Sex Differences in Outcomes of Adults With Isolated Coarctation of the Aorta.

Background: Data are limited about the effect (or lack thereof) of sex on clinical outcomes in adults with coarctation of the aorta (COA). The purpose of this study was to compare atherosclerotic cardiovascular disease (ASCVD) risk profile, blood pressure (BP) data, echocardiographic indices, and mortality between men and women with COA. Methods: Retrospective study of adults with COA, and no associated left-sided obstructive lesions, who received care at Mayo Clinic (2003-2022). ASCVD risk profile was assessed as the prevalence of hypertension, hyperlipidemia, type 2 diabetes, obesity, smoking history, and coronary artery disease. A 24-hour BP monitor was used to assess daytime and nighttime BP and calculate nocturnal dipping. Results: Of 621 patients with isolated COA, 375 (60%) were men, and 246 (40%) were women. Women had similar ASCVD risk profile and daytime BP as men. However, women had less nocturnal dipping (7 ± 5 mm Hg vs 16 ± 7 mm Hg, P < 0.001), higher pulmonary artery mean pressure (23 mm Hg [interquartile range: 16-31] vs 20 mm Hg [interquartile range: 15-28], P = 0.04), and higher pulmonary vascular resistance index (3.41 ± 1.14 WU · m2 vs 3.02 ± 0.76 WU · m2, P = 0.006). Female sex was associated with all-cause mortality (adjusted hazard ratio 1.26, 95% confidence interval 1.04-1.94) and cardiovascular mortality (adjusted hazard ratio 1.38, 95% confidence interval 1.09-2.18). Conclusions: Women had a higher risk of both cardiovascular mortality and all-cause mortality compared to the risks in men. This difference may be related to the higher-than-expected ASCVD risk factors, abnormal nocturnal blood pressure, and pulmonary hypertension observed in women in this cohort. Further studies are required to identify optimal measures to address these risk factors.

Contexte: Il existe peu de données sur l'issue clinique en fonction du sexe chez les adultes présentant une coarctation de l'aorte (CoA). Le but de cette étude consistait donc à comparer le profil de risque de maladie cardiovasculaire athéroscléreuse (MCVAS), les données relatives à la pression artérielle (PA), les indices échocardiographiques et le taux de mortalité chez des hommes et des femmes présentant une CoA. Méthodologie: Il s'agissait d'une étude rétrospective réalisée chez des adultes présentant une CoA en l'absence de lésions obstructives gauches, soignés à la clinique Mayo entre 2003 et 2022. Le profil de risque de MCVAS a été évalué en fonction de la prévalence de l'hypertension, de l'hyperlipidémie, du diabète de type 2, de l'obésité, des antécédents tabagiques et de la coronaropathie. Une surveillance sur 24 heures a été utilisée pour évaluer la PA diurne et nocturne, en plus de calculer la chute nocturne de la PA. Résultats: Parmi les 621 patients présentant une CoA isolée, 375 (60 %) étaient des hommes et 246 (40 %) étaient des femmes. Les femmes présentaient une PA diurne et un profil de risque de MCVAS semblables aux hommes. Elles présentaient néanmoins une chute nocturne de la PA moins prononcée (7 ± 5 mmHg vs 16 ± 7 mmHg, p < 0,001), une pression artérielle pulmonaire moyenne plus haute (23 mmHg [max.-min. : 16-31] vs 20 mmHg [max.-min. : 15-28], p = 0,04) et un indice de résistance vasculaire pulmonaire plus élevé (3,41 ± 1,14 UW · m2 vs 3,02 ± 0,76 UW · m2, p = 0,006). Le sexe féminin a été associé à un plus fort taux de mortalité toutes causes confondues (rapport de risques ajusté : 1,26; intervalle de confiance à 95 % : 1,04-1,94) et de mortalité cardiovasculaire (rapport de risques ajusté : 1,38; intervalle de confiance à 95 % : 1,09-2,18). Conclusions: Les femmes sont exposées à un risque de mortalité cardiovasculaire et de mortalité toutes causes confondues plus élevé que les hommes. Cette différence pourrait être attribuable au rôle plus important que prévu joué par les facteurs de risque de MCVAS ainsi qu'à la pression artérielle nocturne anormale et à l'hypertension pulmonaire chez les femmes de cette cohorte. D'autres études sont nécessaires pour savoir quels seraient les paramètres optimaux qui permettraient d'évaluer ces facteurs de risque.

