Summary
Protein-losing enteropathy occurred in a 7-year-old girl with tricuspid atresia, concordant
ventriculo-arterial connexions and a relatively large hypoplastic right ventricle,
one year after an atrio-ventricular type of Fontan operation by means of a valveless
woven Dacron conduit. Severe conduit regurgitation and a marked enlargement of the
hypoplastic right ventricle were demonstrated at recatheterization. Insertion of a
bioprosthetic valve at the base of the right atrial appendage led to a dramatic clinical
recovery of the patient.
The use of a valved conduit is recommended when an atrioventricular type of Fontan
repair is planned in patients with tricuspid atresia, concordant ventriculo-arterial
connexions and relatively large hypoplastic right ventricle.
Key words
Protein-losing enteropathy - Fontan operation - Hypoplastic right ventricle - Conduit
regurgitation
