Background Risk stratification for ventricular arrhythmia in congenital heart disease (CHD) remains challenging, and the role of programmed ventricular stimulation is incompletely defined. Objective This study evaluated long-term outcomes after electrophysiology study (EPS) for ventricular arrhythmia assessment in CHD practice. Methods Retrospective, single-center cohort study including consecutive patients with structural CHD who underwent EPS with programmed ventricular stimulation for ventricular arrhythmia evaluation between 1995 and 2022. Baseline characteristics were compared between inducible (EPS+) and non-inducible (EPS-) patients. The primary outcomes were a composite of all-cause mortality and heart transplantation (HTX) and a composite ventricular arrhythmia (VA) outcome. Results Among 174 patients, median age at EPS was 33.8 years (IQR 24.1-46.1), 65 (37.4%) were female, 106 (60.9%) had conotruncal CHD, and 37 (21.3%) were EPS+. EPS+ patients more often had conotruncal CHD, abnormal RVEF, and longer PR and QRS duration. During a median follow-up of 13.0 (8.1-19.4) years, there was no significant difference in mortality/HTX (EPS+ 24.3% vs EPS- 18.2%, log-rank p=0.924) or VA outcome (EPS+ 27.0% vs 12.4%, log-rank p=0.113) between EPS+ and EPS- patients. After a median time of 2.8 (0.3-4.5) years 28 EPS- patients (20.4%) received an ICD. Of these, 16 (57.1%) received the ICD for out-of-hospital cardiac arrest or sustained ventricular tachycardia. Conclusions In patients with CHD referred for EPS for ventricular arrhythmia evaluation, a positive EPS was associated with a distinct phenotype but not with worse long-term mortality/HTX or VA outcome. A negative EPS did not exclude clinically relevant arrhythmic risk.
Background Mechanical valve thrombosis is a serious complication in adults with congenital heart disease (ACHD). Although low-dose thrombolytic therapy has emerged as a potential alternative to surgery, evidence in ACHD patients with right-sided mechanical valve thrombosis remains limited. We evaluated the feasibility and safety of a standardized low-dose thrombolytic protocol in this population. Methods We retrospectively reviewed consecutive ACHD patients with right-sided mechanical valve thrombosis treated at our institution between 2022 and 2025. Diagnosis was based on fluoroscopic, echocardiographic, and computed tomography findings. All patients received a standardized low-dose alteplase regimen (25 mg over 6 hours). Treatment efficacy was assessed primarily by restoration of prosthetic leaflet mobility on fluoroscopy. Results Five patients were included, including four with mechanical pulmonary valves and one with a mechanical tricuspid valve. Thrombosis occurred a median of 2.3 years after valve replacement, and all patients had subtherapeutic anticoagulation (international normalized ratio <2.0) during the preceding month. At presentation, four patients were in New York Heart Association functional class II and one in class III. Following a median of 5 days of thrombolytic therapy, complete restoration of prosthetic leaflet mobility was achieved in all patients. No major bleeding or thromboembolic complications occurred. During a median follow-up of 0.7 years, no recurrent valve thrombosis was observed. Conclusions Standardized low-dose thrombolytic therapy was safe and feasible in this case series and may provide a non-surgical treatment option for ACHD patients with right-sided mechanical valve thrombosis.
