Mechanical thrombectomy of COVID-19 DVT with congenital heart disease leading to phlegmasia cerulea dolens: a case report.

BACKGROUND: COVID-19 and Fontan physiology have each been associated with an elevated risk of venous thromboembolism (VTE), however little is known about the risks and potential consequences of having both. CASE PRESENTATION: A 51 year old male with tricuspid atresia status post Fontan and extracardiac Glenn shunt, atrial flutter, and sinus sick syndrome presented with phlegmasia cerulea dolens (PCD) of the left lower extremity in spite of supratherapeutic INR in the context of symptomatic COVID-10 pneumonia. He was treated with single session, catheter directed mechanical thrombectomy that was well-tolerated. CONCLUSIONS: This report of acute PCD despite therapeutic anticoagulation with a Vitamin K antagonist, managed with emergent mechanical thrombectomy, calls to attention the importance of altered flow dynamics in COVID positive patients with Fontan circulation that may compound these independent risk factors for developing deep venous thrombosis with the potential for even higher morbidity.

Using a Daily Diary Approach to Examine Substance Use and Negative Sexual Experiences Among College Students.

This article examined substance use and sexual behavior by conducting an analysis of college students' reported behaviors using a daily diary approach. By isolating particular sexual events across a 2-month period, we examined situational predictors of engagement in sex and of negative sexual experiences (coerced sex and/or sex that lacks perceived control) for college men and women. Data come from the daily diary sub-study of the Sexual Health Initiative to Foster Transformation. These data include 60 days of daily responses from 420 undergraduates at one New York City institution. This was a relatively diverse sample comprised of 49% women, 28% identifying as non-heterosexual, 60% non-white, and a roughly equal number of college freshman, sophomores, juniors, and seniors. Analyses examined the effects of alcohol use, binge drinking, marijuana use, and other drug use on sexual experiences. Between-person and within-person substance uses were related to an increased likelihood of having at least one sexual encounter during the study period. After adjusting for each participants' average substance use, both the number of alcoholic drinks consumed (AOR 1.13 (1.05-1.21)) and binge drinking scores (AOR 2.04 (1.10-3.79)) increased the likelihood of negative sex. Interaction analyses showed that compared to men, women were more likely to use alcohol and marijuana prior to sexual encounters. Given that sex and substance use are co-occurring, current prevention approaches should be paired with strategies that attempt to prevent negative sexual experiences, including sexual assault, more directly. These include consent education, bystander training, augmentation of sexual refusal skills, and structural change. Efforts promoting increased sex positivity might also help make all students, and women in particular, less likely to use substances in order to facilitate sex.

The effects of pulmonary valve replacement on QRS duration in repaired tetralogy of Fallot patients with pulmonary regurgitation.

INTRODUCTION: Chronic pulmonary regurgitation (PR) following surgical correction in Tetralogy of Fallot (TOF) leads to right ventricular (RV) dysfunction, arrhythmias and sudden cardiac death (SCD). Pulmonary valve replacement (PVR) decreases PR and improves RV function, but data regarding QRS duration reduction remain scarce. METHODS: All adult TOF patients undergoing transcatheter PVR or surgical PVR from 2010 to 2016 were included. Clinical characteristics and QRS duration were measured and compared to post-intervention QRS duration using an institutional software and manually verified. Significantly wide QRS was defined as QRS >140 ms. RESULTS: Of 133 PVR patients, 85 had TOF and 27 (21.1%) had QRS > 140 ms (14 transcatheter, 13 surgical) and were included in this analysis. A 6 ms decrease in QRS duration was seen at 3-year follow-up (168.0 ±â€¯3.5 ms vs. 161.8 ±â€¯3.5 ms, p = .04). There was a significant decrease in the median RV size (defined as RV/LV diameter ratio) pre-intervention to 3-year post-intervention: (0.96 vs 0.89, p = .03). The median PR decreased significantly from moderate-severe to mild post-intervention (p < .0001). CONCLUSIONS: Replacement of the pulmonary valve in high risk TOF patients reduces QRS duration at 3 years. Further study is needed to assess whether this QRS duration reduction may identify patients at lower risk of ventricular arrhythmias.