Evaluation of right ventricular strain in two separate cohorts with precapillary pulmonary hypertension.

Evaluation for right ventricular (RV) dysfunction is an important part of risk assessment in care of patients with pulmonary hypertension (PH) as it is associated with morbidity and mortality. Echocardiography provides a widely available and acceptable method to assess RV function. RV global longitudinal strain (RVGLS), a measure of longitudinal shortening of RV deep muscle fibers obtained by two-dimensional echocardiography, was previously shown to predict short-term mortality in patients with PH. The purpose of the current study was to assess the performance of RVGLS in predicting 1-year outcomes in PH. We retrospectively identified 83 subjects with precapillary PH and then enrolled 50 consecutive prevalent pulmonary arterial hypertension (PAH) subjects into a prospective validation cohort. Death as well as combined morbidity and mortality events at 1 year were assessed as outcomes. In the retrospective cohort, 84% of patients had PAH and the overall 1-year mortality rate was 16%. Less negative RVGLS was marginally better than tricuspid annular plane systolic excursion (TAPSE) as a predictor for death. However, in the prospective cohort, 1-year mortality was only 2%, and RVGLS was not predictive of death or a combined morbidity and mortality outcome. This study supports that RV strain and TAPSE have similar 1-year outcome predictions but highlights that low TAPSE or less negative RV strain measures are often false-positive in a cohort with low baseline mortality risk. While RV failure is considered the final common pathway for disease progression in PAH, echocardiographic measures of RV function may be less informative of risk in serial follow-up of treated PAH patients.

Transcatheter Mitral Valve Repair in a Tricuspid Atresia Patient With Potts and Glenn Shunts.

Our patient was a 50-year-old woman with tricuspid atresia who had undergone palliation with a Potts shunt to the left pulmonary artery as an infant and a classic Glenn shunt to the right pulmonary artery as a young child. Under general anesthesia, she underwent transcatheter edge-to-edge repair of the mitral valve for severe symptomatic mitral regurgitation. (Level of Difficulty: Advanced.).

Recurrent Coronary Artery Fistulae and a Novel Transforming Growth Factor Beta-3 Mutation.

Loeys-Dietz syndrome (LDS) is a rare connective tissue disease associated with mutations in transforming growth factor (TGF) signaling leading to an increased risk of arterial calcification, aneurysms, and/or dissections. We report a case in which genetics evaluation revealed a rare variant E244K in the TGFB3 gene. The variant leads to the substitution of glutamic acid for lysine, two amino acids with dissimilar properties. Analysis from evolutionary data shows the glutamic acid is maintained across species. The clinical significance of the E244K variant in association with LDS was never previously reported as pathologic. This case report aims to report that the significance of the E244K variant in the TGFB3 gene is found to be pathologic in our case. A search on the Genome Aggregation Database (gnomAD) did not reveal any previously identified individuals with this variant, despite being a well-covered region. ClinVar has a few entries for E244K, where most of them are listed as unknown significance. Bringing together the genotype evidence with our patient's clinical picture, we consider the variant to be pathogenic for this family.

Single-Barrel, Double-Barrel, and Fenestrated Endografts to Facilitate Transcatheter Pulmonary Valve Replacement in Large RVOT.

