Protein-losing enteropathy after Fontan operation for tricuspid atresia (imperforate tricuspid valve).
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.
D001022:Aortic Valve Insufficiency / Q000209:etiology; D006332:Cardiomegaly / Q000150:complications; D002648:Child; D005260:Female; D006343:Heart Septal Defects / Q000150:complications / Q000601:surgery; D006350:Heart Valve Prosthesis; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D008722:Methods; D011183:Postoperative Complications; D011504:Protein-Losing Enteropathies / Q000209:etiology*; D011859:Radiography; D014261:Tricuspid Valve / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*