Knowledge of Life-Long Cardiac Care by Adolescents and Young Adults with Congenital Heart Disease.

National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption. We, therefore, sought to determine the knowledge of adolescents and young adults regarding LLCC. In this multi-center study, we administered a survey to patients 13-20 years of age with surgically repaired congenital heart disease. We assessed the understanding of both their need for LLCC and awareness of the type of recommended care providers. A total of 290/302 (96%) patients approached in the outpatient clinic setting (10 centers) agreed to study participation; mean age was 16.3 ± 2.3 years; patients were 62% male. While the need for LLCC was recognized by 78% of subjects, only 37% understood that an ACHD specialist in adulthood should guide this care. Only 37% of respondents stated that their current cardiology team had spoken to them about LLCC, but 90% wished to learn more. A substantial number of adolescents and young adults with moderate and complex CHD lack adequate knowledge about LLCC, but most have a desire to learn more about the type of care they will require in adulthood. Transition education/assessment ensuring successful transfer to adult-oriented care for this population should emphasize the importance of LLCC.

Wine night, 'bro-dinners', and jungle juice: Disaggregating practices of undergraduate binge drinking.

Undergraduate binge drinking, a well-documented problem at US institutions of higher education, has been associated with a host of negative behavioral health outcomes such as sexual assault, poor academic functioning, and mental health problems. Scholars have extensively examined individual-and institutional-level risk factors for binge drinking on campuses. However, these data have not been effectively translated into interventions to reduce rates of binge drinking. To inform the development of additional evidence-based binge-drinking prevention programs for college campuses, this paper documents the varied goals and social contexts that constitute 'binge drinking', drawing on primarily ethnographic data. By disaggregating what survey research has largely examined as a unified outcome, we offer a descriptive account of the different reasons for and contexts in which students consume alcohol in amounts that constitute binge drinking: to meet new people at parties, to socialize with close friends, when hoping to find a sexual partner, when anticipating moving to a space where alcohol is more difficult to procure, to provide a way to move between affectively different situations, to cope with stress or anxiety, and to fit in. Our discussion links these motivations to factors beyond the individual and institutional levels, and points to modifiable social factors in university life as strategy for prevention. The implication of our argument is that acknowledging and responding to the varied motivations underlying students' alcohol use is one strategy to enhance campus binge-drinking prevention.

Prognostic utility of MELD-XI in adult congenital heart disease patients undergoing cardiac transplantation.

BACKGROUND: Model of End-Stage Liver Disease eXcluding INR (MELD-XI) at cardiac transplant has demonstrated prognostic survival utility, but has not been specifically validated in adult congenital heart disease (ACHD) in a registry study. METHODS: Adults undergoing first-time orthotopic heart transplant from 2005 to 2015 in the United Network for Organ Sharing (UNOS) registry were examined in parallel: ACHD (n = 543), ischemic-dilated cardiomyopathy (IDCM, n = 6954) and valvular heart disease (VHD, n = 355). Our primary endpoint was a composite of death, graft failure, and retransplantation assessed at 3 months (early), and those with freedom from early endpoint were reassessed at 5 years (late). Interactions between hepatorenal indices and waitlist time were examined. Secondary outcomes relating to long-term morbidity were assessed at late endpoint. Freedom from endpoint analysis in ACHD at clinically relevant endpoints was also conducted. RESULTS: Model of End-Stage Liver Disease eXcluding INR score at transplant associated with an increased risk of early endpoint in all cohorts. At late endpoint, bilirubin level associated with increased risk uniquely in ACHD. CONCLUSIONS: Model of End-Stage Liver Disease eXcluding INR holds prognostic application to ACHD in early time points and demonstrates unique waitlist interactions. Transplant bilirubin level may hold significance in long-term risk stratification of the ACHD population. Time on waitlist is an important consideration to contextualize these values.

