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The Fontan procedure results in chronic hepatic congestion and Fontan-associated liver disease (FALD) characterized by progressive liver fibrosis and cirrhosis. Exercise is recommended in this population, but may accelerate the progression of FALD from abrupt elevations in central venous pressure. The aim of this study was to assess if acute liver injury occurs after high-intensity exercise in patients with Fontan physiology. Ten patients were enrolled. Nine had normal systolic ventricular function and one had an ejection fraction < 40%. During cardiopulmonary exercise testing, patients had near-infrared spectroscopy (NIRS) to measure oxygen saturation of multiple organs, including the liver, and underwent pre- and post-exercise testing with liver elastography, laboratory markers, and cytokines to assess liver injury. The hepatic and renal NIRS showed a statistically significant decrease in oxygenation during exercise, and the hepatic NIRS had the slowest recovery compared to renal, cerebral, and peripheral muscle NIRS. A clinically significant increase in shear wave velocity occurred after exercise testing only in the one patient with systolic dysfunction. There was a statistically significant, albeit trivial, increase in ALT and GGT after exercise. Fibrogenic cytokines traditionally associated with FALD did not increase significantly in our cohort; however, pro-inflammatory cytokines that predispose to fibrogenesis did significantly rise during exercise. Although patients with Fontan circulation demonstrated a significant reduction in hepatic tissue oxygenation based on NIRS saturations during exercise, there was no clinical evidence of acute increase in liver congestion or acute liver injury following high-intensity exercise., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Utilization of Fontan fenestration varies considerably by center., Objectives: Using a multicenter Pediatric Heart Network dataset linking surgical and preoperative hemodynamic variables, the authors evaluated factors associated with use of Fontan fenestration and the impact of fenestration on post-Fontan length of stay (LOS)., Methods: Patients 2 to 6 years old at Fontan surgery from 2010 to 2020 with catheterization<1 year prior were included. Factors associated with fenestration were evaluated using multivariable logistic regression adjusting for key covariates. Restrictive cubic spline analysis was used to evaluate potential cut-points for hemodynamic variables associated with longer postoperative LOS stratified by fenestration with multivariable linear regression to evaluate the magnitude of effect., Results: Fenestration was used in 465 of 702 patients (66.2%). Placement of a fenestration was associated with center (range 27%-93% use, P < 0.0001) and Fontan type (OR: 14.1 for lateral tunnel vs extracardiac conduit, P < 0.0001). No hemodynamic variable was independently associated with fenestration. In a multivariable linear model adjusting for center, a center-fenestration interaction, prematurity, preoperative mean pulmonary artery pressure (mPAP), and cardiac index, fenestration was associated with shorter hospital LOS after Fontan ( P = 0.0024). The benefit was most pronounced at mPAP ≥13 mm Hg (median LOS: 9 vs 12 days, P = 0.001)., Conclusions: There is wide center variability in use of Fontan fenestration that is not explained by preoperative hemodynamics. Fenestration is independently associated with shorter LOS, and those with mPAP ≥13 mm Hg at pre-Fontan catheterization benefit the most. We propose this threshold as minimal criteria for fenestration.

