Comment
Patients with a Fontan circulation are at risk for both liver disease and congestive heart failure. Ascites is one complication of this that is seen in roughly one-third of adults post-Fontan and can itself adversely affect Fontan hemodynamics.1,3Recurrent ascites poses a management challenge due to limited efficacy of diuretic therapy. In some patients, repeated paracentesis may become necessary but comes with a risk for complications including protein depletion, hypovolemia, electrolyte abnormalities, infection, and bleeding.
Ascites related to heart failure or liver disease is typically expected to have a SAAG≄1.1. In cases where SAAG is low, an alternative or coexisting process should be considered. Chylous ascites has been described in congenital heart disease and can be associated with low SAAG, but can be recognized by high levels of fluid triglyceride and white blood cells with a lymphocytic predominance. Several studies have also suggested that inflammation plays a role in Fontan morbidity.4,5
Having excluded other etiologies for ascites in our patients and given the poor prognosis associated with intractable ascites, we felt it appropriate to consider a novel intervention targeting inflammation. Intraperitoneal triamcinolone has been described for ascites related to lupus, hemodialysis, and malignancy.6-11 We speculated that intraperitoneal administration of a large-molecule steroid would allow a higher dose of steroid to the vessels responsible for the ascites while limiting systemic absorption.
In each case, triamcinolone hexacetonide was administered following large-volume paracentesis. Sometimes a second dose was given after the subsequent paracentesis. Patients 1 and 3 tolerated triamcinolone administration without clinical evidence of systemic steroid absorption, and the rate of ascites accumulation appeared to decrease (Table 1). While Patient 1 died shortly afterward from presumed arrhythmic arrest, Patient 3 experienced complete resolution of ascites until her death from unrelated causes over a year later. Patient 2 also experienced a decrease in the rate of ascites accumulation after intraperitoneal triamcinolone, but due to adverse effects, it was not felt to be an option when her ascites recurred. She eventually died from complications of frequent paracentesis.
Given the lack of efficacy of diuretics in this clinical situation and the risks associated with frequent paracentesis, we feel that further studies exploring the efficacy and toxicity of intraperitoneal steroids in Fontan patients with low-SAAG ascites using objective metrics would be of great interest to physicians caring for this unique population.