Heart and heart-liver transplantation in adults with failing Fontan physiology.

BACKGROUND: As the population of patients with a Fontan palliation grows so does, the number of patients with cardiac failure necessitating orthotopic heart transplant (OHT) and combined heart-liver transplant (CHLT). There is recent evidence that current era cardiac transplant in Fontan patients has improved outcomes, but most studies have a preponderance of pediatrics patients in their cohorts. We examine our institutional experience with adult OHT and CHLT transplantation for failed Fontan physiology. METHODS AND RESULTS: Retrospective analysis of patients at the Ahmanson/UCLA Adult Congenital Heart Disease Center who underwent OHT or CHLT for failing Fontan physiology from January 1, 2002 to May 31, 2017. We identified 20 patients with single-ventricle physiology and Fontan palliation who underwent OHT or CHLT. The median age was 29.5 years (range 19-44). Five patients underwent CHLT because of biopsy proven hepatic cirrhosis. The median length of hospital stay was 23 days (range 8-76) post-OHT and 51 days (range 26-77) post-CHLT. During a median follow-up of 56 months (range 2-178), there was one mortality occurring at 34 months post-OHT due to coronary vasculopathy. Most frequent early postoperative complications included bleeding and infection (55% and 20%, respectively) and surgical reintervention for bleeding complications (n = 8, 40%). One CHLT patient experienced clinically significant hepatic rejection requiring admission and steroid treatment. CONCLUSIONS: Despite inherent risks and complexities of OHT or CHLT in patients with a failed Fontan, transplant is a reasonable therapy. Peri- and postoperative complications are common and may require surgical reintervention. Continued observation of practices and unifying themes may help improve patient selection, pre- and postoperative treatment and ultimately outcomes.

An Observation from Liver Biopsies Two Decades Post-Fontan.

This brief report describes an observation from liver biopsy results in nonfailing Fontan patients, currently in their second postoperative decade. In three patients, with either atriopulmonary or atrioventricular connections and functional left ventricles, we found no portal fibrosis. In contrast, we found portal fibrosis in three clinically similar, nonfailing Fontan patients with lateral tunnel connections and functional left ventricles. We recognize the results may be secondary to chance; nevertheless, we speculate about possible relevancy.

Fontan Outcomes and Pulmonary Blood Flow at Birth.

We previously noted, in a small group of post-Fontan patients, a possible association between hepatic fibrosis scores and the status of pulmonary blood flow at birth. To further explore this observation, we examined data from all Fontan patients seen in our center from July 2010 to March 2015. We identified 200 patients for analysis. Of the 200 patients, 56 underwent transvenous-hepatic biopsy. Of the 200 patients, 13 (6.5%) had protein-losing enteropathy. We divided both the 56 biopsy patients and the entire cohort of 200 patients into 4 groups: (1) unobstructed pulmonary blood flow at birth with functional left ventricles, (2) unobstructed pulmonary blood flow at birth with functional right ventricles, (3) obstructed pulmonary blood flow at birth with functional left ventricles, and (4) obstructed pulmonary blood flow at birth with functional right ventricles. Analysis of the 56 liver-biopsy patient groups showed median hepatic total-fibrosis scores for the 4 groups of 2 (0-6), 2 (0-8), 3 (2-6), and 4 (1-8), respectively, with statistical significance between groups 4 and 1 (p = 0.031). For the entire cohort of 200 patients, we analyzed the incidence of protein-losing enteropathy for each of the four groups and found protein-losing enteropathy percent occurrences of 0, 2.9, 8.8, and 16.1, respectively, with statistical significance between groups 4 and 2 (p = 0.031) and between groups 4 and 1 (p = 0.025). A history of obstructed pulmonary blood flow at birth, coupled with a functional right ventricle, may predict a poorer long-term Fontan outcome.

Emphasizing Sexual Orientation and Gender Identity Data Capture for Improved Cardiovascular Care of the LGBTQ+ Population.

