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Functional status in adolescents and adults with Fontan circulation

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Abstract

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 mm Hg). 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 connections; 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.

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Authors and Affiliations

  1. Department of Cardiovascular Surgery and Paediatrics, National Cardiovascular Center, Osaka, Japan

    Yoshiro Yoshikawa, Hideki Uemura, Toshikatsu Yagihara, Youichi Kawahira, Hideo Ohuchi & Soichiro Kitamura

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  1. Yoshiro Yoshikawa
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  2. Hideki Uemura
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  3. Toshikatsu Yagihara
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  4. Youichi Kawahira
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  5. Hideo Ohuchi
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  6. Soichiro Kitamura
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Additional information

Read at the 13th Annual Meeting of the European Association for Cardio-Thoracic Surgery, Glasgow, Scotland, September 5–8, 1999.

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Yoshikawa, Y., Uemura, H., Yagihara, T. et al. Functional status in adolescents and adults with Fontan circulation. Jpn J Thorac Cardiovasc Surg 50, 141–145 (2002). https://doi.org/10.1007/BF02913194

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  • DOI: https://doi.org/10.1007/BF02913194

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