Introduction:Pharmacological therapy has transformed the management of heart failure, yet pivotal trials have primarily focused on acquired left ventricular disease. Evidence for renin-angiotensin-aldosterone system (RAAS) inhibition in systemic right ventricle (sRV) remains scarce, and current practice is largely based on extrapolation from left ventricular studies and expert consensus. This meta-analysis evaluates the efficacy and safety of RAAS blockade in patients with a failing sRV. Methods:A PubMed search identified studies investigating RAAS inhibition in sRV failure. Reported outcomes included changes in NT-pro-BNP, right ventricular ejection fraction (RVEF), and peak oxygen uptake (VO2-max). Data were analyzed using conventional statistics and visualized with forest plots. Results:Ten eligible studies were included, comprising a pooled cohort of 325 patients, of which 261 received active treatment. The population was predominantly male (67.1%), with mean cohort ages ranging from 25.2 to 48.5 years. Mean treatment durations spanned 3.5 to 37.2 months. RAAS inhibition was associated with a reduction in NT-pro-BNP (Cohen's d = -0.28, 95% CI -0.49 to -0.07, p = 0.01). However, no improvements in RVEF or VO2-max were observed. The therapy was generally well-tolerated. The overall mortality incidence was 5.6 deaths per 1000 person-years in the active treatment groups. Conclusion:The evidence for RAAS inhibition in patients with sRV is limited but suggests a beneficial reduction in NT-pro-BNP, indicating a hemodynamic effect but without improvements in RVEF or VO2-max. When combined with extrapolated data from large left ventricular heart failure trials, these findings support that RAAS inhibition is safe and may be considered in patients with sRV failure.
Background Pulmonary valve replacement remains challenged by structural valve deterioration and the need for reinterventions. The RESILIA bioprosthesis incorporates anti-calcification technology designed to improve tissue durability. This study assessed early hemodynamic performance of the RESILIA valve in the pulmonary position. Methods This retrospective study included 28 patients who underwent Resilia-based PVR at a single center between 2021 and 2024. Demographics, surgical details, and echocardiographic data were retrieved, focusing on peak transvalvular gradient and severity of pulmonary regurgitation (PR). Linear mixed-effects modeling was applied to account for repeated measurements at variable follow-up intervals. Results A total of 28 patients underwent pulmonary valve replacement with the RESILIA valve, with no procedural deaths. Median follow-up was 18 months (interquartile range 11–27; range 0–38). A total of 86 postoperative echocardiograms were analyzed. The median peak gradient was 13.0 mmHg (interquartile range 10.2–19.4; range 4.0–31.4). Patient-level worst pulmonary regurgitation during follow-up was none in 18 patients (64.3%), mild in 9 (32.1%), and moderate in 1 (3.6%); no severe regurgitation occurred. No valve-related reinterventions, valve thrombosis, or endocarditis were observed during follow-up. Peak gradient increased modestly over time (+0.155 mmHg/month; 95% confidence interval 0.059–0.251; p=0.002). Conclusions In this early single-center experience, surgical pulmonary valve replacement with the RESILIA bioprosthesis was feasible, with predominantly none-to-mild regurgitation and no reinterventions during short-term follow-up. The modest increase in gradients over time supports continued surveillance and highlights the need for longer-term, multicenter studies with standardized right ventricular outflow tract prosthesis assessment to define durability.
Introduction and objective:Adults with congenital heart disease affecting the right ventricular outflow tract often require repeated pulmonary valve replacements (PVR) throughout life, making prosthesis durability a critical determinant of long term outcomes. Bovine pericardial and porcine bioprosthetic valves are the most widely used in surgical PVR, yet comparative durability data remain inconsistent, partly due to reliance on nominal valve size rather than functional parameters. This study aimed to compare long term durability between both valves using indexed effective orifice area (iEOA) and analytic methods focused on prosthesis related performance. Methods:We conducted a retrospective, multicenter observational study including pediatric and adult patients undergoing surgical PVR between 2013 and 2024 at three tertiary hospitals in Spain. The primary endpoint was a composite outcome of all cause mortality, reintervention, and structural valve deterioration. Propensity scores were constructed using clinically selected covariates and applied through inverse probability weighting (IPW). To isolate prosthesis related durability, survival was analysed by Cox regression model with left truncation at 1 year. Results:A total of 231 patients were included (49.4% female; 64.1% with tetralogy of Fallot). Overall, 19.0% experienced a MACE during follow up, occurring at a median of 4.8 years after the truncation point. After IPW adjustment, baseline characteristics were well balanced. In the IPW adjusted Cox regression model, porcine prostheses were associated with a lower hazard of MACE (HR 0.37; 95% CI 0.16-0.83; p = 0.015). Conclusions:When early postoperative events are excluded and baseline differences minimized, porcine bioprostheses exhibit superior mid and long term durability compared with bovine pericardial valves.
