Background: During the 1970s (1973-1979), 41 patients with tricuspid atresia and 25 patients with double-inlet left ventricle had a Fontan procedure performed at the Mayo Clinic. In the tricuspid atresia group, there were seven hospital deaths and six late deaths, leaving 28 survivors 11-16 years after surgery. In the double-inlet left ventricle group, there were six hospital deaths and six late deaths, leaving 13 survivors 11-13 years after surgery.
Methods and results: The present status of 38 of the 41 long-term survivors was recently (September 1990) determined. Of these 38 patients contacted, 34 felt they were in excellent or good condition, three considered themselves in fair condition, and one was in poor condition. Nineteen of the 38 (50%) were receiving no cardiovascular medications. Twenty-eight (76%) were employed full-time or were full-time students with three others working part-time. Only four patients (11%) felt physically incapable of working or attending school. Seven patients have graduated from college since their Fontan procedure with three others presently enrolled. Present occupations include architect, lawyer, industrial manager with a master's degree, policeman, truck driver, horseback riding instructor, and artist. Nine patients are married, and one woman tolerated a pregnancy without difficulty. The most troublesome late problems requiring medical therapy were atrial dysrhythmia in seven patients (18%) and protein-losing enteropathy in two patients (5%).
Conclusions: Surgical mortality of the Fontan procedure for tricuspid atresia and double-inlet left ventricle is now less than 10%, and that, plus these late results, justify continued application of this operation in patients with these lesions.