Importance: The rising self-identifying lesbian, gay, bisexual, transgender, and queer (LGBTQ+) population makes understanding the unique health care needs of sexual and gender minoritized patients an urgent one. The interaction between minority stress and cardiovascular disease has been well described among underrepresented minoritized populations. The underrepresentation of minoritized populations in clinical research is partly responsible for worse cardiovascular outcomes in these populations. The absence of sexual orientation and gender identity and expression (SOGIE) data makes it difficult to understand the cardiovascular health of LGBTQ+ adults, thereby widening health care disparities in this population. Advancing cardiovascular health equity for LGBTQ+ patients must begin with careful and accurate SOGIE data collection. Observations: Current SOGIE data capture remains inadequate despite federal mandates. Challenges in data collection include political and regulatory discrimination, patient/practitioner hesitancy, lack of supportive guidance on SOGIE data collection, improper terminology, regulatory inertia, and inadequate and often incorrect integration of SOGIE data into electronic health records (EHRs). Additional challenges include grouping participants as "others" for statistical significance. The inclusion of SOGIE data has demonstrated an impact in other fields like cancer survivorship and surgery. The same needs to be done for cardiology. Conclusions and Relevance: Potential solutions for improving much-needed SOGIE data collection include (1) implementing LGBTQ+ inclusive policies, (2) integrating SOGIE data into the EHR, (3) educating health care professionals on the relevance of SOGIE to patient-centered care, and (4) creating a diverse cardiovascular workforce. These steps can substantially enhance the ability to collect SOGIE data to address LGBTQ+ cardiovascular health care disparities.

Intrahepatic Transcriptomics Differentiate Advanced Fibrosis and Clinical Outcomes in Adults With Fontan Circulation.

BACKGROUND: The molecular mechanisms underlying Fontan-associated liver disease (FALD) remain largely unknown. OBJECTIVES: This study aimed to assess intrahepatic transcriptomic differences among patients with FALD according to the degree of liver fibrosis and clinical outcomes. METHODS: This retrospective cohort study included adults with the Fontan circulation. Baseline clinical, laboratory, imaging, and hemodynamic data as well as a composite clinical outcome (CCO) were extracted from medical records. Patients were classified into early or advanced fibrosis. RNA was isolated from formalin-fixed paraffin-embedded liver biopsy samples; RNA libraries were constructed with the use of an rRNA depletion method and sequenced on an Illumina Novaseq 6000. Differential gene expression and gene ontology analyses were performed with the use of DESeq2 and Metascape. RESULTS: A total of 106 patients (48% male, median age 31 years [IQR: 11.3 years]) were included. Those with advanced fibrosis had higher B-type natriuretic peptide levels and Fontan, mean pulmonary artery, and capillary wedge pressures. The CCO was present in 23 patients (22%) and was not predicted by advanced liver fibrosis, right ventricular morphology, presence of aortopulmonary collaterals, or Fontan pressures on multivariable analysis. Samples with advanced fibrosis had 228 upregulated genes compared with early fibrosis. Samples with the CCO had 894 upregulated genes compared with those without the CCO. A total of 136 upregulated genes were identified in both comparisons and were enriched in cellular response to cytokine stimulus or oxidative stress, VEGFA-VEGFR2 signaling pathway, TGF-ß signaling pathway, and vasculature development. CONCLUSIONS: Patients with FALD and advanced fibrosis or the CCO exhibited upregulated genes related to inflammation, congestion, and angiogenesis.

A new algorithm DEtectS critical Congenital Heart Disease at different altitudes: ANDES-CHD study.