Introduction:Robotic magnetic navigation (RMN) allows for catheter ablation in the presence of complex cardiac anatomy, such as that seen in congenital heart disease (CHD). The objective of this study was to evaluate the feasibility, efficacy and safety of this procedure in a wide range of CHD conditions. Methods:Patients with CHD who underwent catheter ablation for cardiac arrhythmias using RMN were included in this single-centre observational study. Patients' baseline characteristics, procedural data, periprocedural complications and acute success rates were obtained. The efficacy of the ablation was evaluated during follow-up in the outpatient clinic using 24-h Holter monitoring. Results:The study population consisted of 38 patients (26 males) with a median age of 36.8 (IQR 30.8-39.6) years. Surgically corrected transposition of the great arteries was the most common CHD, observed in 14 patients (36.8%). Nineteen patients (50.0%) had more than one congenital defect. Intra-atrial re-entrant tachycardia was the most common arrhythmia ablated, accounting for 21 procedures (55.3%).Median procedural time was 232.5 min (IQR 186.3-281.3); the median radiation dose and radiation time were 2.4 mGy m2 (IQR 1.3-4.4) and 14.0 min (IQR 9.5-30.4), respectively. Acute arrhythmia cessation was observed in 81.6% of procedures. Major complications were noted in 2 cases (5.3%). The median time from the ablation to follow-up was 5.4 months (IQR 3.4-9.3). Arrhythmia recurrence was observed in 7 cases (18.4%). Conclusions:RMN is a valuable tool for catheter ablation of arrhythmias in patients with congenital heart disease and complex anatomy.
Background:Perimembranous ventricular septal defect (PM-VSD) with right coronary cusp (RCC) prolapse carries a high risk of progressive aortic regurgitation and often lacks a measurable aortic rim, complicating device closure. While surgery remains the standard treatment, transcatheter therapy using the Amplatzer Duct Occluder II (ADO II) offers a less invasive option, though data in Middle Eastern populations are scarce. Objective:To evaluate the feasibility, safety, and intermediate-term outcomes of ADO II closure in PM-VSD patients with RCC prolapse. Methods:We conducted a retrospective multicenter study of 29 consecutive patients (mean age: 8.86 ± 5.77 years; 55.2% male) with PM-VSD and RCC prolapse who underwent ADO II closure at two Iraqi cardiac centers (January 2023-November 2024). Inclusion criteria required an effective flow diameter ≤3 mm (left ventricular side), hemodynamic significance (Qp:Qs > 1.5 or left ventricular volume overload), and absence of severe aortic regurgitation or pulmonary hypertension. Results:Device implantation succeeded in 28 of 29 patients (96.6%). Immediate complete closure occurred in 23 patients (79.3%); 5 had small residual shunts (<2 mm). One patient required surgical conversion. At 6 months, complete closure persisted in 23 patients (79.3%), and 27 patients (93.1%) had favorable outcomes (complete closure or mild valvular regurgitation only). Critically, no new-onset aortic regurgitation developed, and baseline mild aortic insufficiency did not worsen. Conclusion:Transcatheter PM-VSD closure with ADO II is feasible, safe, and effective in carefully selected patients with RCC prolapse-even without a traditional aortic rim. This first systematic multicenter experience from Iraq supports expanding minimally invasive therapy for this high-risk subgroup in resource-constrained settings.