OBJECTIVES: The aim of this study was to test the hypothesis that narrowing the landing zone using commercially available endografts would enable transcatheter pulmonary valve replacement (TPVR) using commercially available transcatheter heart valves. BACKGROUND: TPVR is challenging in an outsized native or patch-repaired right ventricular outflow tract (RVOT). Downsizing the RVOT for TPVR is currently possible only using investigational devices. In patients ineligible because of excessive RVOT size, TPVR landing zones were created using commercially available endografts. METHODS: Consecutive patients with native or patch-repaired RVOTs and high or prohibitive surgical risk were reviewed, and this report describes the authors' experience with endograft-facilitated TPVR (EF-TPVR) offered to patients ineligible for investigational or commercial devices. All EF-TPVR patients were surgery ineligible, with symptomatic, severe pulmonary insufficiency, enlarged RVOTs, and severe right ventricular (RV) enlargement (>150 ml/m2). TPVR and surgical pulmonary valve replacement (SPVR) were compared in patients with less severe RV enlargement. RESULTS: Fourteen patients had large RVOTs unsuitable for conventional TPVR; 6 patients (1 surgery ineligible) received investigational devices, and 8 otherwise ineligible patients underwent compassionate EF-TPVR (n = 5 with tetralogy of Fallot). Three strategies were applied on the basis of progressively larger RVOT size: single-barrel, in situ fenestrated, and double-barrel endografts as required to anchor 1 (single-barrel and fenestrated) or 2 (double-barrel) transcatheter heart valves. All were technically successful, without procedure-related, 30-day, or in-hospital deaths. Two late complications (stent obstruction and embolization) were treated percutaneously. One patient died of ventricular tachycardia 36 days after EF-TPVR. Compared with 48 SPVRs, RV enlargement was greater, but 30-day and 1-year mortality and readmission were no different. The mean transvalvular pressure gradient was lower after EF-TPVR (3.8 ± 0.8 mm Hg vs. 10.7 ± 4.1 mm Hg; p < 0.001; 30 days). More than mild pulmonary insufficiency was equivalent in both (EF-TPVR 0.0% [n = 0 of 8] vs. SPVR 4.3% [n = 1 of 43]; p = 1.00; 30 days). CONCLUSIONS: EF-TPVR may be an alternative for patients with pulmonic insufficiency and enlarged RVOTs ineligible for other therapies.

PCSK9 inhibition in patients with heart transplantation: A case series.

PCSK9 inhibitors are potent low-density lipoprotein cholesterol-lowering medications. There is a lack of data regarding safety and efficacy of PCSK9 inhibitors in cardiac transplant patients. In this case series, we provide data supporting the low-density lipoprotein-lowering efficacy and short-term safety of PCSK9 inhibitors in three cardiac transplant patients.

Association of Antidepressant Medication Type With the Incidence of Cardiovascular Disease in the ARIC Study.

Background The association of antidepressant medication type with the risk of cardiovascular disease ( CVD ) is unclear. We hypothesized that selective serotonin reuptake inhibitors ( SSRI s) are associated with lower risks of CVD events relative to tricyclics and other non- SSRI antidepressants. Methods and Results We studied 2027 participants from the ARIC (Atherosclerosis Risk in Communities) study (mean age 63±10 years; 29% men; 78% white) treated with antidepressants at some time between 1987 and 2013. Antidepressant usage was confirmed by participants bringing pill bottles to study visits. CVD events in the study sample were identified, including atrial fibrillation, heart failure, myocardial infarction, and ischemic stroke. Hazard ratios were used to compare CVD events adjusted for sociodemographic and clinical risk factors in SSRI s users (47%) versus non- SSRI users. Participants were followed from antidepressant initiation up to 2016 for a median of 13.5 years. We identified 332 atrial fibrillation, 365 heart failure, 174 myocardial infarction and 119 ischemic stroke events. CVD risk was similar for SSRI s and non- SSRI antidepressant users (hazard ratio, 1.10; 95% CI , 0.86-1.41 for atrial fibrillation; hazard ratio, 0.98; 95% CI, 0.77-1.25 for heart failure; hazard ratio, 0.91; 95% CI , 0.64-1.29 for myocardial infarction; and hazard ratio, 1.07; 95% CI , 0.70-1.63 for ischemic stroke). Conclusions SSRI use was not associated with reduced risk of incident CVD compared with non- SSRI antidepressant use. These results do not provide evidence supporting the use of SSRI s compared with tricyclics and other non- SSRI antidepressants in relation to CVD risk.

Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary.

The initial "Frontiers in Fontan Failure" conference in 2015 in Atlanta, GA, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with "Failing Fontan" physiology. Four types of "Fontan Failure" were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan procedure "imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation." The purpose of the second "Frontiers in Fontan Failure" was to further the discussion regarding new data and technologies as well as novel interventions. The 2017 "Frontiers in Fontan Failure: Innovation and Improving Outcomes" was sponsored by Children's Healthcare of Atlanta, Sibley Heart Center Cardiology, and Emory University School of Medicine. Future directions in the management of Fontan failure include further investigations into the risk of sudden cardiac death and how to properly prevent it, achievable interventions in modifying the Fontan physiology to treat or prevent late complications, and improved and refined algorithms in Fontan surveillance. Finally, further research into the interventional treatment of lymphatic-related complications hold the promise of marked improvement in the quality of life of advanced Fontan failure patients and as such should be encouraged and contributed to.

Repeat Pulmonary Valve Replacement: Similar Intermediate-Term Outcomes With Surgical and Transcatheter Procedures.