The Pediatric Heart Network's Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT02741115) demonstrated improvements in some measures of exercise capacity and in the myocardial performance index following 6 months of treatment with udenafil (87.5 mg twice daily). In this post hoc analysis, we evaluate whether subgroups within the population experienced a differential effect on exercise performance in response to treatment. The effect of udenafil on exercise was evaluated within subgroups defined by baseline characteristics, including peak oxygen consumption (VO 2 ), serum brain-type natriuretic peptide level, weight, race, gender, and ventricular morphology. Differences among subgroups were evaluated using ANCOVA modeling with fixed factors for treatment arm and subgroup and the interaction between treatment arm and subgroup. Within-subgroup analyses demonstrated trends toward quantitative improvements in peak VO 2 , work rate at the ventilatory anaerobic threshold (VAT), VO 2 at VAT, and ventilatory efficiency (VE/VCO 2 ) for those randomized to udenafil compared to placebo in nearly all subgroups. There was no identified differential response to udenafil based on baseline peak VO 2 , baseline BNP level, weight, race and ethnicity, gender, or ventricular morphology, although participants in the lowest tertile of baseline peak VO 2 trended toward larger improvements. The absence of a differential response across subgroups in response to treatment with udenafil suggests that the treatment benefit may not be restricted to specific sub-populations. Further work is warranted to confirm the potential benefit of udenafil and to evaluate the long-term tolerability and safety of treatment and to determine the impact of udenafil on the development of other morbidities related to the Fontan circulation.Trial Registration NCT0274115., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background and Aims: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear., Methods: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death., Results: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome., Conclusions: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background: Patients with single ventricle CHD have significant morbidity and healthcare utilisation throughout their lifetime, including non-cardiac hospital admissions. Respiratory viral infections are the main cause of hospitalisation in children, but few data exist for single ventricle patients. We sought to identify how respiratory viral infections impact patients with single ventricle CHD and potential differences between Glenn and Fontan circulation., Methods: We conducted a retrospective study of patients seen from 01/01/2011-12/31/2020. We identified patients with a history of single ventricle CHD and Glenn palliation, and a normoxic control group with isolated atrial septal defect requiring surgical closure. We compared viral-related clinical presentations, admissions, and admission characteristics., Results: A total of 312 patients were included (182 single ventricle, 130 atrial septal defect). Single ventricle patients were more likely than children with isolated atrial septal defect to be admitted with a respiratory virus (odds ratio 4.15 [2.30-7.46]), but there was no difference in mechanical ventilation or hospital length of stay (p = 0.4709). Single ventricle patients with Glenn circulation were more likely than those with Fontan circulation to present and be admitted (odds ratio 3.25 [1.62-6.52]), but there was no difference in ICU admission, mechanical ventilation, or hospital length of stay (p = 0.1516)., Conclusions: Respiratory viral infections are prevalent but uncomplicated in patients with single ventricle CHD. Viral-related presentations and admissions are more prevalent during the period of Glenn circulation compared to Fontan circulation; however, rate of mechanical ventilation and hospital length of stay are similar.

Background: The supported Ross is used to mitigate the neoaortic root dilation that has been described with the unsupported Ross. There is limited literature assessing the efficacy of the supported Ross in young patients. In this study, the fate of the neoaortic root was compared in the supported and unsupported Ross procedure in adolescent patients., Methods: A retrospective review was performed of patients who underwent the Ross procedure between 1996 and 2019. An analysis was conducted of patients aged 10 to 18 years who underwent the supported and unsupported Ross operation, without a Konno enlargement, to assess for longitudinal echocardiographic changes. Given differences in follow-up time, both regression analysis and Mann-Whitney nonparametric tests were used to correct for time from discharge to most recent follow-up., Results: The median follow-up time for supported and unsupported Ross patients without a Konno enlargement was 2.90 years (0.21-13.03 years) and 12.13 years (2.63-19.47 years), respectively. Unsupported Ross patients experienced a higher rate of change per year in the aortic annulus (P = .003 and P = .014) and aortic sinus (P = .002 and P = .002) diameters, respectively. There was no significant difference in the rate of change of end-diastolic left ventricular internal diameter (P = .703 and P = .92) and aortic insufficiency (P = .687 and P = .215) between the supported and unsupported Ross patients., Conclusions: Progressive dilation of the neoaortic root in unsupported Ross patients is significantly mitigated with the supported Ross with excellent stability. The supported Ross is safe and effective and may play an increasing role in the management of children with aortic disease., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Aortopulmonary collaterals (APCs) develop universally, but to varying degrees, in patients with single ventricle congenital heart disease (CHD). Despite their ubiquitous presence, APCs remain poorly understood. We sought to evaluate the association between APC burden and common non-invasive clinical variables. We conducted a single center, retrospective study of patients with single ventricle CHD and previous Glenn palliation who underwent pre-Fontan cardiac magnetic resonance (CMR) imaging from 3/2018 to 3/2021. CMR was used to quantify APC flow, which was normalized to aortic (APC/Q Ao ) and pulmonary vein (APC/Q PV ) blood flow. Univariate, multivariable, and classification and regression tree (CART) analyses were done to investigate the potential relationship between CMR-quantified APC burden and clinical variables. A total of 29 patients were included, all of whom had increased APC flow (APC/Q Ao : 26.9, [22.0, 39.1]%; APC/Q PV : 39.4 [33.3, 46.9]%), but to varying degrees (APC/Q Ao : range 11.9-44.4%; APC/Q PV : range 17.7-60.0%). Pulmonary artery size (Nakata index, at pre-Fontan CMR) was the only variable associated with APC flow on multivariable analysis (APC/Q Ao : p = 0.020, R 2  = 0.19; APC/Q PV : p = 0.0006, R 2  = 0.36) and was the most important variable associated with APC burden identified by CART analysis (size inversely related to APC flow). APC flow is universally increased but highly variable in patients with single ventricle CHD and Glenn circulation. Small branch pulmonary artery size is a key factor associated with increased APC burden; however, the pathogenesis of APCs is likely multifactorial. Further research is needed to better understand APC pathogenesis, including predisposing and mitigating factors., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Children and adults with single ventricle congenital heart disease (CHD) develop many sequelae during staged surgical palliation. Universal pulmonary vascular sequelae in this patient population include two inter-related but distinct complications: pulmonary arteriovenous malformations (PAVMs) and aortopulmonary collaterals (APCs). This review highlights what is known and unknown about these vascular sequelae focusing on diagnostic testing, pathophysiology, and areas in need of further research.

