Long-term results after the Fontan operation for tricuspid atresia

Between 1968 and 1977, 32 patients who underwent the Fontan operation for tricuspid atresia at Bordeaux, France, or Leiden, The Netherlands, survived at least 1 year after surgery. These patients were evaluated clinically 7 to 16 years (mean 8.9 years) after surgery by review of clinical records, questionnaire, or direct examination. There were five deaths: one during arrhythmia, the second sudden and unexplained, and three others after reoperation. Four of the five patients had evidence of obstruction of the atriopulmonary or atrioventricular conduit. There were eight reoperations. Two were for residual atrial septal defects, one for an atrial septal defect and an intrapulmonary arteriovenous fistula, and one for a residual ventricular septal defect. The other four were reoperated for severe conduit or homograft obstruction. One of these four also had a residual shunt, severe mitral regurgitation, and reduced left ventricular function. One patient was found to have left ventricular dysfunction 3 months after surgery, which persisted. Another patient had left ventricular dysfunction unmasked after successful mitral annuloplasty for severe mitral regurgitation. No other patients had clinical evidence of left ventricular dysfunction. Recurrent supraventricular tachycardia occurred in four patients and was eventually fatal in one patient with associated conduit obstruction. Of the 27 survivors, 13 are in NYHA class I, 13 are in class II, and one is in class III. We conclude that clinical results of the Fontan operation remain good in most patients 7 to 16 years after surgery.(ABSTRACT TRUNCATED AT 250 WORDS)