Background
In the Fontan procedure, a palliative operation for single-ventricle
congenital heart disease, the systemic veins are connected directly to
the pulmonary circulation without a subpulmonary ventricle. Individuals
with Fontan circulation are at risk for heart failure and liver disease,
with recurrent ascites being one cause of late
morbidity.1 Ascites
associated with heart failure or liver failure is usually characterized
by a serum-ascites albumin gradient
(SAAG)≥1.1.2 However, we
have observed multiple instances of ascites in Fontan patients with low
SAAG, suggesting a possible inflammatory process. We present three such
cases in which recalcitrant ascites severely and adversely impacted
quality of life, and describe our initial experience with
intraperitoneal corticosteroids in this setting.