Background: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes., Objectives: The authors aimed to determine factors associated with survival in a large cohort of such individuals., Methods: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS)., Results: From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography., Conclusions: For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk., Competing Interests: Funding Support and Author Disclosures This study was funded by a joint grant from the Children’s Heart Foundation and the American Heart Association (17GRNT33670334). Dr Opotowsky was supported by the Heart Institute Research Core at Cincinnati Children’s Hospital and the Dunlevie Family Fund. The authors used the Research Electronic Data Capture (REDCap) online database, which is funded by the National Institutes of Health (UL1TR002369). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Introduction: There is a paucity of literature evaluating the impact of and barriers to participation in cardiac rehabilitation (CR) in the adult congenital heart disease population. The aims of this study were to evaluate the impact of CR on physical activity and health-related quality of life, as well as to evaluate the barriers to participation in CR in a post-operative adult congenital heart disease population., Methods: Patients ≥18 yr of age seen in the Wisconsin Adult Congenital Heart Disease Program and post-open sternotomy surgery from 2010-2015 were eligible for inclusion. Subjects were mailed a novel physical activity survey and the validated EuroQOL-5D 3L health questionnaire. A retrospective medical record review was performed to extract demographic and clinical data., Results: One hundred thirty-five patients underwent open sternotomy surgery from 2010-2015. Of these, 22 were excluded because of intellectual disability, three opted out, and three survey packets were returned to the sender. A total of 54 of the remaining 107 patients returned completed surveys. Of these, 47 (87%) were referred to CR. Thirty-five patients completed the entire CR program (74%). Those who completed CR were more likely to develop a home/independent exercise program (P = .027). Barriers to completing CR included insurance coverage, psychiatric disease, and a perception that CR would not be of benefit., Conclusion: Completing CR was associated with developing a home/independent exercise program in post-sternotomy adult patients with congenital heart disease. Barriers to participating in and completing CR in this population could lead to an improved completion rate if modified., Competing Interests: Conflicts of Interest: None., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Pulmonary arteriovenous malformations in single ventricle congenital heart disease are poorly understood. Previous studies investigating pulmonary arteriovenous malformations predominantly focus on patients with heterotaxy syndrome and interrupted inferior caval vein. It is unknown if development and resolution of pulmonary arteriovenous malformations are similar for patients with and without heterotaxy syndrome., Methods: In this retrospective single-institution study, we identified patients with a history of single ventricle congenital heart disease and Fontan palliation. We then matched patients with heterotaxy syndrome (intact and interrupted inferior caval vein) and non-heterotaxy hypoplastic left heart syndrome. To compare development of pulmonary arteriovenous malformations, we identified the frequency of positive diagnoses pre-Fontan. To compare resolution of pulmonary arteriovenous malformations, we recorded oxygen saturation changes for 12 months following Fontan., Results: A total of 124 patients were included. Patients with heterotaxy and interrupted inferior caval vein were more likely to have a pre-Fontan contrast echocardiogram performed (p < 0.01) and more likely to be diagnosed with pulmonary arteriovenous malformations pre-Fontan (p < 0.01). There was no difference in oxygen saturation prior to Fontan, yet all patient groups had increased their oxygen saturations in the first year after Fontan discharge., Conclusions: Pulmonary arteriovenous malformations are variably diagnosed prior to Fontan palliation; however, all study groups had increased oxygen saturations after Fontan discharge, potentially indicating resolution of pulmonary arteriovenous malformations in all groups. The prevalence of pulmonary arteriovenous malformations pre-Fontan is likely underestimated. A quantitative, systematic approach to diagnosis and follow-up of pulmonary arteriovenous malformations is needed to better understand susceptibility and pathophysiology.