Background:The available data about the prognostic role of N-terminal pro-B-type natriuretic peptide (NT-proBNP) in adults with congenital heart disease (CHD) are derived from small sample size with limited follow-up. The purpose of this study was to assess the role of NT-proBNP assay for prognostication in adults with CHD based on a large cohort. Method:Retrospective cohort study of adults with CHD, biventricular physiology, and systemic morphologic left ventricle who had ≥2 NT-proBNP measurements (2003-2023). Temporal change in NT-proBNP was calculated as relative change from baseline levels (relative Δ_NT-proBNP). Results:Of 3674 patients (age 42 ± 16 years; 47% males), median baseline and follow-up NT-proBNP levels were 178 (72; 539) pg/ml and 236 (103; 854) pg/ml, respectively. The relative Δ_NT-proBNP was 22% (-9, 51), and 2496 (68%) had temporal increase in NT-proBNP levels (i.e., relative Δ_NT-proBNP >0). The correlates of higher baseline NT-proBNP measurement were older age, atrial fibrillation, hepatorenal dysfunction, biventricular systolic dysfunction, and elevated biventricular filling pressures. Overall, 431 (12%) patients died during median follow-up. Both baseline NT-proBNP level and temporal increase in NT-proBNP were associated with all-cause mortality in all patient subgroups. Serial NT-proBNP measurement had superior prognostic value compared to single (baseline) NT-proBNP measurements and had superior prognostic performance compared to conventional clinical indices (echocardiographic indices, invasive hemodynamic indices, exercise test indices). Conclusions:NT-proBNP assay is inexpensive, readily available, and noninvasive, making it ideal for longitudinal monitoring. Further studies are required to determine whether NT-proBNP data can be used to guide therapeutic interventions in this population.
Background: Adults with congenital heart disease (ACHD) have a rising prevalence of atrial fibrillation/flutter (AF) and elevated thrombotic and bleeding risk. Although direct oral anticoagulants (DOACs) are established in non-ACHD AF, comparative effectiveness in ACHD is not well defined. Methods: In a propensity score–matched retrospective cohort study using the TriNetX database, adults (age ≥18) with ACHD and AF receiving oral anticoagulation were identified, excluding those with mechanical valves, LVAD, antiphospholipid syndrome, or rheumatic mitral stenosis. Patients were matched 1:1 to DOAC or warfarin. The primary outcomes were composite embolic events, composite bleeding events, and net clinical outcome (embolism, bleeding, or all-cause mortality). Secondary outcomes included gastrointestinal bleeding, nontraumatic intracranial hemorrhage (ICH), cardioembolic stroke, systemic arterial embolization, cerebral infarction, and all-cause mortality. Associations were estimated using hazard ratios (HRs). Sensitivity analysis was performed to confirm the robustness of magnitude and direction of outcomes. Results: After matching, 27,166 patients were included (13,583 per group). DOAC use was associated with lower hazard of net clinical outcome (HR 0.87, 95% CI 0.81–0.93), driven by reduced composite bleeding (HR 0.82, 95% CI 0.75–0.90) and all-cause mortality (HR 0.68, 95% CI 0.64–0.72). Hazard of composite embolic events did not differ (HR 0.94, 95% CI 0.87–1.03). DOACs were associated with lower hazard of systemic arterial embolization (HR 0.62, 95% CI 0.52–0.73), nontraumatic ICH (HR 0.74, 95% CI 0.61–0.90), and gastrointestinal bleeding (HR 0.84, 95% CI 0.76–0.93). No significant differences were observed for cerebral infarction or cardioembolic stroke. Conclusions: In a large propensity-matched cohort of patients with ACHD and AF, DOAC therapy was associated with lower hazard of net clinical outcome, all bleeding events, and all-cause mortality compared with warfarin, without increased embolic risk.
