Patients with cyanotic CHD and those with metabolic dysfunction-associated steatotic liver disease are at risk of liver fibrosis. We compared hepatic extracellular volumes and native T1 values to better understand the burden of liver disease in these populations. The sample comprised 136 patients in 5 groups: control (n = 23), metabolic dysfunction-associated steatotic liver disease [mild (F0-F1) and significant (F2-F4) fibrosis; n = 45], repaired tetralogy of Fallot (n = 30), and Fontan circulation (n = 38). Differences were assessed using linear regression models, with adjustment for the body mass index and sex. The hepatic extracellular volume was significantly larger in the Fontan group (43.96% ± 4.22%) than in the other groups, even with adjustment. Patients with Fallot had significantly larger extracellular volumes (36.77% ± 5.63%) than did controls and mild liver disease (p < 0.001 and p = 0.011, respectively), although smaller extracellular volumes than patients with significant liver disease (p = 0.042). These trends were corroborated by native T1 values, which were highest in patients with Fontan (1013.7 ± 86.1 ms), although not significantly different from patients with F2-F4 steatotic liver disease. The potential burden of CHD-related hepatic injury and steatotic liver disease highlights the importance of early identification. Given the possible additional risk of liver fibrosis in patients with coexisting metabolic dysfunction and CHD, comprehensive clinical management should prioritise regular metabolic risk assessment and the promotion of a healthy lifestyle to reduce the likelihood of liver disease development in this vulnerable population.

This study was performed to evaluate surgical outcomes in patients prenatally diagnosed with severe Ebstein's anomaly and a circular shunt. This retrospective study included 13 patients diagnosed with severe Ebstein's anomaly accompanied by a circular shunt on foetal echocardiography between 2012 and 2024 at our institution. All patients were delivered by planned caesarean section. Foetal hydrops was present in 4 patients. At birth, chest radiographs showed cardiothoracic ratios ranging from 90% to 100%, and echocardiography demonstrated severe pulmonary and tricuspid regurgitation in all cases. Surgical strategies-including main pulmonary artery ligation and the modified Starnes procedure-were evaluated based on perioperative clinical course, incorporating chest X-rays, echocardiograms, laboratory data, and urine output. The median follow-up after the modified Starnes procedure was 3.46 years (interquartile range, 2.9 years). All patients underwent surgical intervention on the day of birth: main pulmonary artery ligation in 7 and a modified Starnes procedure in 6. Of those who initially underwent ligation, 4 patients required conversion to the modified Starnes procedure on postoperative day 1, 2 patients on day 3, and 1 patient on day 6. One patient died of infection after the Glenn procedure. Of the remaining 12 patients, 7 completed Fontan circulation, and 5 were awaiting the Fontan. The 5-year postoperative survival rate was 92.3%. Surgical outcomes for severe Ebstein's anomaly with a circular shunt were excellent. However, the main pulmonary artery ligation as an initial palliation did not stabilize haemodynamics. Early implementation of the modified Starnes procedure appears critical to overcoming the haemodynamically unstable period immediately after birth.

Background: Ventricular function assessments in Fontan patients remain challenging. Ejection fraction (EF) lacks sensitivity for early dysfunction, and the roles of strain and advanced imaging in systemic left ventricle (LV) physiology are not fully defined. We aimed to compare (i) LV and atrial strain indices between pediatric Fontan patients with preserved EF (P-LVEF) versus reduced EF (R-LVEF) and (ii) echocardiographic global longitudinal strain, segmental longitudinal strain indices, and conventional 2D and 3D echocardiographic parameters through cardiac morphology. Methods: Pediatric patients with Fontan circulation and systemic LV morphology underwent clinical, hemodynamic, and multimodality echocardiographic evaluation, including 2D/3D parameters, global and segmental LV strain, and left atrial strain. Outcomes were analyzed according to EF status and congenital morphology. Significant results from multiple comparisons were followed by post hoc analysis, where appropriate. Results: Patients with a reduced EF exhibited a worse clinical status, a higher pulmonary vascular resistance index, and greater systemic congestion compared with those with a preserved EF. Conventional 2D indices showed no significant differences between the two studied groups except for LV end-systolic volume (ESV) (p = 0.0315) and LV end-systolic longitudinal diameter (ESL) (p = 0.0024), which showed higher values in the R-LVEF group. Although the relative frequency of impaired deformation was higher in Fontan patients with an unbalanced atrioventricular canal compared with the Fontan patients with a tricuspid atresia + pulmonary stenosis + ventricular septal defect, the difference did not reach statistical significance (p = 0.1365). Most segmental longitudinal strain values were not significantly different across patients with different cardiac morphology, except for the basal anterior segment and apical inferoseptal segment (p < 0.05). Conclusions: In pediatric Fontan patients with systemic LV morphology, a reduced EF was associated with a worse clinical and hemodynamic status. Conventional echocardiographic indices showed a limited ability to differentiate between the compared groups. Although no statistically significant differences were detected between pediatric Fontan patients with preserved EF and reduced EF, LV and atrial strain indices provided complementary information on ventricular–atrial interactions and myocardial deformation. These findings are exploratory and warrant confirmation in larger, prospective studies.

