CI [1.38, 3.19] for moderate and 4.61 [2.80, 7.58] for severe AVVR), comparable to AVVR reported by echocardiography. AVVRAVV-AO showed stronger concordance with AVVRReport-Cat (Kappa=0.58) and higher hazards of the adverse outcome than AVVRStandard. The subset of patients with examiner-reported regurgitation fraction (AVVRReport-RF) showed the highest hazard associated with AVVR and the highest C-statistic among all methods including echocardiography. CONCLUSIONS:This is the first study evaluating different CMR measurements of AVVR in a large cohort of Fontan patients. Compared with AVVRStandard, AVVRAVV-AO had higher concordance with the reported categorical AVVR using CMR. Moderate and severe AVVR determined by both CMR and echocardiography were associated with comparably increased hazards of adverse clinical outcomes.

Atrioventricular valve regurgitation (AVVR) remains a significant complication post-Fontan. This study evaluates the accuracy of 4D flow AVVR quantification in post-Fontan patients by comparing 4D flow to conventional echocardiographic (AVVREcho) and CMR assessments. Patients in the multi-institutional FORCE registry with high-quality 4D flow CMR were included. Regurgitant fractions (RFs) were calculated using two 4D-flow based methods: AVVRAVV−Ao (4D), calculated from atrioventricular valve and aortic flows, and AVVRJet, which directly measures AVVR jet volume. 4D flow RFs and severity classifications were compared with AVVREcho and CMR-reported AVVR severity (AVVRReport−Cat), and examiner-reported RFs (AVVRReport−RF). Statistical tests included Pearson correlation, Cohen’s kappa, and Bland-Altman analysis. The study included 218 post-Fontan patients (median age: 16.2 years, IQR: 12.8–22.5) and 36.2

OBJECTIVE:The hybrid stage 1 palliation (HS1P) procedure has been proposed as an alternative to the Norwood operation. We utilized a multicenter database to compare both strategies. METHODS:The Fontan Outcomes Registry using Clinical Examinations was queried for patients who underwent HS1P or Norwood. Propensity score matching was performed. Composite outcome (death, transplant listing, protein losing enteropathy, plastic bronchitis, atrial/ventricular tachyarrhythmia, or pulmonary artery reintervention) and cardiac magnetic resonance variables were compared. Secondary analyses compared between HS1P and shunt type (Sano vs Blalock-Thomas-Taussig). RESULTS:Two hundred twenty-eight patients were analyzed (76 HS1P, 152 Norwood) after exclusion and matching. Median follow-up after Fontan was 14 years (95% CI, 13.0-15.1 years) using the reverse Kaplan-Meier method. The freedom from composite outcome was 82.3%, 74.5%, and 61.2% for HS1P patients compared with 81.4%, 69.5%, and 54.7% for Norwood patients at 5-, 10-, and 15-year follow-ups, respectively (hazard ratio, 1.02; 95% CI, 0.62-1.70; P = .9305). Individual components of the composite outcome were also similar between HS1P and Norwood in the matched cohort, including pulmonary artery reintervention. Cardiac magnetic resonance and echocardiographic parameters did not differ between groups. In a 3-group analysis of the unmatched cohort (n = 954) using Blalock-Thomas-Taussig conduit as the reference, HS1P was not associated with differences in adjusted hazard for the composite outcome or its individual components compared with Blalock-Thomas-Taussig. CONCLUSIONS:HS1P in those who survived to Fontan completion had comparable Fontan era outcomes and cardiac magnetic resonance findings to Norwood in a propensity matched multicenter cohort. Further longitudinal work is essential to refine patient selection and to optimize long-term outcomes.

AIM:The "2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease" provides recommendations to guide clinicians on the evaluation and treatment of adult patients with congenital heart disease. It incorporates new evidence to replace the "2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease." METHODS:A comprehensive literature search was conducted with a focus on literature published from 2017 to 2024; in some instances, older literature was also collected and reviewed. Clinical studies, systematic reviews and meta-analyses, and other evidence conducted on human participants and published in English were identified from MEDLINE (via PubMed), EMBASE, the Cochrane Library, Agency for Healthcare Research and Quality, and CINAHL for selected searches. STRUCTURE:Recommendations from the "2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease" have been updated with new evidence to guide clinicians.

