Dysplasia of AV valve in complete AV canal with tetralogy of Fallot: surgical repair.

A five-year-old girl underwent surgery for tetralogy of Fallot and complete persistent atrioventricular (AV) canal. At operation, severe dysplasia of the right side of the common AV valve was found, with an accessory orifice and muscularization of a leaflet. Repair was carried out by closing the AV defect, through a combined right atrial and right ventricular approach, by a pericardial patch with a large anterior extension. Closure of the resulting mitral cleft, resection of anomalous muscle bundles and pulmonary valvotomy were also performed. Postoperatively the patient presented severe tricuspid regurgitation. Repair of the valve was deemed impossible and valve replacement was considered to have a very high risk due to the age of the patient, the small size of the right ventricle, and the previous repair of the AV defect. A modified Fontan operation was, therefore, considered preferable and successfully carried out. After the second operation, mediastinal infection developed and was successfully treated by local irrigation of diluted providone-iodine solution.