Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Long term prognosis of patients who had a Fontan operation

폰탄 수술을 받은 환아들의 장기적 예후

  • Kim, Hyun-Jung (Department of Pediatrics, Seoul National university Children's Hospital) ;
  • Bae, Eun-Jung (Department of Pediatrics, Seoul National university Children's Hospital) ;
  • Noh, Jung-Il (Department of Pediatrics, Seoul National university Children's Hospital) ;
  • Choi, Jung-Yun (Department of Pediatrics, Seoul National university Children's Hospital) ;
  • Yun, Yong-Su (Department of Pediatrics, Seoul National university Children's Hospital) ;
  • Kim, Wong-Hwan (Department of Thoracic Surgery, Seoul National university Children's Hospital) ;
  • Lee, Jung-Yeul (Department of Thoracic Surgery, Seoul National university Children's Hospital) ;
  • Kim, Yong-Jin (Department of Pediatrics, Seoul National university Children's Hospital)
  • 김현정 (서울대학교 의과대학 소아과학교실) ;
  • 배은정 (서울대학교 의과대학 소아과학교실) ;
  • 노정일 (서울대학교 의과대학 소아과학교실) ;
  • 최정연 (서울대학교 의과대학 소아과학교실) ;
  • 윤용수 (서울대학교 의과대학 소아과학교실) ;
  • 김웅한 (서울대학교 의과대학 흉부외과) ;
  • 이정렬 (서울대학교 의과대학 흉부외과) ;
  • 김용진 (서울대학교 의과대학 소아과학교실)
  • Received : 2006.08.31
  • Accepted : 2006.10.02
  • Published : 2007.01.15
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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 $8.3{\pm}5.3years$ (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 $12.8{\pm}3.6years$ 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 $8.4{\pm}5.6years$. 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.

목 적 : 폰탄 술식은 술전 환아관리 및 수술 적응증의 세분화, 수술 방법과 술후 관리의 발전에 힘입어 조기 생존율이 향상되고 있으나 추적 관찰 기간이 길어질수록 후기 합병증이 점차 대두되고 있다. 본 논문에서는 후기 합병증의 위험요인을 분석하고 현재까지 추적 관찰되고 있는 환아들의 임상경과를 알아보고자 하였다. 방 법 : 1986년부터 2000년까지 폰탄 수술을 받은 환아 302명(수술 연령; 3.5세(5개월-15세))을 대상으로 하여 후향적 분석을 하였다. 결 과 : 폰탄 수술을 받을 당시의 나이는 5개월에서부터 15세 사이로 평균 3.5세, 중앙값 2.4세였다. 수술방법은 Lateral tunnel (LT) 폰탄 154명, Atriopulmonary connection(APC) 폰탄 107명, Extracardiac(EC) 폰탄 37명이었다. 추적 관찰 기간은 3년 5개월에서 18년 사이로 평균 추적 관찰 기간은 수술 후 8.3년이었으며 조기 사망을 제외한 241명의 5년 생존율은 91.6%, 10년 생존율은 87.7%였다. 후기 사망의 원인으로는 심혈관 연관성이 55.5%로 가장 많았으며 급사가 22.2%이었다. 심방성 빈맥의 발생빈도는 11.2%였고 폰탄 수술 후 평균 $8.4{\pm}5.6$년 후 빈맥이 발생하였다. 심방성 부정맥은 혈전동반 유무와 수술방법이 유의하게 영향을 미쳤다. 혈전 발생은 9.3%의 환자에서 관찰되었으며 수술 후 발생한 서맥이 관련 있는 요인으로 서맥이 발생하지 않은 환아에 비해 혈전이 생길 위험도를 9.9배 높였다. 폰탄 수술 후 발생한 서맥 발생 환자의 빈도는 7.4%로 완전방실차단 8명, 동결절 기능 장애 8명이었다. 완전방실차단이 발생한 환아 모두 심박동기를 부착하였는데 폰탄 수술과 직접적인 연관성은 없었다. 혈전이 발생한 환아가 발생하지 않은 환아에 비해 서맥이 생길 위험도는 9.1배 높았다. 단백소실성 장증은 10명(4.6%)에서 폰탄 수술 후 평균 6.5년(1-12.9년)이 지나서 발생하였으며 수술 당시 나이가 많을수록 발생할 위험도는 2.3배 높았다. 폰탄 전환술을 시행한 환아는 총 19명이었으며, 적응증으로는 심방성 부정맥, 판막 역류, 혈전, 단백 소실성 장증이었다. 조기 사망한 2명 모두 단백소실성 장증이 동반되어 있었다. 결 론 : 폰탄 수술의 후기 생존율은 받아들일만큼 향상되었다. 그러나 생존해 있는 환아들의 상당수에서 부정맥, 혈전, 단백소실성 장증, 급사, 판막역류 그리고 심부전 등의 후기 합병증이 관찰되어 이들 환아들에 대한 철저한 추적 관찰과 조기 치료가 필요하다.

Keywords

References

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