Abstract Atrioventricular valve regurgitation (AVVR) remains a significant complication post-Fontan. This study evaluates the accuracy of 4D flow AVVR quantification in post-Fontan patients by comparing 4D flow to conventional echocardiographic (AVVR Echo ) and CMR assessments. Patients in the multi-institutional FORCE registry with high-quality 4D flow CMR were included. Regurgitant fractions (RFs) were calculated using two 4D-flow based methods: AVVR AVV−Ao (4D) , calculated from atrioventricular valve and aortic flows, and AVVR Jet , which directly measures AVVR jet volume. 4D flow RFs and severity classifications were compared with AVVR Echo and CMR-reported AVVR severity (AVVR Report−Cat ), and examiner-reported RFs (AVVR Report−RF ). Statistical tests included Pearson correlation, Cohen’s kappa, and Bland-Altman analysis. The study included 218 post-Fontan patients (median age: 16.2 years, IQR: 12.8–22.5) and 36.2% were female. Both 4D flow methods displayed excellent interobserver reproducibility (interclass correlation > 0.95) and RFs approximately 7% lower than AVVR Report−RF . AVVR AVV−Ao (4D) showed stronger agreement with AVVR Report−RF (κ = 0.50 [95% CI: 0.25–0.75]; r = 0.49, p < 0.01). AVVR Jet ​ demonstrated better concordance with AVVR Report−Cat (κ = 0.31 [95% CI: 0.17–0.45]). Both AVVR AVV−Ao (4D) (OR = 1.53, p = 0.004; AUC = 0.71) and AVVR Jet (OR = 1.74, p = 0.002; AUC = 0.65) were associated significantly with the composite outcome of death and heart transplantation listing. This is the first study analyzing the accuracy of 4D flow AVVR quantification in post-Fontan patients, demonstrating reproducibility, prognostic value, and good concordance with conventional methods. These findings suggest that 4D flow AVVR quantification may serve as a complementary method for assessing AVVR in post-Fontan patients.

Background: To explore the differences in exercise capacity between the extracardiac conduit (ECC) and lateral tunnel (LT) Fontan. Methods: 2169 patients (36% LT (n = 774); 64% ECC (n = 1395)) underwent a Fontan operation between 2000 to 2023 in a multi-institutional Fontan registry. LT patients were age-matched to ECC patients, and cardiopulmonary exercise test (CPET) results were compared. Following age-matching and exclusion of those without CPET data, 470 patients emerged with 235 LT and 235 ECC patients. Results: ECC achieved higher peak heart rates (174 vs. 169 bpm, p = 0.0008) and heart rates at ventilatory anaerobic threshold (VAT) (130 vs. 119 bpm p = 0.0005). Oxygen saturations at peak (93.0 vs. 90.0%, p = 0.0003) and baseline (95 vs. 92.5%, p < 0.0001) were higher in the ECC group. The VO2 at VAT was higher in the ECC (17.8 vs. 16.4 mL/kg/min p = 0.0123). Baseline pre-exercise heart rate, peak oxygen pulse, VE/VCO2 slope, peak VO2, peak % of predicted VO2, peak work rate, and peak % of predicted work rate were similar (all, p > 0.05). Notably, less than 35% of the cohort had a documented CPET. Conclusions: We found that the ECC performed statistically better on many parameters of exercise capacity, including the ability to increase heart rate, have higher peak and baseline saturations, and to achieve superior VO2 at VAT. However, the magnitude of difference was small, suggesting that the translational value into the clinical realm may be limited. With a minority of the registry patients having CPET completed, this illuminates the need for the implementation of CPET surveillance for Fontan patients.

BACKGROUND:The long-term effect of open fenestration in Fontan patients is unclear, leading to wide practice variation of fenestration creation and closure. We evaluated the long-term outcomes of the fenestration using data from the Fontan Outcome Registry using Cardiac Magnetic Resonance Examinations (FORCE) Study. METHODS:Patients were categorized by fenestration status determined by post-Fontan cardiac magnetic resonance imaging (CMR) as open fenestration, nonfenestrated Fontan, spontaneous closure, and device closure. The primary outcome was the time from the CMR to the earliest event of death, listing or receiving a heart transplant, plastic bronchitis, or protein-losing enteropathy. The association between fenestration status and the outcome measure was evaluated using Cox proportional hazard models, adjusted for patients' clinical and CMR characteristics. RESULTS:The cohort consisted of 2,923 patients with a median age at CMR of 14.3 years. Patients with open fenestration were younger and less likely to have a systemic left ventricle. Nonfenestrated Fontan patients were more likely to have a systemic left ventricle and lower indexed single ventricle end-diastolic volume (SVEDVi). An open fenestration was associated with adverse outcomes adjusted for clinical variables (hazard ratio 1.70, 95% CI [1.09, 2.64], P = .02). The association was no longer significant when adjusted for CMR variables, while every 10 mL/m2 increase in SVEDVi was associated with a 5% increase in the hazard of clinical outcomes (P < .0001). CONCLUSIONS:Open fenestration is associated with adverse outcomes when adjusted for clinical characteristics. The association disappeared when additionally adjusting for CMR variables. The current practice of fenestration closure in selected patients leads to comparable outcomes with spontaneous closure and nonfenestrated Fontan.

