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 SAAGâ„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.