Stable Cytokine Network during Hypoxia and Exercise in Patients with Fontan Circulation.

Extracorporeal Membrane Oxygenation in Adults with Congenital Heart Disease: Considerations, Cannulation and Challenges for Complex Cardiac Anomalies.

Hepatic cholate clearance as assessed by HepQuant-SHUNT is predictive of clinical outcomes in individuals with Fontan circulation

Pulmonary vascular resistance in Fontan circulation: what are the prognostic implications?

Decrease in pulmonary vascular resistance (PVR) during exercise confers favourable prognosis in biventricular physiology, but its role in Fontan circulation remains unknown. This study aimed to evaluate the prognostic value of PVR index (PVRI) response to exercise in adults post-Fontan palliation. Retrospective cohort of 88 adults (age ≥18 years) post-Fontan undergoing exercise (supine cycling) cardiac catheterization. Patients were categorized into absence (n = 23) or presence (n = 65) of exercise-induced reduction in PVRI. The clinical endpoint was a composite of death or transplantation. Age at catheterization was 32.2 ± 8.8 years, and 45.5% were female. Resting and exercise PVRI were 1.9 ± 0.9 and 1.2 (interquartile range [IQR] 0.8-2.1) WU·m2, respectively. Clinical, biochemical and echocardiographic parameters were not associated with PVRI response to exercise. During a follow-up of 2.2 ± 1.4 (IQR 1.2-2.6) years, resting PVRI was not associated with the clinical endpoint (hazard ratio [HR] 0.95 per WU·m2, 95% confidence interval [CI] 0.52-1.74; P = 0.86), whereas exercise PVRI was (HR 2.15 per WU·m2, 95% CI 1.24-3.73; P = .007). Exercise PVRI remained predictive of outcomes after adjusting for exercise pulmonary artery wedge pressure (HR 2.08 per WU·m2, 95% CI 1.19-3.62; P = .01) or clinical risk factors individually. Two-year event-free survival was lower in the no PVRI reduction than in the reduction group (67% vs 95%; P < .001). In adults post-Fontan, PVRI response to exercise is a robust predictor of clinical outcomes and offers additive prognostic information beyond resting invasive haemodynamic data and clinical risk factors, underscoring the importance of invasive exercise testing in this population.

Impaired muscle function, aerobic capacity, and fatigue are common in individuals with Fontan circulation. Knowledge regarding the effects of strength training in this population is limited. Therefore, the study aimed to investigate the effects of strength training on dynamic muscle function, aerobic capacity, and fatigue in adults with Fontan circulation compared to matched controls. In this pilot non-randomised controlled trial, nine patients with Fontan circulation (median age 28.9 years [IQR: 23.4-35.0], 44.4% women) and nine age- and sex-matched controls completed a 10-week strength training intervention. Dynamic muscle function was assessed through shoulder flexion, heel rise, elbow flexion, and knee extension tests. Aerobic capacity was evaluated using cardiopulmonary exercise testing, and fatigue using the questionnaire Multidimensional Fatigue Inventory. All assessments were conducted pre- and post-intervention. Within-group changes were analysed using the Wilcoxon signed rank test and between-group differences using the Mann-Whitney U test. Patients showed improvements in all muscle function tests post-intervention (shoulder flexions 39.3% [IQR: 18.9-69.7], p = 0.008; heel rise 26.7% [IQR:17.5-58.1], p = 0.008; elbow flexions 57.1% [IQR: 50.0-173.8], p = 0.007; knee extensions 66.7% [24.3-92.9], p = 0.008). The improvements were at comparable levels to controls. Only controls reported reduced fatigue (-19.4% [IQR: -28.7, -10.5], p = 0.01), while patients showed no change (-5.9% [IQR: -25.5, 3.2], p = 0.1). Aerobic capacity remained unchanged. No severe adverse events occurred. Strength training is safe and improves dynamic muscle function in patients with Fontan circulation, with changes comparable to those of healthy controls. However, the effect of strength training on fatigue and aerobic capacity requires further investigation.ClinicalTrials.gov, ID: NCT05454254, https://clinicaltrials.gov.

