Journal of Chest Surgery

The Korean Society for Thoracic and Cardiovascular Surgery (대한심장혈관흉부외과학회)
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Early and Mid-term Results for Repair of Ebstein's Anomaly

엡스타인 기형에 대한 수술적 치료의 조기 및 중기 결과

  • Lee Chang-Ha (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Hwang Seong Wook (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Lim Hong Gook (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Kim Woong-Han (Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul National University College of Medicine) ;
  • Kim Chong Whan (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Lee Cheul (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute)
  • 이창하 (세종병원 흉부외과 세종심장연구소) ;
  • 황성욱 (세종병원 흉부외과 세종심장연구소) ;
  • 임홍국 (세종병원 흉부외과 세종심장연구소) ;
  • 김웅한 (서울대학교병원 흉부외과, 서울대학교 의과대학 흉부외과학교실) ;
  • 김종환 (세종병원 흉부외과 세종심장연구소) ;
  • 이철 (세종병원 흉부외과 세종심장연구소)
  • Published : 2005.04.01
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Abstract

We evaluated the early and mid-term results for repair of Ebstein's anomaly. Material and Method: Between January 1989 and June 2004, 29 patients underwent repair of Ebstein's anomaly. The median age was 11.4 years (4 days-50 years). Tricuspid insufficiency equal to or greater than grade 3 was present in 21 $(72.4\%)$ patients. Surgical techniques included tricuspid valve repair with vertical plication of the atrialized ventricle (n=14), Carpentier's technique (n=7), tricuspid valve replacement (n=4), systemic-to-pulmonary arterial shunt (n=2), tricuspid valve repair (n=1), and Fontan operation (n=1). Bi-directional cavopulmonary shunt (BCPS) was required in 5 patients. Among the 2 neonates, one patient underwent successful biventricular repair, and the other patient underwent systemic-to-pulmonary arterial shunt. Follow-up was possible in 21 patients $(75\%)$, and the average follow-up was 37.6 months (3 months-11.3 years). Result: There were $1(3.4\%)$ early and 1 late deaths. Reoperation was required in 4 patients. Two patients underwent tricuspid valve re-replacement, and the other 2 tricuspid valve repair. At recent follow-up, only 2 patients showed tricuspid insufficiency equal to or greater than grade 3, and most patients showed clinical improvement. Excluding the patients who underwent tricuspid valve replacement, the actuarial rate of freedom from reoperation at 1 and 5 years were $94.7\%\;and\;79.0\%$, respectively. Conclusion: Tricuspid valve repair was possible in most patients with good mid-term outcome. Most patients showed clinical and hemodynamic improvement. Indications for the BCPS should be clarified.

본 연구에서는 엡스타인 기형에 대한 수술적 치료의 조기 및 중기 성적에 대하여 알아보고자 하였다. 대상 및 방법 1989년 1월부터 2004년 6월까지 총 29명의 환자들을 대상으로 하였다 중심 연령은 11.4세(4일$\~$50세)이었다. Grade 3 이상의 삼첨판막 폐쇄부전을 동반한 환자는 21명$(72.4\%)$이었다. 수술 방법은 삼첨판막 성형술 및 주름성형술 14예, Carpentier성형술 7예, 삼첨판막 치환술 4예, 체폐동맥 단락술 2예, 삼첨판막 성형술 1예, Fontan수술 1예이었으며, 양방향성 상대정맥-폐동맥 단락술이 5예에서 추가적으로 시행되었다. 신생아기에 수술을 받은 2명 중 1명은 양심실 교정을 받았고, 또 다른 1명은 체폐동맥 단락술을 시행 받았다. 조기 사망례를 제외한 28명의 환자들 중 21명에서 추적관찰이 가능하였으며$(75\%)$, 평균 추적기간은 37.6개월(3개월$\~$11.3년)이었다. 결론: 1명$(3.4\%)$의 조기 사망과 1명의 만기 사망이 발생하였다. 4명의 환자들에서 재수술이 시행되었다. 2명은 삼첨판막 재치환술을 시행 받았고, 2명은 삼첨판막 재성형술을 받았다. 추적관찰이 가능했던 21명의 환자중 grade 3 이상의 삼첨판막 폐쇄부전을 보인 환자는 2명이었으며, 대부분의 환자에서 현저한 증상의 개선을 보였다. 삼첨판막 치환술을 시행 받은 환자들을 제외하고 Kaplan-Meier 방법으로 산출한 삼첨판막과 관련한 재수술이 없을 확률은 1년 및 5년에 각각 $94.7\%,\;79.0\%$이었다. 결론: 대부분의 환자에서 판막 성형술이 가능하였으며, 이의 중기 성적은 비교적 양호하였으나 장기간의 추적관찰이 요구된다. 대부분의 환자에서 현저한 증상 및 혈역학적 개선을 보였다. 다섯 명의 환자들$(17\%)$에서 양방향성 상대정맥-폐동맥 단락술이 시행되었으며, 이의 적응증에 대한 추가적인 연구 및 추적 관찰이 필요하리라 생각한다.

Keywords

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