Aims: The Fontan operation has resulted in improved survival in patients with single-ventricle congenital heart disease. As a result, there is a growing population of teenagers and adults with a Fontan circulation. Many co-morbidities have been increasingly recognized in this population due to the unique features of the Fontan circulation. Standardization of how Fontan co-morbid conditions are defined will help facilitate understanding, consistency and interpretability of research and clinical experience. Unifying common language usage in Fontan is a critical precursor step for data comparison of research findings and clinical outcomes and ultimately accelerating improvements in management for this growing group of patients. This manuscript aimed to create unified definitions for morbidities seen after the Fontan palliation., Methods: In association of many congenital heart disease organizations, this work used Delphi methodology to reach a broad consensus among recognized experts regarding commonly used terms in Fontan care and research. Each definition underwent at least three rounds of revisions to reach a final definition through surveys sent to experts in the field of single-ventricle care., Results: The process of reaching a consensus on multiple morbidities associated with the Fontan procedure is summarized in this manuscript. The different versions that preceded reaching the consensus are also presented in the Supporting Information. Table 1 represents the final definitions according to the consensus., Conclusions: We propose the use of these definitions for clinical care, future research studies, registry development and clinical trials., (© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Background: The impact of aortic valve replacement (AVR) type on health-related quality of life (HRQOL) in adolescents and young adults is unclear, but may vary depending on need for anticoagulation or re-intervention. We sought to determine the differences in HRQOL following AVR with either the Ross procedure or mechanical AVR in this young population., Methods: Patients 14-35 years old and at least 1 year post-AVR were included. HRQOL was assessed using the Short Form-36 (SF-36). Valve-specific concerns regarding anticoagulation and reoperation were also assessed. Clinical outcome data were obtained by chart review., Results: A total of 51 patients were enrolled: 24 (47%) Ross and 27 (53%) mechanical AVR. Ross patients were younger at time of AVR (16 vs. 22 years, p < 0.01) and study enrollment (23.7 vs 29.5 years, p < 0.01). Median follow-up from AVR to study enrollment was similar (5.4 years for Ross vs. 5.6 years for mechanical, p = 0.62). At last follow-up, clinical outcomes including cardiac function, functional class, and aortic valve re-intervention rates were similar between groups, although mechanical valve patients had more bleeding events (p = 0.012). SF-36 scores were generally high for the entire cohort, with no significant difference between groups in any domain. Mechanical AVR patients reported more concern about frequency of blood draws (p < 0.01). Concern for reoperation was similar between both groups., Conclusion: Despite more bleeding events and concern about the frequency of blood draws, adolescents and young adults with mechanical AVR reported similarly high levels of HRQOL compared to those following Ross AVR.

Introduction: Cardiac rehabilitation programmes for paediatric patients with congenital heart disease (CHD) have been shown to promote emotional and physical health without any associated adverse events. While prior studies have demonstrated the effectiveness of these types of interventions, there has been limited research into how the inclusion of psychological interventions as part of the programme impacts parent-reported and patient-reported quality of life., Materials and Methods: Patients between the ages of 7 and 24 years with CHD completed a cardiac rehabilitation programme that followed a flexible structure of four in person-visits with various multidisciplinary team members, including paediatric psychologists. Changes in scores from the earliest to the latest session were assessed regarding exercise capacity, patient functioning (social, emotional, school, psychosocial), patient general and cardiac-related quality of life, patient self-concept, and patient behavioural/emotional problems., Results: From their baseline to final session, patients exhibited significant improvement in exercise capacity (p = 0.00009). Parents reported improvement in the patient's emotional functioning, social functioning, school functioning, psychosocial functioning, cognitive functioning, communication, and overall quality of life. While patients did not report improvement in these above areas, they did report perceived improvement in certain aspects of cardiac-related quality of life and self-concept., Discussion: This paediatric cardiac rehabilitation programme, which included regular consultations with paediatric psychologists, was associated with divergent perceptions by parents and patients on improvement related to quality of life and other aspects of functioning despite improvement in exercise capacity. Further investigation is recommended to identify underlying factors associated with the differing perceptions of parents and patients.