Background: Fontan associated liver disease (FALD) is a known complication in adult patients with total cavo-pulmonary connection. The relationship between the severity of FALD and cardiovascular disease progression is yet to be clarified. We investigated the role of liver stiffness measured by transient elastography (TE) in predicting adverse events (AEs) in adult patients with Fontan circulation. Material and methods: We retrospectively analysed TE data from our cohort of Fontan patients from September 2014 to December 2023. Baseline characteristics were recorded. The association of TE, liver function tests, and pro-collagen3 with AEs were analysed. Results: 167/207 patients (median age 20.0 years; 60% male) for a total of 613 TE from September 2014 to December 2023 (median follow up 56.0 months; 1.893 patient-year) were included in the study. TE values remained stable over time and did not show any correlation with baseline characteristics. TE values < 23.9 kPa could discriminate patients with advanced FALD (Se 50%; Sp 79%; NPV 97%). Forty AEs occurred (0.06 events per patient-year). Albumin (HR 0.91, p = 0.010) and APRI (HR 1.92, p = 0.034) were independent predictors of morbidity at multivariate analysis. Conclusions: In this relatively young cohort, with low mortality and no HCC during follow-up, no significant association was found between TE and AEs. Nevertheless, TE appeared to be useful in discriminating advanced FALD. As no convincing risk factor related to FALD severity or progression has been yet identified, a multidisciplinary approach to risk assessment and surveillance is advised to reduce the impact of FALD and prevent progression to HCC.
Introduction:Poverty is linked to an increased risk of acquired heart disease, but its impact on outcomes in adults with congenital heart disease (CHD) is not well defined. Medicaid insurance is a strong proxy for poverty in United States. We characterize population characteristics and healthcare utilization among adults with CHD on Medicaid insurance, both overall and by CHD disease complexity. Methods:The Transformed Medicaid Statistical Information System (T-MSIS) collects comprehensive public health insurance data from all 50 states and territories (Medicaid and Children's Health Insurance Program). We identified 260,535 adults (>18 years of age) with ICD-10 diagnosis codes consistent with CHD across the years 2016-2018. Descriptive statistics were used to compare groups. Results:Of 260,535 adults with CHD studied, 63.1% were female with a mean age of 40.2 ± 19.1 years. 44.5% had moderate or complex CHD, and 5.8% died during the study period. Medical and cardiac comorbidities were common: rhythm disorders (46.7%), mental health diagnoses (44.9%), hypertension (44.6%), and metabolic syndrome (42.4%). One quarter (24.8%) had a disability, 15.5% had a diagnosis consistent with substance abuse, and 17.7% used nicotine. 23.9% had incidence equivalents of one or more hospitalizations per year, and 32.1% had incidence equivalents of ≥5 emergency department visits in three years. Conclusion:We characterized the population of adults with CHD on Medicaid and found high rates of medical comorbidities, disability, smoking, substance use, and utilization of emergency department services. This vulnerable population may benefit from targeted interventions aimed at reducing comorbid conditions and thus improving long term outcomes.
Background:Randomized controlled trials represent robust foundational material for evidence-based clinical practice in cardiology; however, such trials appear to be scarce in congenital heart disease (CHD). No bibliometric analysis has formally quantified nor characterized this gap in the literature, nor examined the impact of existing medication studies in CHD patients. Objectives:This investigation aimed to evaluate and characterize existing medication studies in congenital heart disease. Methods:PubMed, Embase, Web of Science, Cochrane Library, Emcare and Academic Search Premier were searched (Jan 2000-May 2024) for CHD drug studies. Trials focusing on genetic conditions associated with cardiovascular abnormalities (e.g. Marfan syndrome) and pulmonary hypertension not associated with congenital heart disease were excluded.PROSPERO ID: CRD420251073438. Results:A total of 160 CHD medication studies involving 59,208 participants were included, spanning a study period of 24.5 years (2000-2024). Sex was reported for 20,092 participants; 10,514 (52.3%) were women. Only 29.4% (n = 47) of trials were RCTs. Time-series breakpoint analysis showed an inflection in annual publication output in 2013 (p = 0.009), with growth before and contraction thereafter. Pulmonary-hypertension drugs accounted for 58.1% of publications. Collectively, industry-funded trials were over twice as likely to be RC type trials (56.7% vs 23.1% in non-industry funded studies). Conclusions:Contemporary CHD pharmacotherapy research is limited in spectrum/demographics and appears to have been plateauing globally since 2013. Many studies are single-center, pulmonary-hypertension-focused and not placebo-controlled. There is a need to develop a broader disease evidence-base for medication interventions in CHD.