Clinical Characteristics Associated With Variability in Biopsy Grade Severity of Liver Fibrosis in Fontan Circulation

Galectin-3 (Gal-3) is a biomarker associated with myocardial fibrosis, a key factor in the dysfunction of the functionally single ventricle (FSV) in patients after the Fontan procedure. This study aimed to evaluate the relationship between serum Gal-3 levels and echocardiographic and cardiopulmonary exercise parameters in this population. Thirty-seven patients (23 males, 14 females) with Fontan circulation were included. All underwent speckle-tracking echocardiography (STE), cardiopulmonary exercise testing (CPET), and serum Gal-3 measurement using ELISA. Correlations between Gal-3 and clinical, echocardiographic, and CPET parameters were analyzed. Gal-3 levels correlated positively with patient age and time since Fontan completion (p < 0.05). No significant associations were found between Gal-3 and ejection fraction, global longitudinal strain (GLS), or free wall strain. However, Gal-3 showed a significant correlation with the transmural strain gradient (p < 0.05). No association was observed between Gal-3 and CPET parameters, including peak VO2. Galectin-3 may reflect fibrotic remodeling of the FSV, as suggested by its correlation with the transmural strain gradient. The absence of a relationship with exercise capacity highlights the complexity of Fontan-related dysfunction. Gal-3 shows promise as a noninvasive biomarker of myocardial fibrosis in this unique patient group.

Management strategies of unplanned hospitalizations (UPH) vary and have not been well characterized in patients with Fontan circulation. We prospectively followed 3226 Fontan patients for 3 years to characterize the current UPH status and elucidate the associations between in-hospital management strategies and postdischarge outcomes. A total of 243 UPHs occurred (2.5 cases/100 person-years). Fontan-related UPHs accounted for 154 cases (63%), most commonly due to heart failure (19%), protein-losing enteropathy (16%), hemorrhage (14%), arrhythmia (9%), thromboembolism (3%), and plastic bronchitis (2%). Fontan-unrelated UPHs (89 cases; 37%) were mainly due to infections (19%). Adult patients were more frequently hospitalized for hemorrhage and thromboembolism (P<0.05). The longest length of hospital stay was observed in protein-losing enteropathy, followed by thromboembolism and heart failure. Among nonpharmacological treatments, oxygen therapy was commonly used, followed by surgical procedures and catheter-based interventions. Among pharmacological treatments, diuretics were commonly administered, followed by heparin and catecholamines. Of 240 patients discharged alive, 76 (15.4 cases/100 person-years) were readmitted. Protein-losing enteropathy was the most frequent cause of readmission, followed by heart failure and hemorrhage. In addition to conventional risk factors, in-hospital management practices, such as oxygen therapy, surgical intervention, and diuretic adjustment were associated with readmission risk (P<0.01-0.001). We clarified real-world practices related to UPHs in Fontan patients. Specific in-hospital treatment strategies were associated with the risk of readmission. Further studies are warranted to determine the causal relationships between treatment approaches and outcomes for standardized management strategies.

Live(r) with a Fontan circulation: A European survey and a proposed expert consensus on liver surveillance in collaboration with the EuroFontan group.

Systolic performance of the single ventricle, exercise capacity, and endothelial function in pediatric Fontan patients.

