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

Abstract

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 2001-2011 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), 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.

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

“…125 In addition, extrahepatic findings such as splenomegaly, ascites, or collaterals and varices may offer additional prognostic value. 126 Any concerning changes on physical examination or laboratory testing are a prompt for imaging of the liver, preferably with MR. Whether to image the liver in an asymptomatic patient who is at risk for liver disease but without examination findings or serological tests suggestive of liver disease is open to debate.…”

Changing Indications for Pediatric Heart Transplantation

“…In the Fontan patient, chronic hepatic congestion has been found to lead to fibrosis and ultimately cirrhosis. 100,101 The impacts of portal fibrosis and cirrhosis on outcomes after transplantation are under investigation, with a few patients undergoing heart and liver transplantation when advanced cirrhosis is present. [102][103][104] The type of congenital heart disease also affects survival after transplantation.…”

Exercise Oscillatory Ventilation in Patients With Fontan Physiology

“…[35][36][37][38] Investigators are actively exploring other predictors of poor outcome for Fontan patients, including creatinine, MELD-XI (Model for End-stage Liver Disease eXcluding INR), and other composite clinical scores. [39][40][41] Because EOV represents an acute physiological response and is potentially modifiable, 42 it may provide additive information when used in conjunction with static measures of chronic disease to help guide clinical decision making.…”