OBJECTIVES: This study compares 30-day, 1-year, and 3-year echocardiographic findings and clinical outcomes of transcatheter pulmonary valve-in-valve replacement (TPVR) and repeat surgical pulmonary valve replacement (SPVR). BACKGROUND: In patients with adult congenital heart disease and previous pulmonary valve replacement (PVR) who require redo PVR, it is unclear whether TPVR or repeat SPVR is the preferred strategy. METHODS: We retrospectively identified 66 patients (TPVR, n = 36; SPVR, n = 30) with bioprosthetic pulmonary valves (PVs) who underwent either TPVR or repeat SPVR at Emory Healthcare from January 2007 to August 2017. RESULTS: The TPVR cohort had fewer men and more patients with baseline New York Heart Association (NYHA) functional class III or IV. There was no difference in mortality, cardiovascular readmission, or post-procedural PV reintervention at 30 days, 1 year, or 3 years. Post-procedural echocardiographic findings showed no difference in mean PV gradients between the TPVR and SPVR groups at 30 days, 1 year, or 3 years. In the TPVR cohort, there was less right ventricular dysfunction at 30 days (2.9% vs. 46.7%; p < 0.01), despite higher baseline NYHA functional class in the SPVR cohort. CONCLUSIONS: In patients with bioprosthetic PV dysfunction who underwent either TPVR or SPVR, there was no difference in mortality, cardiovascular readmission, or repeat PV intervention at 30 days, 1 year, or 3 years. Additionally, TPVR and SPVR had similar intermediate-term PV longevity, with no difference in PV gradients or PVR. The TPVR cohort also had less right ventricular dysfunction at 30 days despite a higher baseline NYHA functional classification. These intermediate-term results suggest that TPVR may be an attractive alternative to SPVR in patients with previous bioprosthetic surgical PVs.

De novo DQ donor-specific antibodies are associated with worse outcomes compared to non-DQ de novo donor-specific antibodies following heart transplantation.

BACKGROUND: Antibody-mediated rejection (AMR) resulting from de novo donor-specific antibodies (dnDSA) leads to adverse outcomes following heart transplantation (HTx). It remains unclear what role dnDSA to specific HLA antigens play in adverse outcomes. This study compares outcomes in patients developing dnDSA to DQ antigens with those developing non-DQ dnDSA and those free from dnDSA. METHODS: The present study was a single-center, retrospective analysis of 122 consecutive HTx recipients. The primary outcome was a composite of death or graft dysfunction. RESULTS: After 3.3 years of follow-up, 31 (28%) patients developed dnDSA. Mean time to dnDSA was 539 days. Of 31 patients, 19 developed DQ antibodies and 12 developed non-DQ antibodies. Compared to non-DQ dnDSA, DQ antibodies presented with higher MFI values (P=.001) were more likely persistent (P=.001) and appeared later post-HTx (654 vs 359 days, P=.035). In a multivariable analysis, DQ dnDSA was associated with increased risk of the primary endpoint (HR 6.15, 95% CI 2.57-14.75, P=.001), whereas no increased risk was seen with non-DQ dnDSA (P=.749). CONCLUSIONS: dnDSA to DQ antigens following HTx are associated with increased risk of death and graft dysfunction.

"Frontiers in Fontan failure: A summary of conference proceedings".

"Frontiers in Fontan Failure" was the title of a 2015 conference sponsored by Children's Healthcare of Atlanta and Emory University School of Medicine. In what is hoped to be the first of many such gatherings, speakers and attendees gathered to discuss the problem of long-term clinical deterioration in these patients. Specific focuses included properly defining the problem and then discussing different treatment strategies, both medical and surgical. The health of the liver after Fontan palliation was a particular point of emphasis, as were quality of life and future directions.

Acute Embolic Myocardial Infarction and Heart Failure in a Fontan Patient: Recovery with Impella Device and Successful Transplantation.

Patients with Fontan palliation are at a higher risk of thromboembolic events, as well as sudden death. We report a case of acute thromboembolic myocardial infarction in a patient with Fontan circulation, presenting with acute heart failure. Coronary thrombolysis and reperfusion did not improve the myocardial dysfunction and warranted a unique application of mechanical support with Impella 2.5 pump as a bridge to decision. The patient was successfully transplanted. The case highlights the thromboembolic risks and provides support for the early use of mechanical support in such scenarios, even in single ventricle patients.