BACKGROUND: Neonatal pulse oximetry screening (POS) algorithms for critical congenital heart disease (CCHD) have contributed towards decreasing neonatal mortality but cannot be applied at high altitudes. New POS algorithms at high altitudes are needed. METHODS: This observational, prospective study included newborns born at different altitudes from 0 to 4380 meters above the sea level in Peru. Healthy newborns underwent neonatal preductal and postductal oximetry, echocardiography and telephonic follow-up up to 12 months of age. Newborns with CCHD underwent preductal and postductal oximetry at the time of telemedicine evaluation while located at the high-altitude hospital where they were born, and their diagnoses were confirmed with echocardiography locally or after arriving to the referral center. Two new algorithms were designed using clinically accepted neonatal oximetry cutoffs or the 5th and 10th percentiles for preductal and postductal oximetry values. RESULTS: A total of 502 healthy newborns and 15 newborns with CCHD were enrolled. Echocardiography and telephonic follow-up were completed in 227 (45%) and 330 healthy newborns (65%), respectively. The algorithm based on clinically accepted cutoffs had a sensitivity of 92%, specificity of 73% and false positive rate of 27% The algorithm based on the 5th and 10th percentiles had a sensitivity of 80%, specificity of 88% and false positive rate of 12%. CONCLUSIONS: Two algorithms that detect CCHD at different altitudes had adequate performance but high false positive rates.

The role of race and english proficiency on the health of older immigrants.

This study applies the Commission on Social Determinants of Health model to identify the effect of ethnicity/race and English proficiency on the health of older immigrants. California Health Interview Survey data of foreign-born respondents aged 65 and over were used to examine the four outcomes of health-related quality of life (HRQOL). The study included 1,196 immigrant Latinos, Asians, and non-Hispanic Whites. The results show that ethnicity/race-based differences in HRQOL exist. Furthermore, the results indicate that English proficiency has a significant moderating relationship on racial/ethnic background. The likelihood of reporting more Limited Combined Days increased with lower levels of English proficiency for both Latino and Asian-American old adults as compared to non-Hispanic Whites. In addition to focusing on racial disparities, health promotion efforts with older immigrants need to examine language-based stratification. Social work and gerontological advocates need to develop and employ evidence-based interventions that reach limited-English-proficient older immigrants to address the health, psychosocial, and access to health care challenges they face.

Clinical and invasively-measured predictors of high exercise capacity in Fontan patients.

INTRODUCTION: Fontan patients have variable exercise capacity. Contemporary understanding as to which factors predict high tolerance is limited. METHODS: Records from the Ahmanson/University of California, Los Angeles Adult Congenital Heart Disease Center were reviewed for adult Fontan patients who underwent CPET. Patients were considered "high performers" if their maximum oxygen uptake (VO2 max/kg)-predicted was greater than 80%. Cross-sectional clinical, hemodynamic, and liver biopsy data was gathered. High-performers were compared to control patients across these parameters via associations and regression. RESULTS: A total of 195 adult patients were included; 27 patients were considered "high performers". They had lower body mass indices (BMI, p < 0.001), mean Fontan pressures (p = 0.026), and cardiac outputs (p = 0.013). High performers also had higher activity levels (p < 0.001), serum albumin levels (p = 0.003), non-invasive and invasive systemic arterial oxygen saturations (p < 0.001 and p = 0.004), lower New York Heart Association (NYHA) heart failure class (p = 0.002), and were younger at Fontan completion (p = 0.011). High performers had less severe liver fibrosis (p = 0.015). Simple regression found Fontan pressure, non-invasive O2 saturation, albumin level, activity level, age at Fontan surgery, NYHA class, and BMI to predict significant changes in VO2 max/kg %-predicted. These associations persisted in multiple regression for non-invasive O2 saturation, NYHA class II, activity level, and BMI. CONCLUSIONS: Thin Fontan patients who exercise more had better exercise capacity, Fontan hemodynamic profiles, and less liver fibrosis.

Sexual Health and Well-Being in Adults With Congenital Heart Disease: A International Society of Adult Congenital Heart Disease Statement.