Background:Right ventricular (RV) function is crucial in the prognosis of adults with congenital heart disease (ACHD). However, its relationship with exercise capacity (EC) and quality of life (QoL) in ACHD remains underexplored. Despite the established benefits of physical activity in CHD, there is limited knowledge regarding the cardiac effects of exercise training in ACHD. Aim:To assess the relationship between RV function, comprehensively evaluated using two-dimensional multiplane echocardiography (2D MPE), and both EC and QoL in adults with various types of CHD. Additionally, to investigate the effect of a 16-week home-based aerobic and strength exercise program on these parameters. Methods:Fifty-five ACHD underwent transthoracic echocardiographic assessment of RV function, including conventional parameters and longitudinal RV strain, both measured from four RV walls using 2D MPE. The patients also completed a cardiopulmonary exercise test and a QoL questionnaire, and were randomized to either conventional care or a 16-week home-based rehabilitation program combining aerobic interval cycle training and dynamic strength exercises, followed by retesting. Results:At baseline, RV function correlated with EC, measured by percent-predicted peak oxygen consumption. Moreover, significant correlations were observed between RV and QoL parameters, including physical functioning, general health perception and visual analogue scale score. EC, muscle strength and QoL improved following the exercise program, while 39% of patients in the intervention group were non-adherent. Conclusion:RV function correlates with EC and QoL in a diverse ACHD population. ACHD patients should be encouraged to engage in regular exercise, and exercise training should be integrated into CHD treatment.
Background: Adults with congenital heart disease (ACHD) have a lifelong health condition that may diminish quality of life (QOL) and cause serious illness. Promoting resilience and advance care planning (ACP) can both improve QOL. The relationship between resilience and patients’ comfort discussing ACP is unknown. Objectives: This study evaluates the association between patients’ self-reported resilience and comfort discussing ACP. It also assesses the association between prior clinical events and resilience and comfort discussing ACP. Methods: We conducted a cross-sectional study of outpatients with moderate or complex ACHD. Self-reported resilience and comfort discussing ACP were evaluated using surveys. Demographic and clinical data were collected from surveys and health records. We performed linear and logistic regression to evaluate relationships. Results: Among 149 patients (41 ± 14 years; 52% female), 84% were non-Hispanic White and 28% had complex ACHD. Mean resilience was 29.0 ± 7.4; 42% reported being very comfortable talking about ACP with family and friends, and 56% with their ACHD clinician. Higher resilience was associated with higher odds of feeling very comfortable discussing ACP with families and friends (OR 1.06, 95% CI 1.01–1.12, p = 0.015) and ACHD clinicians (OR 1.10, 95% CI 1.04–1.15, p < 0.001). Participants with prior mental health clinical events (therapist encounter, psychiatric medication prescription, mental health provider encounter) tended to report lower mean resilience and similar comfort discussing ACP. Conclusion: Patients who reported higher resilience were more likely to feel comfortable discussing ACP. Strengthening resilience may be an avenue for supporting patients with ACHD to more comfortably engage in ACP.
Background:Heart failure (HF) hospitalization is a marker of HF progression, and it is associated with mortality in adults with congenital heart disease (CHD). The purpose of this study was to assess the effect of guideline directed medical therapy (GDMT) on HF readmission and mortality in CHD patients with HF with reduced ejection fraction (HFrEF). We hypothesized that higher GDMT use was associated with lower risk of HF readmission and mortality. Method:Retrospective study of CHD patients and HFrEF, admitted for HF (2003-2023). GDMT use was assessed at hospital discharge (baseline) and 1-year follow-up using a standardized GDMT score. GDMT uptitration was assessed as difference between GDMT score at baseline versus 1-year follow-up. Results:Of 153 patients (age 51 ± 15 years, 39% males, left ventricular EF 29 ± 7%), the median baseline GDMT score was 2 (1, 3). Baseline GDMT score was associated with lower risk of HF readmission (hazard ratio [HR] 0.73, 95% confidence interval [CI] 0.54, 0.92, p < 0.001) and mortality (HR 0.71, 95%CI 0.50, 0.93, p < 0.001) per 1-unit increase in baseline GDMT score. Among patients with 1-year follow-up (N = 128), GDMT uptitration was associated with lower risk of HF readmission (HR 0.72, 95%CI 0.49, 0.94, p < 0.001) and mortality (HR 0.69, 95%CI 0.41, 0.92, p = 0.02) per 1-unit increase. Patients with GDMT uptitration (N = 49, 38%) had greater improvement in neurohormonal activation and left ventricular systolic function, consistent with a lower risk of HF readmission and mortality in that group. Conclusions:GDMT optimization was associated with lower risk of HF readmission and all-cause mortality amongst adults with CHD. Further studies are required to determine whether strategies to improve GDMT optimization would improve clinical outcomes in this population.
