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Long term prognosis of patients who had a Fontan operation

Korean Journal of Pediatrics 2007;50(1):40-46.
Published online January 15, 2007.
Long term prognosis of patients who had a Fontan operation
Hyun-Jung Kim1, Eun-Jung Bae1, Jung-Il Noh1, Jung-Yun Choi1, Yong-Su Yun1, Wong-Hwan Kim2, Jung-Yeul Lee2, Yong-Jin Kim1
1Department of Pediatrics , Seoul National University Children's Hospital, Seoul, Korea
2Department of Thoracic Surgery, Seoul National University Children's Hospital, Seoul, Korea
폰탄 수술을 받은 환아들의 장기적 예후
김현정1, 배은정1, 노정일1, 최정연1, 윤용수1, 김웅한2, 이정렬2, 김용진1
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 흉부외과
Correspondence: 
Eun-Jung Bae, Email: eunibae@plaza.snu.ac.kr
Abstract
Purpose
: This study assessed the long term survival rate and long term complications of patients who had a modified Fontan operation for functionally univentricular cardiac anomaly.
Methods
: Between June 1986 and December 2000, 302 patients with a functional single ventricle underwent surgical interventions and were followed up until February 2006. The mean follow-up period was 8.3?.3 years (range 3.5-18 years). Their median age was 2.4 years at the Fontan operation. The survival rate, the incidence and the risk factor of late complications were evaluated retrospectively.
Results
: The verall survival rate was 91 percent at 5 years and 87 percent at 10 years. In multivariate analysis, early calendar year of operation and significant regurgitation were risk factors of death. The surviving patients showed NYHA functional class I in 82 percent, class II in 15 percent, and class III in 3 percent. Redo Fontan operations were necessary in 8.8 percent of patients at average 12.8?.6 years after initial Fontan operation. The most common cause of Fontan conversion was atrial arrhythmia. The incidence of thromboembolic events was 9.3% and these complications were associated with the occurrence of atrial tachyarrhythmia. Supraventricular tachycardia including atrial flutter or fibrillation were reported on the follow-up examination by 11.2 percent of survivors after 8.4?.6 years. Atriopulmonary connection showed higher rates of late tachycardia than lateral tunnel operation.
Conclusions
: This study revealed that the recent survival rate of Fontan type operation was satisfactory, but the occurrence of late complications after a Fontan type operation increased with the longer survival. There is a need for strict follow up and early treatment of late complications in patients who had a Fontan operation.
Key Words: Fontan type operation , Late complication , Risk factors


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