As health care outcomes improve the priority for those living with adult congenital heart disease have changed to a more holistic focus on quality of life and well-being. Although health care has embraced this, there are still areas where there is a deficit in advice, allyship, and advocacy. One of these deficits is in the area of sexual health and well-being. A healthy sexual life has a myriad of physical and psychosocial benefits. However, individuals with adult congenital heart disease may have significant barriers to achieving well-being in this aspect of their lives. These barriers and their potential solutions are outlined in this paper.

Early aspirin use and the development of cardiac allograft vasculopathy in pediatric heart transplant recipients: A pediatric heart transplant society analysis.

BACKGROUND: Cardiac allograft vasculopathy (CAV) remains a leading cause of graft loss in pediatric heart transplant (HTx) recipients. Adult literature suggests that aspirin (ASA) use in the early post-HTx period may reduce the risk of CAV. This study aimed to determine the impact of early ASA use on the development of CAV in pediatric HTx recipients. METHODS: All subjects <17 years of age at time of primary HTx who survived ≥3 years without evidence of CAV were identified for inclusion from the Pediatric Heart Transplant Society database (1996-2019). Early ASA use was defined as ASA started within the first 3 years post-HTx and was classified as continuous or intermittent. Frequency of ASA use was described across centers. Kaplan-Meier method assessed freedom from CAV and overall graft survival. Multiphase parametric hazard analyses and propensity score matched analysis were used to identify independent risk factors. RESULTS: 3,011 patients were included with 387 (13%) receiving continuous ASA, 676 (22%) receiving intermittent ASA, and 1,948 (65%) receiving no ASA. ASA use was highly variable across centers (0%-100%). At baseline patients receiving continuous ASA therapy demonstrated inferior graft survival (p < 0.001) and worse freedom from CAV (p = 0.002), but with lower CAV grades (p = 0.05). In multiphase parametric hazard modeling continuous ASA use was not independently associated with CAV, but remained associated with inferior graft survival. Propensity-matched sub-analysis between continuous and no ASA groups demonstrated no difference in freedom from CAV or overall graft loss. CONCLUSIONS: ASA use varies widely across pediatric HTx centers. Early ASA use did not reduce the risk of CAV or graft loss in pediatric heart transplant recipients.

Intrahepatic transcriptomics differentiate advanced fibrosis and clinical outcomes in adults with the Fontan circulation.

Background: The molecular mechanisms underlying Fontan associated liver disease (FALD) remain largely unknown. We aimed to assess intrahepatic transcriptomic differences among patients with FALD according to the degree of liver fibrosis and clinical outcomes. Methods: This retrospective cohort study included adults with the Fontan circulation at the Ahmanson/UCLA Adult Congenital Heart Disease Center. Clinical, laboratory, imaging and hemodynamic data prior to the liver biopsy were extracted from medical records. Patients were classified into early (F1-F2) or advanced fibrosis (F3-F4). RNA was isolated from formalin-fixed paraffin embedded liver biopsy samples; RNA libraries were constructed using rRNA depletion method and sequencing was performed on Illumina Novaseq 6000. Differential gene expression and gene ontology analyses were carried out using DESeq2 and Metascape. Medical records were comprehensively reviewed for a composite clinical outcome which included decompensated cirrhosis, hepatocellular carcinoma, liver transplantation, protein-losing enteropathy, chronic kidney disease stage 4 or higher, or death. Results: Patients with advanced fibrosis had higher serum BNP levels and Fontan, mean pulmonary artery and capillary wedge pressures. The composite clinical outcome was present in 23 patients (22%) and was predicted by age at Fontan, right ventricular morphology and presence of aortopulmonary collaterals on multivariable analysis. Samples with advanced fibrosis had 228 up-regulated genes compared to early fibrosis. Samples with the composite clinical outcome had 894 up-regulated genes compared to those without it. A total of 136 up-regulated genes were identified in both comparisons and these genes were enriched in cellular response to cytokine stimulus, response to oxidative stress, VEGFA-VEGFR2 signaling pathway, TGF-beta signaling pathway, and vasculature development. Conclusions: Patients with FALD and advanced liver fibrosis or the composite clinical outcome exhibit up-regulated genes including pathways related to inflammation, congestion, and angiogenesis. This adds further insight into FALD pathophysiology.