BACKGROUND:Disparities in congenital heart disease care exist between high-income and low- and middle-income countries (LMICs), likely extending to congenital cardiac catheterization (CCC). OBJECTIVES:This study compares patient characteristics and outcomes of CCC in the U.S.-based Congenital Cardiac Catheterization Project on Outcomes (C3PO) and the International Quality Improvement Collaborative-Congenital Heart Disease Catheterization Registry (IQIC-CHDCR) from LMICs. METHODS:The analysis included all CCC procedures recorded in C3PO (19 sites) and IQIC-CHDCR (19 sites) from 2019 to 2022. Patient and procedural characteristics, resource utilization, and outcomes were compared. RESULTS:A total of 28,957 C3PO and 6,485 IQIC-CHDCR cases were analyzed. Single ventricle patients accounted for 30% of C3PO and 13% of International Quality Improvement Collaborative (IQIC), with high-risk procedures (procedural risk in congenital cardiac catheterization 3-5) performed more frequently in C3PO (42% vs 23%). Median procedure duration was longer in C3PO (1.5 vs 0.8 hours). Clinically meaningful adverse event (CMAE) rates were higher in C3PO (3.9% vs 1.5%), though mortality was comparable (0.5% vs 0.7%). Risk-adjusted analysis showed a lower ratio in IQIC for both CMAE (0.50; 95% CI: 0.39-0.62) and severity level 4/5 events (0.71; 95% CI: 0.52-0.96). However, failure-to-rescue rates were higher in IQIC (7.1% vs 2.1%). CONCLUSIONS:The harmonized databases facilitated direct comparison of CCC practices, revealing more complex patients and resource-intensive procedures in C3PO, while the IQIC cohort demonstrated lower CMAE rates but a slightly higher mortality rate. These findings emphasize the need for further risk adjustment modeling for LMICs and identify areas to enhance global resource access and patient outcomes.

Background Technical success (TS) and procedural safety (PS) have been reported individually for transcatheter atrial septal defect (ASD) and patent ductus arteriosus (PDA) closures. A composite procedural performance (PP) metric as a patient-centered strategy has not been developed or studied. Methods A multicenter expert working group created PP metrics for ASD and PDA device closures as a composite of TS and PS. TS criteria were defined and categorized into 3 classes (optimal, satisfactory, and unsatisfactory). PS was defined using established adverse event (AE) definitions from the Congenital Cardiac Catheterization Project on Outcomes (C3PO) registry. PP was divided into 3 outcome classes (I to III). Retrospective C3PO data were collected for all cases of isolated ASD and PDA closure from 2014 through 2017. Exclusion criteria included complex congenital heart disease, significant comorbidities, ASD patients with multiple defects or ≥2 deficient rims, and PDA patients weighing <6 kg or with pulmonary hypertension. Factors correlating with class III (suboptimal) PP were analyzed. Results A total of 542 ASD and 688 PDA closure cases were included. Most ASD cases (99%) had optimal or satisfactory TS while 1% had a high severity AE. Class III PP occurred in 2% of ASD cases, mostly due to new mitral valve insufficiency. There were no identified patient or procedural factors associated with class III PP for ASD closures. Optimal or satisfactory TS occurred in 98% of PDA cases, with high severity AEs in <1%. Class III PP occurred in 2% of PDA cases, predominantly due to new arch obstruction, and was associated with younger age (P < .001) and lower weight (P = .001). Conclusions This study introduces PP as a composite variable to comprehensively measure outcomes of standard-risk ASD and PDA device closure. The incorporation of both TS and PS aims to better reflect patient outcomes compared to individual measurements alone. PP may serve as a valuable tool for identifying areas for further investigation and quality improvement.