Catheter-measured Hemodynamics of Adult Fontan Circulation: Associations with Adverse Event and End-organ Dysfunctions.

BACKGROUND: In heart failure, a high systemic vascular resistance index (SVRI), high central venous pressure (CVP), and low cardiac index (CI) predict poor outcomes. Conversely, late hemodynamic manifestations of failing Fontan circulation and associations with end-organ dysfunction are not well understood. METHODS: A retrospective review of right-heart catheterization data of adult Fontan patients between 2002 and 2014 was conducted. Relationships between hemodynamic variables and serious adverse events (death or heart transplant) were examined using the Cox proportional hazard analysis. Correlations between the hemodynamic measurements and signs of end-organ dysfunction (MELD-XI, Child-Pugh, VAST score, estimated glomerular filtration rate [eGFR]) were analyzed. RESULTS: Sixty post-Fontan patients (85% systemic left ventricle, 40% atriopulmonary Fontan, mean age of 28 years, and mean time since Fontan operation of 21.9 years) were included. At baseline, those with an event were statistically younger, had lower transcutaneous oxygen saturations, were more likely to have an atriopulmonary Fontan, and were more likely to have a pacemaker. Eighteen experienced a cardiovascularly significant event. Using univariate analysis to compare the event and nonevent groups, mean CI was 2.8 ± 0.9 vs. 2.4 ± 0.5 L/min/m2 (P = .004), and CVP was 18.6 ± 6.5 vs. 16.1 ± 4.3 mmHg (P = .03). However, the statistical significances did not persist in the multivariate model. Higher CVP and pulmonary capillary wedge pressure (PCWP) were associated with higher MELD-XI and Child-Pugh scores, and the VAST score was only associated with PCWP. CONCLUSIONS: Symptomatic adult Fontan patients who experienced an event manifested with a higher CI and CVP, although the multivariate Cox proportional hazard analysis did not yield any significant associations. The presences of hepatic dysfunction and portal venous outflow obstruction were associated with a higher CVP and PCWP. Renal dysfunction was prevalent but no statistically significant association between the hemodynamic measurements was identified, although trends toward a higher CVP and transpulmonary gradient were identified.

The effect of angiotensin-converting enzyme inhibitors on the rate of ascending aorta dilation in patients with bicuspid aortic valve.

BACKGROUND: Bicuspid aortic valve (BAV), the most common congenital heart defect, is associated with progressive aortic dilation and increased risk of dissection and rupture. The use of an angiotensin-converting enzyme (ACE) inhibitor has recently proven effective in slowing the rate of aortic dilation in other aortopathies, suggesting it may also be useful in managing BAV. We sought to determine whether ACE inhibitors effectively reduce the rate of aortic dilation in adolescents and young adults with BAV. METHODS: A retrospective cohort study was undertaken. Subjects receiving ACE inhibitor therapy were compared with controls with BAV and matched for the degree of aortic regurgitation. The rate of change in ascending aorta diameter was compared between the two groups. RESULTS: Among the 141 subjects identified in the patient records, 103 were receiving no medication and 38 were taking ACE inhibitors. Over a mean follow-up of 37 months, the ascending aorta Z-score increased slightly among those receiving ACE inhibitor +4.5 ± 1.6 to +4.7 ± 1.6. The rate of change was no different than those subjects not receiving ACE inhibitors, P = .64. CONCLUSION: In an adolescent and young adult population with an isolated BAV, there is no proven benefit to ACE inhibitor use with regard to slowing rate of ascending aortic dilation.

Risk factors for major adverse events late after Fontan palliation.

