Nancy McCabe

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.

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 livers role in late outcomes is warranted.

Hepatocellular carcinoma in an adult with repaired tetralogy of fallot.

Liver fibrosis is a growing concern among adults with congenital heart disease, particularly for those who have undergone a Fontan operation. Liver fibrosis leads to cirrhosis, a precursor of hepatocellular carcinoma. A few cases of hepatocellular carcinoma in patients with prior palliative surgery for congenital heart disease have been identified in the literature. The current case reports the first known case of hepatocellular carcinoma in a 45-year-old male with repaired tetralogy of Fallot.

Antecedents of self-care in adults with congenital heart defects

BACKGROUND Adults with congenital heart defects (ACHD) face long-term complications related to prior surgery, abnormal anatomy, and acquired cardiovascular conditions. Although self-care is an important part of chronic illness management, few studies have explored self-care in the ACHD population. The purpose of this study is to describe self-care and its antecedents in the ACHD population. METHODS Persons with moderate or severe ACHD (N=132) were recruited from a single ACHD center. Self-care (health maintenance behaviors, monitoring and management of symptoms), and potential antecedents including sociodemographic and clinical characteristics, ACHD knowledge, behavioral characteristics (depressive symptoms and self-efficacy), and family-related factors (parental overprotection and perceived family support) were collected via self-report and chart review. Multiple regression was used to identify antecedents of self-care maintenance, monitoring, and management. RESULTS Only 44.7%, 27.3%, and 23.3% of participants performed adequate levels of self-care maintenance, monitoring and management, respectively. In multiple regression analysis, self-efficacy, education, gender, perceived family support, and comorbidities explained 25% of the variance in self-care maintenance (R(2)=.248, F(5, 123)=9.44, p<.001). Age, depressive symptoms, self-efficacy, and NYHA Class explained 23% of the variance in self-care monitoring (R(2)=.232, F(2, 124)=10.66, p<.001). Self-efficacy and NYHA Class explained 9% of the variance in self-care management (R(2)=.094, F(2, 80)=5.27, p=.007). CONCLUSIONS Low levels of self-care are common among persons with ACHD. Multiple factors, including modifiable factors of self-efficacy, depressive symptoms, and perceived family support, are associated with self-care and should be considered in designing future interventions to improve outcomes in the ACHD population.

REDUCED HEPATIC VENOUS FLOW IS A MARKER OF ADVERSE OUTCOMES IN PATIENTS WITH FONTAN CIRCULATION

background: Liver injury and portal hypertension are significant complications of the Fontan circulation and are thought to result from chronic venous hypertension. The flow characteristics of the involved veins, specifically the hepatic vein (HV), are unknown in this population, and their association with clinical outcomes has not been studied. We hypothesize that decreased HV flow is associated with adverse clinical outcomes.

Six-minute walk distance predicts 30-day readmission after acute heart failure hospitalization

Objectives To determine the relationship between 6‐min walk test distance (6MWD) and 30‐day readmission in hospitalized heart failure (HF) patients. Background 6MWD is known to predict hospitalizations in outpatients with HF, but its ability to predict recidivism in hospitalized HF patients is relatively unknown. Methods Seventy‐one hospitalized HF patients with NYHA Class II/III (mean age 52.6 ± 12.3 years, 42.3% female, 73.2% African American) performed 6MWD prior to discharge. Logistic regression was used to determine relationships between 6MWD and 30‐day readmission. Results 30‐day readmission occurred in 14 (19.7%) patients. Average 6MWD was 756.4 ± 403.2 feet. Higher 6MWD significantly decreased risk of 30‐day readmission, even after adjusting for sociodemographic and clinical characteristics (OR = .84, 95% CI [.71, .99]). For each additional 100 feet walked, odds of a 30‐day readmission decreased by 16%. Conclusions 6MWD predicted 30‐day readmission in this study, warranting further investigation to understand how the 6MWD may predict readmissions and guide treatment in hospitalized HF patients.

IMMUNOLOGIC AGING IN ADULTS WITH CONGENITAL HEART DISEASE: DOES INFANT STERNOTOMY MATTER?

Thymectomy is preformed routinely in infants during cardiothoracic surgery to enable repair of congenital heart disease (CHD). Prior work has shown that children post-sternotomy have decreased T-lymphocyte levels, without proven compromise in immune function. Long-term assessment of immune function