Clinical Outcomes of Adult Fontan-Associated Liver Disease and Combined Heart-Liver Transplantation.

BACKGROUND: The impact of Fontan-associated liver disease (FALD) on post-transplant mortality and indications for combined heart-liver transplant (CHLT) in adult Fontan patients remains unknown. OBJECTIVES: The purpose of this study was to assess the impact of FALD on post-transplant outcomes and compare HT vs CHLT in adult Fontan patients. METHODS: We performed a retrospective-cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers. Inclusion criteria were as follows: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at referral. Pretransplant FALD score was calculated using the following: 1) cirrhosis; 2) varices; 3) splenomegaly; or 4) ≥2 paracenteses. RESULTS: A total of 131 patients (91 HT and 40 CHLT) were included. CHLT recipients were more likely to be older (P = 0.016), have a lower hemoglobin (P = 0.025), require ≥2 diuretic agents pretransplant (P = 0.051), or be transplanted in more recent decades (P = 0.001). Postmatching, CHLT demonstrated a trend toward improved survival at 1 year (93% vs 74%; P = 0.097) and improved survival at 5 years (86% vs 52%; P = 0.041) compared with HT alone. In patients with a FALD score ≥2, CHLT was associated with improved survival (1 year: 85% vs 62%; P = 0.044; 5 years: 77% vs 42%; P = 0.019). In a model with transplant decade and FALD score, CHLT was associated with improved survival (HR: 0.33; P = 0.044) and increasing FALD score was associated with worse survival (FALD score: 2 [HR: 14.6; P = 0.015], 3 [HR: 22.2; P = 0.007], and 4 [HR: 27.8; P = 0.011]). CONCLUSIONS: Higher FALD scores were associated with post-transplant mortality. Although prospective confirmation of our findings is necessary, compared with HT alone, CHLT recipients were older with higher FALD scores, but had similar survival overall and superior survival in patients with a FALD score ≥2.

Morbidity and Mortality in Adult Fontan Patients After Heart or Combined Heart-Liver Transplantation.

BACKGROUND: An increasing number of adult Fontan patients require heart transplantation (HT) or combined heart-liver transplant (CHLT); however, data regarding outcomes and optimal referral time remain limited. OBJECTIVES: The purpose of this study was to define survivorship post-HT/CHLT and predictors of post-transplant mortality, including timing of referral, in the adult Fontan population. METHODS: A retrospective cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers in the United States and Canada was performed. Inclusion criteria included the following: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at the time of referral. Date of "failing" Fontan was defined as the earliest of the following: worsening fluid retention, new ascites, refractory arrhythmia, "failing Fontan" diagnosis by treating cardiologist, or admission for heart failure. RESULTS: A total of 131 patients underwent transplant, including 40 CHLT, from 1995 to 2021 with a median post-transplant follow-up time of 1.6 years (Q1 0.35 years, Q3 4.3 years). Survival was 79% at 1 year and 66% at 5 years. Survival differed by decade of transplantation and was 87% at 1 year and 76% at 5 years after 2010. Time from Fontan failure to evaluation (HR/year: 1.23 [95% CI: 1.11-1.36]; P < 0.001) and markers of failure, including NYHA functional class IV (HR: 2.29 [95% CI: 1.10-5.28]; P = 0.050), lower extremity varicosities (HR: 3.92 [95% CI: 1.68-9.14]; P = 0.002), and venovenous collaterals (HR: 2.70 [95% CI: 1.17-6.20]; P = 0.019), were associated with decreased post-transplant survival at 1 year in a bivariate model that included transplant decade. CONCLUSIONS: In our multicenter cohort, post-transplant survival improved over time. Late referral after Fontan failure and markers of failing Fontan physiology, including worse functional status, lower extremity varicosities, and venovenous collaterals, were associated with post-transplant mortality.