Background: Total cavopulmonary connection (TCPC) is associated with a lower risk of incident atrial arrhythmias as compared to atriopulmonary Fontan, but the risk of recurrent atrial arrhythmias is unknown in this population. The purpose of this study was to determine the incidence and risk factors for recurrent atrial arrhythmias and thromboembolic complications in patients with TCPC., Methods: This is a retrospective multicenter study conducted by the Alliance for Adult Research in Congenital Cardiology (AARCC), 2000-2018. The inclusion criteria were TCPC patients (age > 15 years) with prior history of atrial arrhythmia., Results: A total of 103 patients (age 26 ± 7 years; male 58 [56%]) met inclusion criteria. The mean age at initial arrhythmia diagnosis was 13 ± 5 years, and atrial arrhythmias were classified as atrial flutter/tachycardia in 85 (83%) and atrial fibrillation in 18 (17%). The median duration of follow-up from the first episode of atrial arrhythmia was 14.9 (12.1-17.3) years, and during this period 64 (62%) patients had recurrent atrial arrhythmias (atrial flutter/tachycardia 51 [80%] and atrial fibrillation 13 [20%]) with annual incidence of 4.4%. Older age was a risk factor for arrhythmia recurrence while the use of a class III anti-arrhythmic drug was associated with a lower risk of recurrent arrhythmias. The incidence of thromboembolic complication was 0.6% per year, and the cumulative incidence was 4% and 7% at 5 and 10 years respectively from the time of first atrial arrhythmia diagnosis. There were no identifiable risk factors for thromboembolic complications in this cohort., Conclusions: Although TCPC provides superior flow dynamics and lower risk of incident atrial arrhythmias, there is a significant risk of recurrent arrhythmias among TCPC patients with a prior history of atrial arrhythmias. These patients may require more intensive arrhythmia surveillance as compared to other TCPC patients., (© 2021 The Author(s).)

Background: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation., Methods: A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described., Results: Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months., Conclusions: HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage., (Crown Copyright © 2020. Published by Elsevier B.V. All rights reserved.)

Individuals with single ventricle congenital heart disease (CHD) undergo multiple staged surgical palliations. Staged single ventricle palliation with a superior cavopulmonary connection (SCPC) in infancy followed by a Fontan in early childhood relies on passive, unobstructed pulmonary blood flow and normal pulmonary vasculature. We hypothesized that patients with echocardiographic identification of retrograde flow in a branch pulmonary artery (PA) after SCPC or Fontan are at increased risk for adverse outcomes. We conducted a retrospective chart review of patients seen at Children's Wisconsin from 1999 to 2019. Inclusion criteria included a history of single ventricle congenital heart disease and surgical palliation with a superior cavopulmonary connection (SCPC). We created two cohorts based on transthoracic echocardiographic identification of branch PA flow patterns: those with color Doppler-defined pulmonary artery flow reversal (PA reversal cohort) and those with normal anterograde flow (Non-reversal cohort). We identified 21 patients in the PA reversal cohort and 539 patients in the Non-reversal cohort. The PA reversal cohort had increased hospital length of stay after SCPC palliation (p < 0.001) and decreased transplant-free survival (p = 0.032), but there was no difference in overall survival (p = 0.099). There was no difference in hospital length of stay after Fontan (p = 0.17); however, the PA reversal cohort was significantly less likely to progress to Fontan palliation during early childhood (p = 0.005). Echocardiographic color Doppler identification of branch PA flow reversal in patients with single ventricle physiology is a high-risk indicator for adverse short- and long-term outcomes.

The Ross procedure in children, teenagers, and young adults, especially among those with a bicuspid aortic valve with aortic regurgitation, has been associated with neoaortic root dilatation and recurrence of aortic regurgitation. We have shown that at intermediate follow-up, patients who underwent a supported Ross technique were less likely to have neoaortic root dilatation. This study summarizes our most recent outcomes. This was a retrospective review of 40 consecutive pediatric and young adult patients undergoing a supported Ross procedure from 2005 to 2018. Clinical outcomes were reviewed in addition to echocardiographic measures of neoaortic dimension and neoaortic valve function. The median age at surgery was 16.0 years (range 10 - 35 years). Preoperative diagnosis was aortic regurgitation in 15 (37.5%) and mixed regurgitation/stenosis in 20 (50%). Median follow-up was 3.5 years (1.4-5.6) with 3 patients followed for more than 10 years. There were no deaths. Five patients had a reintervention, but only 1 on the aortic valve. One patient returned to the operating room on postoperative day 1 for revision of the right coronary button. Two patients required biventricular pacemakers for reduced ejection fraction. One patient developed aortic regurgitation and underwent mechanical valve replacement and another required a reintervention on the homograft with a percutaneously placed pulmonary valve replacement. At last follow-up, 39 patients had mild or less aortic regurgitation with median sinus z-score of 1.40 (0.48-2.07). Mid-term follow-up of pediatric and young adult patients undergoing a supported Ross operation for various aortic valve pathologies demonstrate excellent results with minimal neoaortic root dilation and reintervention., (Copyright © 2019 Elsevier Inc. All rights reserved.)

