Abstract 10056: Clinical Portal Hypertension is Related to Major Adverse Outcomes in Adolescents and Adults With Congenital Heart Disease After Fontan Palliation
Background: Chronic congestive hepatopathy is known to cause hepatic fibrosis and portal hypertension (PortH), presumably post-sinusoidal, in persons post-Fontan operation for single ventricle (SV) palliation, but the clinical significance of these findings is not clear. Inability to augment cardiac output in the vasodilated state of PortH may increase vulnerability to end-organ hypoperfusion. We hypothesized that clinical PortH would be significantly related to major adverse outcomes.
Methods: A retrospective study of patients post-Fontan who underwent a hepatic evaluation at our institution was performed. Patients were screened, but not excluded, for causes of hepatic injury (Hepatitis C, n=0; alcohol, n=1). The relationship between clinical PortH (defined as at least two of the following: thrombocytopenia (platelet <150K), splenomegaly, varices, or ascites) and a major adverse health outcome (combined outcome of death, transplant, or hepatocellular carcinoma (HCC)] was examined using logistic regression.
Results: A total of 72 patients s/p Fontan palliation (30% female, 73% Caucasian, 66% systemic left ventricle (SLV), mean age 24 years (range 9-54 years), mean time post-Fontan 17 ± 9 years) were included in analysis. Clinical PortH was present in 25 persons (34.7%), and 18 persons (23.6%) experienced a major adverse health outcome: death (n=12), transplant (n=6), HCC (n=1). A significant relationship was found between clinical PortH and major adverse health outcomes (OR=10.9, 95% CI [3.2-37.0]) in the presence of normal serologic liver testing. After adjusting for time since Fontan, SLV, age, hemoglobin and type of failure (heart failure (11%), Fontan failure (34%), or no failure (55%)), clinical PortH remained significantly related to major adverse outcomes (OR=8.0, 95% CI [1.3-51.3]).
Conclusion: Post-Fontan patients with clinical portal hypertension have an eight-fold increase in risk for a major adverse health outcome. Monitoring for clinical manifestations of PortH is recommended following Fontan operation. Therapies targeted to manage clinical manifestations of PortH, and early referral to heart transplant may help delay major adverse health outcomes. Future prospective studies are needed to confirm these findings.
- © 2012 by American Heart Association, Inc.