Biventricular Repair of Critically III Neonate with Ebstein′s Anomaly - Report of 1 case -

증상이 심한 신생아 Ebstein 기형의 양심실성교정 -1례 보고-

  • Kong, Joon-Hyuk (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Kim, Woong-Han (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Ryu, Jae-Wook (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Lee, Seog-Ki (Department of Thoracic and Cardiovascular Surgery, College of Medicine, Chosun University) ;
  • Baek, Man-Jong (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Lim, Cheong (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Kim, Soo-Cheol (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Oh, Sam-Se (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Na, Chan-Young (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Kim, Soo-Jin (Department of Pediatric Cardiology, Sejong General Hospital, Sejong Heart Institute) ;
  • Park, Young-Kwan (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Kim, Chong-Whan (Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute)
  • 공준혁 (흉부외과, 부천세종병원, 세종심장연구소) ;
  • 김웅한 (흉부외과, 부천세종병원, 세종심장연구소) ;
  • 류재욱 (흉부외과, 부천세종병원, 세종심장연구소) ;
  • 이석기 (조선대학교 의과대학 흉부외과학 교실) ;
  • 백만종 (흉부외과, 부천세종병원, 세종심장연구소) ;
  • 임청 (흉부외과, 부천세종병원, 세종심장연구소) ;
  • 김수철 (흉부외과, 부천세종병원, 세종심장연구소) ;
  • 오삼세 (흉부외과, 부천세종병원, 세종심장연구소) ;
  • 나찬영 (흉부외과, 부천세종병원, 세종심장연구소) ;
  • 김수진 (소아과, 부천세종병원, 세종심장연구소) ;
  • 박영관 (흉부외과, 부천세종병원, 세종심장연구소) ;
  • 김종환 (흉부외과, 부천세종병원, 세종심장연구소)
  • Published : 2002.04.01

Abstract

Patients with severe Ebstein's anomaly showing in the neonatal period, represent progressive cardiac enlargement with pulmonary hypoplasia and functional pulmonary atresia with patent ductus alteriosus-dependent pulmonary circulation. Biventricular repair in these patients had been mostly unsuccessful except for Starnes' procedure that converts the anatomy to single ventricle physiology for Fontan procedure. A 4-days old male was admitted with the diagnosis of severe Ebstein's anomaly with anatomic pulmonary atresia and severe cardiac enlargement. He successfully underwent biventricular repair with vertical plication method of atrialized right ventricle, tricupid annuloplasty, transannular right ventricular outflow tract reconstrulltion, atrial septal defect patch closure with fenestration, and right atrial reduction angioplasty Postoperatively, cardiothoracic ratio was significantly reduced and mild tricuspid regurgitation was remnant in echocardiography. The patient is currently 10 months old and is fully active without restrictions.

심한 Ebstein기형이 있으면서 신생아시기에 증상이 있는 경우, 심한 심비대를 보이며 이로인해 폐발달의 장애가 생기고 기능적 폐동맥판폐쇄를 보이며, 동맥관의존 폐혈류를 보인다. 지금까지 이런 경우 양심실성교정은 실망적이었으며 근래에 와서 Fontan술식을 목표로 하는 Stames술식으로 좋은 결과를 보고하고 있다. 본원에서는 생후 4일째 심한 심비대와 함께 해부학적 폐동맥판폐쇄가 동반된 Ebstein기형의 환아에서 심방화된 우심실을 수직적 주름성형술, 삼첨판막륜성형술, 우심실유출로재건, 심방중격결손부분폐쇄, 우심방축소술로 양심실고정술을 시행하였다. 술후 환아는 심홉곽비의 현저한 감소와 심초음파검사상 경도의 삼첨판폐쇄부전을 보이며 10개월째 건강한 상태로 외래추적관찰중이다.

Keywords

References

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