BACKGROUND:Following the Fontan procedure, patients with single ventricle physiology are at high risk of diastolic dysfunction (DD) and elevated end-diastolic pressure (EDP). OBJECTIVE:This study aims to determine (1) the optimal EDP threshold correlated with adverse outcomes post-Fontan and (2) the clinical and imaging predictors of DD. METHODS:The study included patients from the Fontan Outcome Registry using CMR Examinations (FORCE) who underwent cardiac catheterization and cardiac magnetic resonance (CMR) within a 2-year window. The composite outcome was defined as all-cause mortality, sustained atrial or ventricular arrhythmia, plastic bronchitis, protein-losing enteropathy, or listing for transplantation. The EDP cutoff was determined using the lowest Brier score from Cox proportional hazard models. RESULTS:The study included 861 patients (mean age 16.4 ± 9.3 years). Mean EDP was 9.0 ± 3.5 mm Hg, with DD defined at an optimal EDP threshold >13 mm Hg. Patients were followed for a median of 3.6 years after catheterization. By univariable analysis patients with DD were more likely to have Fontan associated liver disease (40% vs 29%, P = .03) and kidney disease (19% vs 6%, P < .001). In multivariable analyses, DD was associated with the composite outcome (HR 3.37, 95% CI: 2.03-5.59, P < .001). Ninety-seven patients (11.3%) had DD. Multivariable analysis demonstrated that older age at catheterization, greater body mass index (BMI), nonleft ventricular morphology, and higher ventricular end-diastolic volume (EDV) were associated with DD. CONCLUSION:DD, defined as an EDP >13 mm Hg, is linked to over 3-fold higher risk of adverse outcomes. Risk factors for DD include older age, higher BMI, nonleft ventricular morphology, and larger EDV. The presence of risk factors may warrant screening catheterization to identify DD and modify care accordingly.

Background: The Da Silva Cone procedure for Ebstein anomaly has dramatically improved tricuspid valve competence and clinical outcomes. However, preoperative left ventricular (LV) dysfunction and immediate postoperative right ventricular (RV) systolic dysfunction are frequently observed. While excellent valve outcomes are well established, recovery of biventricular function following the Cone remains less defined. This study aimed to evaluate longitudinal changes in RV and LV function postoperatively and over a minimum of six months post-Cone operation. Methods: A single center retrospective review of 134 patients who underwent Cone repair for Ebstein’s anomaly from 2016 to 2024 was performed. Echocardiograms were analyzed at three time points: preoperative (Time 1), hospital discharge (Time 2), and ≥6 months postoperative (Time 3). RV parameters included fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), and tricuspid S′. LV parameters included left ventricular ejection fraction (LVEF), end-diastolic volume indexed to body surface area (LVEDVi), left ventricular stroke volume (LVSVi), and mitral E/E′. Subgroup analyses examined outcomes by prior Glenn, Starnes procedure, and degree of RV dilation. Paired two sample t-tests were used to compare serial measures. Results: Median age at surgery was 7.8 years (IQR: 2.3–17.7). All patients had discharge echocardiograms; 70 had follow-up studies at ≥6 months. RV function declined postoperatively with reductions in FAC (35% to 21%), TAPSE (2.0 to 0.8 cm), and S′ (13 to 5 cm/s), all p < 0.001. By Time 3, these measures improved (FAC to 29%, TAPSE to 1.3 cm, S′ to 7 cm/s) but did not fully return to baseline. LVEDVi and LVSVi increased significantly by Time 3 (LVEDVi: 47 to 54 mL/m2; LVSVi: 30 to 34 mL/m2; p < 0.001), while LVEF remained unchanged. Patients with prior Glenn or Starnes had greater Time 1 LV volumes and lower RV function, but by Time 3, most differences resolved. Moderate–severe preoperative RV dilation was associated with worse RV function at Time 2 and normalized by Time 3. Conclusions: The Da Silva Cone procedure leads to early postoperative RV dysfunction with partial recovery over the mid-term follow-up. Concurrently, LV filling and stroke volume improve, reflecting favorable interventricular interaction. These findings support echocardiographic surveillance to guide functional recovery post-Cone and inform patient counseling.

