Functional status in adolescents and adults with Fontan circulation

We determined functional status in adolescents and adults with Fontan circulation. Functional status was studied in 25 patients surviving more than 2 years after the definitive procedure and currently no younger than 18 years old. Age at operation was 2 to 44 years old, and follow-up was 12 +/- 5 years. To achieve Fontan circulation, atriopulmonary connection was used in 14 patients, and total cavopulmonary connection in 11 patients. One patient undergoing atriopulmonary connection died suddenly 6 years after the Fontan procedure due to pulmonary thromboembolism. New York heart association functional status was class I in 23, and class II in 2, at the latest follow-up. Catheterization done 6.5 +/- 6.8 years after the Fontan procedure showed that systemic venous pressure was statistically higher (p = 0.019) in the atriopulmonary connection group (13 +/- 3 mmHg) than in the total cavopulmonary connection group (10 +/- 3 mmHg). Exercise tests in 19 patients showed reduced tolerance in all, with maximal oxygen intake being 24.4 +/- 5.1 ml/kg/min. Serum glutamic oxaloacetic transaminase and glutamic pyruvic transaminase were elevated above normal in 11 (44%). Arrhythmia was noted over longer terms in 4 patients undergoing atriopulmonary connection; in 3, atriopulmonary connection was converted to total cavopulmonary connection, and surgical intervention for atrial arrhythmia was successful. Although functional status in adolescents and adults with Fontan circulation was good, arrhythmia and liver dysfunction in such subjects could lead to morbidity.