The objective of our study was to determine the prevalence, risk factors, and the impact of obstructive sleep apnea (OSA) in the adult with congenital heart disease (ACHD). One hundred forty-nine consecutive patients seen in our ACHD program were screened for OSA using the Berlin Questionnaire. Demographic and clinical details on subjects were collected through a chart review. Clinical variables were analyzed to determine risk factors for positive OSA screen, as well as associated outcomes. Seventy-seven (52%) of our cohort were females. The median age of the cohort was 33 years (range = 18-74) and median weight was 79 kg (range = 50-145 kg). Overall, 47 (31%) of our cohort were found to have a positive OSA screen using the Berlin questionnaire. Median age of the patients whom tested positive was 34 years. Compared to patients with a negative screen, patients with a positive OSA screen were more likely to be heavier with a median weight of 99 kg vs 71 kg (p < 0.01) and a larger BMI (31 vs 25 kg/m 2 , p < 0.01). Overall, 55% of patients whom screened positive were obese (defined as a BMI > 30) compared to 15% in the negative group (p < 0.02). Patients with a positive screen were more likely to have other co-morbidities including diabetes (p < 0.04), hypertension (p < 0.05), depression (p < 0.002), and were more likely to have decreased exercise capacity (p < 0.01) and a defibrillator (p < 0.007). Our data demonstrates that OSA is common in the ACHD patient and is associated with increasing weight and BMI. Patients with a positive screen are at increased risk for multiple co morbidities including diabetes, hypertension, and depression. We believe our data supports the use of screening protocols for OSA in the ACHD population in effort to identify early, treat, and potentially prevent late complications.

Background: The Fontan operation creates a total cavopulmonary connection, a circulation in which the importance of pulmonary vascular resistance is magnified. Over time, this circulation leads to deterioration of cardiovascular efficiency associated with a decline in exercise performance. Rigorous clinical trials aimed at improving physiology and guiding pharmacotherapy are lacking., Methods: The FUEL trial (Fontan Udenafil Exercise Longitudinal) was a phase III clinical trial conducted at 30 centers. Participants were randomly assigned udenafil, 87.5 mg twice daily, or placebo in a 1:1 ratio. The primary outcome was the between-group difference in change in oxygen consumption at peak exercise. Secondary outcomes included between-group differences in changes in submaximal exercise at the ventilatory anaerobic threshold, the myocardial performance index, the natural log of the reactive hyperemia index, and serum brain-type natriuretic peptide., Results: Between 2017 and 2019, 30 clinical sites in North America and the Republic of Korea randomly assigned 400 participants with Fontan physiology. The mean age at randomization was 15.5±2 years; 60% of participants were male, and 81% were white. All 400 participants were included in the primary analysis with imputation of the 26-week end point for 21 participants with missing data (11 randomly assigned to udenafil and 10 to placebo). Among randomly assigned participants, peak oxygen consumption increased by 44±245 mL/min (2.8%) in the udenafil group and declined by 3.7±228 mL/min (-0.2%) in the placebo group ( P =0.071). Analysis at ventilatory anaerobic threshold demonstrated improvements in the udenafil group versus the placebo group in oxygen consumption (+33±185 [3.2%] versus -9±193 [-0.9%] mL/min, P =0.012), ventilatory equivalents of carbon dioxide (-0.8 versus -0.06, P =0.014), and work rate (+3.8 versus +0.34 W, P =0.021). There was no difference in change of myocardial performance index, the natural log of the reactive hyperemia index, or serum brain-type natriuretic peptide level., Conclusions: In the FUEL trial, treatment with udenafil (87.5 mg twice daily) was not associated with an improvement in oxygen consumption at peak exercise but was associated with improvements in multiple measures of exercise performance at the ventilatory anaerobic threshold., Clinical Trial Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT02741115.