OBJECTIVE: Risk factors for major adverse events late after Fontan palliation are unknown. Prior studies have suggested ventricular function and morphology as important risk factors. The aim of this study is to (1) characterize the late major adverse event profile in adult Fontan patients and (2) identify additional risk factors that may contribute to adverse outcomes. DESIGN AND SETTING: A retrospective review of all adult patients >15 years post-Fontan seen at a tertiary academic center was conducted. Clinical, laboratory, cardiac data, and abdominal imaging were collected via chart review. Major adverse events (death, cardiac transplantation, or listing) were identified, and timing of events was plotted using Kaplan-Meier methods. Univariate and multivariate logistic regression was used to determine independent predictors of late-term events. RESULTS: A total of 123 adult Fontan patients were identified (mean time post-Fontan 22.4 years [±4.4]). Major adverse events occurred in 19/123 patients (15%). In this 15-year survivor cohort, transplant-free survival rates were 94.6%, 82.9%, and 59.8% at 20, 25, and 30 years postoperation, respectively. Modes of death were Fontan failure with preserved function (4), congestive heart failure with decreased function (2), sudden death (2), thromboembolic event (1), post-Fontan conversion (2), and posttransplant (2). No differences in adverse outcomes were found based on morphology of the systemic ventricle, Fontan type, or systolic ventricular function. On the other hand, features of portal hypertension (OR 19.0, CI 4.7-77.3, P < .0001), presence of a pacemaker (OR 13.4, CI 2.6-69.8, P = .002), and systemic oxygen desaturation (OR 0.86, CI 0.75-0.98, P = .02) were risk factors for major adverse events in the multivariate analysis. CONCLUSIONS: In adult Fontan patients surviving >15 years post-Fontan, portal hypertension, oxygen desaturation, and need for pacemaker were predictive of adverse events. Traditional measures may not predict late-term outcomes in adult survivors; further study of the liver's role in late outcomes is warranted.

Hemodynamic phenotype of the failing Fontan in an adult population.

Fontan failure can occur even with normal systolic ventricular function and often in the context of significant liver disease. We hypothesized that Fontan failure is hemodynamically distinct from traditional heart failure and characterized by low systemic vascular resistance (SVR) index and preserved cardiac index. Twenty-seven symptomatic adult Fontan (SAF) patients who underwent catheterization from 2001 to 2011 constituted our study group. Fifty-four predominantly asymptomatic pediatric Fontan (PF) patients who underwent catheterization during the same period were randomly selected to perform a control:case cohort analysis. Clinical comparisons were made between the 2 groups. The adults were more symptomatic than the PF cohort (New York Heart Association classes I and II or III and IV: 48% or 52% [SAF] vs 94% or 6% [PF], respectively, p <0.01). SAF versus PF mean catheterization findings were central venous pressure 18 ± 6 versus 14 ± 3 mm Hg (p <0.01), SVR index 1,680 ± 368 versus 1,960 ± 550 dyn s/cm(5)/m(2) (p = 0.02), and cardiac index 2.7 ± 0.8 versus 2.8 ± 0.7 L/min/m(2) (p = 0.25). By imaging, the SAF cohort demonstrated a greater incidence of abnormal liver texture changes (96% vs 75%, p = 0.04) and nodularity (77% vs 42%, p = 0.02). In conclusion, adult patients with failing Fontan circulation had a lower SVR index and similar cardiac index compared with the pediatric cohort. Liver disease in the adults was more advanced. Our data suggest that Fontan failure is a distinct circulatory derangement with hemodynamic features similar to portal hypertension, albeit with limited ability to augment cardiac output.

Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study.

BACKGROUND: Chronic congestive hepatopathy is known to cause hepatic fibrosis and portal hypertension in patients post-Fontan operation for single ventricle palliation. The clinical significance of these findings is not clear. We hypothesized that features of portal hypertension would be significantly related to major adverse events. METHODS: A retrospective review of 73 adult and pediatric post-Fontan patients referred for a liver evaluation from 2001 to 2011 was performed. The relationship between features of portal hypertension (VAST score ≥2, 1 point each for Varices, Ascites, Splenomegaly or Thrombocytopenia) and a major adverse event (death, need for transplant, or hepatocellular carcinoma) was examined using logistic regression. RESULTS: 73 post-Fontan patients (30% female, 73% Caucasian, 66% systemic left ventricle (SLV), mean age 24±11 years, mean interval from Fontan 17±6 years) were included in analysis. Features of portal hypertension (VAST score ≥2) were present in 26 (36%), and there were 19 major adverse events: death (n=12), transplant (n=6), and HCC (n=1). A significant relationship was found between VAST score ≥2 and major adverse events (OR=9.8, 95% CI [2.9-32.7]). After adjusting for time since Fontan, SLV, age, hemoglobin and type of failure, VAST score ≥2 remained significant (OR=9.1, 95% CI [1.4-57.6]). CONCLUSION: Fontan patients with features of portal hypertension have a 9-fold increased risk for a major adverse event. Therapies targeted to manage clinical manifestations of portal hypertension, and early referral to heart transplant may help delay major adverse events. Future prospective studies are needed to confirm these findings.