Tricuspid atresia (TA) is a cyanotic congenital heart defect defined by agenesis of the tricuspid valve and resultant right ventricular hypoplasia, representing 1.4–2.9% of congenital heart disease. Survival depends on interatrial and interventricular shunts that permit systemic and pulmonary blood flow, with staged surgical palliation culminating in the Fontan procedure. While surgical advances have improved long-term outcomes, Fontan circulation remains a delicate physiology characterized by preload dependence, elevated pulmonary vascular resistance, chronic venous hypertension, and a prothrombotic state. These features predispose patients to arrhythmias, lymphatic complications, hepatic congestion, and progressive circulatory failure. For anesthesiologists, perioperative management of TA and Fontan patients is uniquely complex. Anesthetic considerations include meticulous preload optimization, modulation of systemic and pulmonary vascular resistance, and ventilatory strategies that minimize adverse effects on venous return. Additional challenges include the high risk of air embolism, individualized anticoagulation needs, and hemodynamic sensitivity to patient positioning. Preoperative evaluation with echocardiography and electrocardiography provides critical insight into anatomy and physiology, while intraoperative planning must emphasize goal-directed fluid management, careful agent selection, and tailored ventilation. Postoperatively, vigilant monitoring, effective pain control, and prevention of complications are essential. This review synthesizes classification systems, pathophysiology, and the evolution of surgical palliation, while emphasizing anesthetic principles for the perioperative care of patients with TA and Fontan circulation. As survival improves and the population of Fontan patients expands, a nuanced understanding of this physiology is essential for optimizing outcomes across cardiac and non-cardiac surgical settings.

Background: The Fontan procedure is a functional palliative surgery for patients with univentricular congenital heart disease. However, Fontan circulation exhibits unique hemodynamics, with chronic elevation of central venous pressure and decreased cardiac output, leading to hepatic venous congestion, reduced portal blood flow, and progressive liver fibrosis, collectively known as Fontan-associated liver disease (FALD). Advanced FALD cases may progress to cirrhosis or hepatocellular carcinoma. Because liver biopsy in FALD carries a risk of bleeding, noninvasive fibrosis assessment methods are needed. This study aimed to analyze the associations among the spleen index (SI), Fibrosis-4 index (FIB-4), and aspartate aminotransferase (AST)-to-platelet (PLT) ratio index (APRI) and ultrasound findings in FALD to examine their utility as noninvasive monitoring indicators.Methods: This single-center retrospective observational study included 46 post-Fontan patients referred between 2018 and 2024. Data on age, sex, years after surgery, circulatory parameters (central venous pressure and B-type natriuretic peptide), and blood test findings were collected, and FIB-4 and APRI were calculated. SI was calculated as the product of splenic diameter and thickness (cm×cm) on abdominal ultrasound, with SI ≥20 indicative of splenomegaly. Two blinded evaluators independently assessed fine hyperechoic foci. Analyses included the following: (1) comparisons between SI groups (≥20 vs. <20) and between FIB-4 groups (median: 0.682), (2) correlation analysis between SI and FIB-4, and (3) comparison of one-year FIB-4 change (ΔFIB-4) among three risk groups.Results: The median patient age was 19 years (range: 11-52 years), and the median post-Fontan duration was 14 years (range: 4-40). Splenomegaly was present in 28 patients (61%). Compared with the no splenomegaly group, the splenomegaly group had significantly lower PLT counts and higher FIB-4. Compared with the low FIB-4 group, the high FIB-4 group had significantly higher age and postoperative years, higher B-type natriuretic peptide, lower PLT count, and more frequent fine hyperechoic foci. The SI and FIB-4 scores showed a significant positive correlation (r=0.46; p=0.0013). The FIB-4, but not SI, showed a strong positive correlation with age (r=0.68; p<0.001). Comparisons among the three risk groups showed a significant difference in ΔFIB-4 (p=0.005). Compared with the low-risk group, the high-risk group showed approximately twice the FIB-4 increase (p=0.003), suggesting a threshold effect for fibrosis progression. SI and FIB-4 are noninvasive indicators of liver fibrosis progression in FALD, reflecting different pathophysiological aspects.Conclusions: SI and FIB-4 are noninvasive indicators of liver fibrosis progression in FALD, reflecting different pathophysiological aspects. SI may reflect the circulatory burden that appears relatively early after surgery, whereas FIB-4 reflects fibrosis accumulation due to aging and chronic congestion. A simultaneously elevated SI and FIB-4 may be associated with rapid fibrosis progression. A combined assessment may be useful for risk stratification and longitudinal monitoring. The relatively low threshold of FIB-4 ≥0.682 may be a practical indicator to detect early FALD-specific fibrosis. Simple monitoring combining splenic assessment by ultrasound and FIB-4 calculation from blood tests can help in the early detection and prognostic evaluation of FALD.