After the Fontan, systemic venous hypertension induces pathophysiologic changes in the lymphatic system that can result in complications of pleural effusion, ascites, plastic bronchitis, and protein losing enteropathy. Advances in medical therapy and novel interventional approaches have not substantially improved the poor prognosis of these complications. A more physiological approach has been developed by decompression of the thoracic duct to the lower pressure common atrium with a concomitant increase of preload. Diverting the innominate vein to the common atrium increases the transport capacity of the thoracic duct, which in most patients enters the circulation at the left subclavian-jugular vein junction. Contrary to the fenestrated Fontan circulation, in which the thoracic duct is drained into the high pressure Fontan circulation, turn down of the innominate vein to the common atrium effectively decompresses the thoracic duct to the lower pressure system with "diastolic suctioning" of lymph. Innominate vein turn-down may be considered for medical-refractory post-Fontan lymphatic complications of persistent chylothorax, plastic bronchitis, and protein losing enteropathy. Prophylactic innominate vein turn-down may also be considered at time of the Fontan operation for patients that are higher risk for lymphatic complications., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: As adults with congenital heart disease (CHD) grow older, preoperative screening for coronary artery disease (CAD) may be indicated prior to CHD surgery. Data regarding the indications for preoperative CAD screening in this population are limited. Current practice is to follow guidelines for patients with valvular heart disease; however, the risk for CAD in certain congenital heart diagnoses may be higher than the general population. This study aimed to assess the results of preoperative CAD screening in patients prior to CHD surgery., Design: Retrospective study., Setting: Single tertiary center., Patients: Patients ≥35 years that had CHD surgery from 1/1/2007 to 5/1/2017., Outcome Measures: Data regarding CAD risk factors and preoperative CAD screening results were obtained. Prevalence and risk factors for CAD were analyzed, along with their relationship to perioperative outcomes., Results: A total of 73 patients underwent CAD screening with either cardiac catheterization (56%) or computed tomography angiography (34%) prior to CHD surgery. Overall 16 (22%) patients were found to have CAD. Only two patients had severe coronary stenosis and underwent coronary bypass grafting at time of CHD surgery. Patients with CAD were more likely to be older and have history of hypertension, dyslipidemia, and tobacco smoking. CHD diagnosis was not significantly associated with presence of CAD., Conclusion: CAD is common in asymptomatic older patients referred for screening prior to CHD surgery; however, severe CAD requiring concomitant coronary intervention is uncommon. Preoperative CAD screening should be based on age and traditional CAD risk factors, rather than underlying CHD., (© 2019 Wiley Periodicals, Inc.)

Background: Hypoxia is a common and sometimes severe morbidity of single ventricle congenital heart disease (CHD). Creation of an arteriovenous fistula (AVF) is occasionally performed for patients after superior or total cavopulmonary connection (SCPC or TCPC) in an attempt to improve oxygen saturations. Despite previous reports, AVF creation is a rare palliation with inadequately defined benefits and risks. We sought to determine changes in peripheral oxygen saturation (SpO 2 ) and risk of adverse event after AVF creation in children with single ventricle CHD at our institution., Methods: We conducted a retrospective chart review of patients with a history of single ventricle palliation and history of surgical AVF creation who were seen at our tertiary care center from 1996 to 2017., Results: A total of seven patients were included in our study. SpO 2 for the overall cohort did not significantly increase after AVF creation (pre-AVF 79.1 ± 6.9%, post-AVF 82.7 ± 6.0% [P = .23]). SpO 2 trended up for large shunts (>5 mm) (pre-AVF 75.0 ± 7.6%, post-AVF 84.0 ± 5.3% [P = .25]). SpO 2 did not improve for small shunts (≤5 mm) (pre-AVF 82.3 ± 6.5%, post-AVF 81.0 ± 8.5% [P = .50]). The 12-month overall and transplant-free survival were 85.7% and 71.4%, respectively. Freedom from AVF-related complication (cephalic edema, thrombotic occlusion) was 51.4% at 12 months., Conclusion: Palliative AVF creation for patients with single ventricle CHD and hypoxia does not universally improve SpO 2 and is prone to early complications. Despite a lack of durable benefit and known risks, AVF creation remains a reasonable palliation for a subset of patients after SCPC who are not candidates for TCPC, or potentially as a bridge to heart transplantation., (© 2019 Wiley Periodicals, Inc.)

