Functional status in adolescents and adults with Fontan circulation

Jpn J Thorac Cardiovasc Surg. 2002 Apr;50(4):141-5. doi: 10.1007/BF02913194.


Objective: We determined functional status in adolescents and adults with Fontan circulation.

Methods: 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.

Results: 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.

Conclusion: Although functional status in adolescents and adults with Fontan circulation was good, arrhythmia and liver dysfunction in such subjects could lead to morbidity.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Arrhythmias, Cardiac / physiopathology*
  • Child
  • Child, Preschool
  • Follow-Up Studies
  • Fontan Procedure*
  • Health Status
  • Humans
  • Liver Diseases / physiopathology*
  • Postoperative Complications / physiopathology*
  • Thrombosis / physiopathology