Background:Prosthetic 'tricuspid valve' placement in Bjork Fontan patients born with tricuspid atresia can transition these patients to a biventricular circulation. Objective:Describe intermediate term results of hybrid prosthetic valve placement in Bjork Fontans using annuloplasty ring placement around the Bjork connection followed by transcatheter valve. Methods:A retrospective review of hybrid attempts to valve large Bjork Fontans at UCLA. Only Bjorks that had failed balloon sizing for transcatheter valve placement or had prohibitively large dimensions on cross sectional imaging were included. All had attempted surgical placement of a non-circumferential annuloplasty ring around the Bjork Fontan and subsequent Sapien valve implant. Results:Median age of the five patients was 40-years. Three had successful placement of annuloplasty rings with a 30 mm (n = 2) or a 34 mm Edwards Physio Annuloplasty ring followed by successful valving with 29 Sapien 3 valves. In two patients, attempts to place a surgical ring resulted in bleeding requiring cardiopulmonary bypass and surgical valve placement. The median follow-up time was 28.7 months. All patients had a significant decrease in CVP and were alive with improvement in their NYHA class to I at most recent follow-up. There was no evidence of valve dysfunction seen on follow up ECHO or cross-sectional imaging. Conclusion:Bjork Fontan patients often have connections too large for available balloon expandable valves. These patients can be treated with a hybrid incomplete annuloplasty ring placement to enable transcatheter valve placement.
The Fontan procedure is a palliative operation for patients with univentricular physiology. Liver complications post-surgery are inevitable and include liver cirrhosis and hepatocellular carcinoma (HCC). Routine surveillance includes transient elastography (TE) and clinical assessment, however longitudinal data on Fontan-associated liver disease (FALD) is scarce. This study aimed to describe the evolution of FALD and assess the correlation between liver stiffness measurement (LSM) and hepatic decompensation. This retrospective case series comprised all adult post-Fontan patients presenting to a tertiary hospital between January 2015 and December 2024. Demographic and clinical information, serum tests, TE and echocardiograms were extracted from medical records. Endpoints assessed were decompensated cirrhosis and findings that warrant HCC multi-disciplinary team review. A derived surrogate outcome, clinically significant portal hypertension (CSPH) was assessed using the Baveno VII criteria. There were a total of 65 patients with 111 Fibroscans™. There was no significant change in LSM by TE when comparing two consecutive decade years post Fontan surgery. On echocardiography, only inferior vena-cava diameter and degree of ventricular function correlated with LSM scores (p = 0.032, p < 0.001). Twelve patients had CSPH, correlating with increased gamma-glutamyltransferase, bilirubin, international normalised ratio and aspartate aminotransferase levels (p = 0.047, 0.034, 0.012, 0.049 respectively). Four patients exhibited features of decompensated cirrhosis, eight warranted multi-disciplinary team discussion however these did not correlate with LSM or steatosis results. LSM and echocardiogram monitoring alone is insufficient for identifying FALD patients at risk of hepatic decompensation. Regular testing and multicenter research is required to develop predictive models that accurately identifies these at-risk patients.