Background/hypothesis/objectives: Postoperative complications after the Fontan operation for single ventricle heart disease are common and include persistent pleural drainage and prolonged length of hospital stay (LOS). Diastolic ventricular dysfunction may increase risk for postoperative complications by raising central venous pressures. We sought to determine the relationship between preoperative echocardiographic measurements of diastolic function, including myocardial deformation imaging, on (a) preoperative invasive catheterization measurements and (b) postoperative outcomes after the Fontan procedure., Design/methods: All patients that underwent Fontan procedure from 2011 to 2017 were included. Echocardiograms performed within 6 months prior to Fontan operation were evaluated. Measurements of ventricular global and diastolic strain and strain rate were performed offline with TomTec speckle tracking software. Other diastolic function measurements included atrioventricular valve inflow and annular tissue Doppler imaging. Diastolic function measurements were correlated with pre-Fontan catheterization measurements and postoperative Fontan outcomes using Spearman's rho. Multivariable logistic regression for a prolonged LOS (>75%ile for postoperative LOS) was performed to adjust for preoperative risk factors., Results: A total of 141 patients were included in the study. Majority had single morphologic right ventricle (58.9%). Median age at time of Fontan was 3.4 years (IQR 2.9-4). Median hospital LOS was 9 days (IQR 7-11). Circumferential diastolic strain rate weakly correlated with LOS (rho = -0.21, P = .01). There was no correlation between any other diastolic strain measurements and pre-Fontan end-diastolic pressure or postoperative LOS. In multivariable analysis, E/E' was the only echo measurement that predicted prolonged hospital LOS (OR 1.4, 95%CI: 1.1-1.8, P = .003)., Conclusion: Preoperative diastolic strain measurements did not have a strong association with postoperative Fontan outcomes. Increased E/E' ratio, however, did predict greater LOS after Fontan procedure, and may be useful in preoperative risk stratification. Future studies are needed to further assess the utility of diastolic strain imaging in the single-ventricle population., (© 2019 Wiley Periodicals, Inc.)

More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan care programs performing a combined estimated 300 Fontan surgeries per year, with a total population of 1500-2000 Fontan patients, were surveyed using a REDCap survey regarding surveillance testing and basic practice philosophies. Fontan care programs were structured both as consultative services (64%) and as the primary clinical team (9%). Electrocardiograms (73%) and echocardiograms (64%) were most commonly obtained annually. Serum studies, including complete blood count (73%), complete metabolic panel (73%), and Brain-type natriuretic peptide (54%), were most commonly obtained annually. Hepatic testing consisted of liver ultrasound in most centers, obtained biennially (45%) or > every 2 years (45%). Liver biopsy was not routinely recommended (54%). Neurodevelopmental outcomes were assessed at most institutions (54%), with a median frequency of every 3-4 years. There is considerable variability in the surveillance testing regimen and management strategy after a Fontan procedure at surveyed programs. There is an urgent need for surveillance guidelines to reduce variability, define quality metrics, streamline collaborative practice, and prospective research to better understand the complex adaptations of the body to Fontan physiology.

Adult congenital heart disease (ACHD) patients often require repeat cardiothoracic surgery, which may result in significant morbidity and mortality. Currently, there are few pre-operative risk assessment tools available. In the general adult population, pre-operative cardiopulmonary exercise testing (CPET) has a predictive value for post-operative morbidity and mortality following major non-cardiac surgery. The utility of CPET for risk assessment in ACHD patients requiring cardiothoracic surgery has not been evaluated. Retrospective chart review was conducted on 75 ACHD patients who underwent CPET less than 12 months prior to major cardiothoracic surgery at Children's Hospital of Wisconsin. Minimally invasive procedures, cardiomyopathy, acquired heart disease, single ventricle physiology, and heart transplant patients were excluded. Demographic information, CPET results, and peri-operative surgical data were collected. The study population was 56% male with a median age of 25 years (17-58). Prolonged post-operative length of stay correlated with increased ventilatory efficiency slope (V E /[Formula: see text] slope) (P = 0.007). Prolonged intubation time correlated with decreased peak HR (P = 0.008), decreased exercise time (P = 0.002), decreased heart rate response (P = 0.008) and decreased relative peak oxygen consumption (P = 0.034). Post-operative complications were documented in 59% of patients. While trends were noted between post-operative complications and some measurements of exercise capacity, none met statistical significance. Future studies may further define the relationship between exercise capacity and post-operative morbidity in ACHD patients.

It is well-recognized now that adult survivors with congenital heart disease (CHD) are at risk for non-cardiac co-morbidities and complications that can impact symptoms and clinical outcomes. Lung disease, in particular, is common in this population, but likely an under-recognized and undertreated cause for long-term morbidity. Abnormal lung function contributes to exercise intolerance and is associated with a higher risk for mortality in this population. The exact mechanisms that contribute to abnormal measurements of lung function are not entirely known, and are likely multifactorial and variable depending on the underlying CHD. Nevertheless, lung disease is a potentially modifiable risk factor in this patient population, the management of which may result in improved clinical outcomes. This review summarizes our current understanding of the prevalence, impact and management of lung disease in adults with CHD., (Copyright © 2018 Elsevier Inc. All rights reserved.)