Fontan associated liver disease is a poorly understood complication of long-term single ventricle palliation. This study sought to describe serial liver function changes in patients with a Fontan circulation from 2014 to 2020 and determine if these findings can be used to predict adverse clinical outcomes. We conducted a retrospective chart review of Fontan patients age≥18 who were followed by a specialist clinic between 2014 and 2020. Annual bloodwork, including liver enzymes and AFP (alpha-fetoprotein), and liver imaging were analyzed. Common liver function scores were calculated. Trends over time and their correlation to clinical events were assessed. Events were defined as congestive heart failure, cardiac hospitalization, thrombosis, protein losing enteropathy, and death. The study assessed possible associations with the composite event of adverse outcomes. One hundred twelve Fontan patients were included with a mean age of 24.9±8.1 years. Age-adjusted AFP and creatinine increased over the study period, whereas alkaline phosphatase decreased. There was no significant change in other liver biomarkers over the study period. The proportion of patients with higher liver imaging scores increased over the study period. Over a follow-up time of 6.1 years (95% control limit, 5.5-6.9 years), 44 (39.3%) patients experienced at least 1 event, most commonly cardiac hospitalization. In multivariable analysis, age was associated with a greater risk of events (hazard ratio, 1.035 [95% CI, 1.008-1.063]; P=0.010); no other biomarkers or scores were significant. Routine liver biomarkers do not increase over time despite increasing proportion of patients with higher liver imaging scores over the same time. Although our sample size was limited, current modalities to assess progression of liver dysfunction on bloodwork and imaging were not related to clinical outcomes.

Completing the Fontan circulation in patients with heterotaxy syndrome and complex cardiac anatomy presents unique surgical challenges. We report a case in which a modification of an intra-atrial lateral tunnel (LT) technique was utilized during the Fontan procedure. The patient, who had previously undergone staged single-ventricle palliation at our institution, was admitted with worsening cyanosis for Fontan completion. Intraoperatively, the intra-atrial tunnel was positioned posteriorly to redirect the hepatic venous return to the left atrial appendage with a posterior tract running on the left of the pulmonary veins. This case provides a variation of the lateral tunnel technique.

Atrial Isomerism Adversely Impaired both Early and Late Outcomes in Fontan Circulation.

Background and aimsFunctionally univentricular hearts (UVH) are a relatively rare congenital heart defect and often require staged palliation leading to the Fontan circulation. The bidirectional superior cavopulmonary anastomosis (BSCA) or bidirectional Glenn shunt (BDG) is a key intermediate procedure that partially separates systemic and pulmonary circulations. Although traditionally delayed until six months of age, earlier BDG has become common. This study compared outcomes in patients undergoing BDG at six months or younger versus seven months to one year and examined time to Fontan completion (FO), outcome predictors, and the influence of early BDG in the Indian context.MethodsIn this retrospective observational study, 173 patients with UVH underwent BDG at age one year or younger from January 2011 to December 2020 at a single tertiary care center. Twenty-seven patients (Group A) were six months or younger, and 146 (Group B) were seven to 12 months. Preoperative, intraoperative, and postoperative data were collected, including morbidity, mortality, and follow-up assessments. The data were statistically analyzed by Fisher’s exact test, Pearson’s chi-square test, and Cox proportional hazards or logistic regression modeling.ResultsEarly mortality was 6.93% (12/173). Mortality was significantly higher in Group A (5/27; 18.52%) than in Group B (7/146; 4.79%; p=0.01). On multivariate analysis, preoperative mechanical ventilation, maximum inotropic score, and maximum vasoactive inotropic score (VIS) were independent predictors of mortality rather than patient age alone. Despite the higher initial risk, infants in Group A benefited from earlier unloading of the systemic ventricle and demonstrated less pulmonary artery (PA) distortion at follow-up.ConclusionsAlthough BDG before age six months carries a higher early mortality risk, primarily linked to clinical severity rather than age, it can offer important advantages in systemic ventricle offloading and reduced PA distortion over time. These findings underscore the need for careful patient selection and highlight the potential for improved long-term outcomes with early BDG in appropriately selected infants.