Background: The Fontan procedure has significantly extended the lifespan of patients with single-ventricle congenital heart disease. Atrial tachyarrhythmias (AT) are known to be associated with an increased risk of morbidity and mortality in this population. Understanding the anatomic, surgical, and clinical elements associated with AT is essential for risk stratification and guiding quality care. Research Question: What is the prevalence of AT in the Fontan population? What are the clinical, imaging, and surgical risk factors for AT development? Methods: The Fontan Outcomes Registry Using CMR Examinations (FORCE) was utilized for the analysis, which contains retrospective clinical and imaging data of Fontan patients from 42 centers. Cardiac MRI (CMR) data spanned 3/1999-1/2025. Patients were included if they had at least one CMR prior to AT diagnosis. The latest CMR assessment served as the starting point for modeling. AT was defined as atrial fibrillation, atrial flutter, or atrial tachycardia. Differences in characteristics between AT groups were assessed using Wilcoxon-Mann-Whitney or Chi-square/Fisher’s exact tests, as appropriate. Cox proportional hazards regression models were used to estimate hazard ratios for the relationship between predictors and risk of AT. Kaplan-Meier survival curves were used to compare freedom from AT across time between differing risk factor groups. Results: The study included 2,534 patients (42% female) with an average age at CMR of 15.7 years old. At a median follow-up of 2 years (IQR: 0.8-4.1 years) post-CMR, 266 (9.3%) developed AT with an overall incidence of 32.0 per 1000 person-years. Patients were 93.8%, 88.4%, and 78.3% free from AT at 2, 4, and 8 years, respectively. In the multivariable model, older age at CMR, atrial-pulmonary or lateral tunnel Fontan, >3 cardiac surgeries, higher BMI, and higher single-ventricle end-diastolic volume index (EDVi) were significantly associated with AT (Image 1&2). Kaplan-Meier analysis demonstrated that increased number of risk factors led to progressively worsened risk of AT (Image 3). Conclusion: Fontan type, number of surgeries, age, and BMI increase the risk of AT development in the Fontan population. Elevated single-ventricle EDVi was identified as a novel imaging risk marker for AT that underscores the utility of CMR in this population. Incorporating CMR into clinical risk stratification provides the opportunity to identify patients at risk for AT development post-Fontan.

BACKGROUND:The extracardiac conduit (ECC) and lateral tunnel (LT) are the most prevalent strategies for the Fontan operation. We used a multicenter database to compare long-term results. METHODS:First-time LT or ECC Fontans performed after the year 2000 in the Fontan Outcome Registry using Cardiac magnetic resonance Examinations (FORCE) registry were included. Propensity score matching was used. Outcomes were assessed individually as well as in a composite outcome that included death, listing for transplantation, sustained atrial arrhythmias, emergent cardioversion, plastic bronchitis, protein-losing enteropathy, and catheter-based intervention on the Fontan pathway. Cox proportional hazards models were used to compare hazards of outcomes between ECC and LT patients. RESULTS:Among 3072 patients (690 LT and 1182 ECC) in the registry, 1290 patients (645 LT and 645 ECC) were identified after matching. In matched samples, the Fontan composite outcome (32.4% vs 19.8%), sustained atrial arrhythmias (15.0% vs 5.0%), emergent cardioversion, defibrillation, or arrhythmogenic cardiac arrest (2.6% vs 0.8%), and Fontan pathway catheter-based intervention (7.1% vs 3.6%) were significantly higher for LT patients (P < .05 for all). Kaplan-Meier analysis demonstrated the freedom from the composite Fontan outcome at 5, 10, and 15 years was 94.5%, 88.3%, and 79.8%, respectively, for ECC patients compared with 90.2%, 80.9%, and 68.3%, respectively, for LT patients (P < .0001). ECC patients had lower hazards for atrial arrhythmia (hazard ratio, 0.33; 95% CI, 0.20-0.54; P < .0001) and the composite Fontan outcome compared to LT patients (hazard ratio, 0.72; 95% CI, 0.54-0.96; P = .0257). CONCLUSIONS:The ECC has substantially lower hazards for atrial arrhythmias compared with the LT Fontan, whereas other independent measures of longitudinal morbidity are similar.

The use of an oral positive expiratory pressure device (oPEP) with sniff breathing (Sniff-PEP) mimics biphasic ventilation. Biphasic ventilation increases pulmonary blood flow and cardiac output in Fontan patients. The aim of this study was to assess the effect of Sniff-PEP on Fontan flow velocities. A single-center, pilot, prospective study was carried out in 15 subjects with Fontan circulation enrolled to use the oPEP device for 1 month. Subjects were instructed on Sniff-PEP and to use the device for 10–15 min 3–4 times a day. Measurements of flow velocity and cardiac output were measured via echocardiogram and quality of life assessments were performed at baseline and 4–6 weeks later. The mean age at enrollment was 19.9 ± 8.7 years (age range of 10–37 years). 7 patients (47 https://clinicaltrials.gov . Unique identifier: NCT03251742.