Protein-losing enteropathy after Fontan operation for tricuspid atresia (imperforate tricuspid valve)

Thorac Cardiovasc Surg. 1980 Oct;28(5):359-63. doi: 10.1055/s-2007-1022109.


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.

Publication types

  • Case Reports

MeSH terms

  • Aortic Valve Insufficiency / etiology
  • Cardiomegaly / complications
  • Child
  • Female
  • Heart Septal Defects / complications
  • Heart Septal Defects / surgery
  • Heart Valve Prosthesis
  • Heart Ventricles / diagnostic imaging
  • Heart Ventricles / surgery
  • Humans
  • Methods
  • Postoperative Complications*
  • Protein-Losing Enteropathies / etiology*
  • Radiography
  • Tricuspid Valve / abnormalities
  • Tricuspid Valve / diagnostic imaging
  • Tricuspid Valve / surgery*