Interrupted Aortic Arch with Apical Muscular Ventricular Septal Defect Associating Esophageal Atresia with Tracheoesophageal Fistula

식도폐쇄 및 기관식도루를 동반한 심첨부 근육성 심실 중격 결손과 대동맥궁 단절 -1예 보고-

  • 조정수 (부산대학교 의과대학 흉부외과학교실) ;
  • 이형두 (부산대학교 의과대학 소아과학교실)
  • Published : 2004.10.01

Abstract

Interrupted aortic arch with concomitant intracardiac defects is a rare congenital anomaly that has an unfavorable natural course. We report a successful staged operation of interrupted aortic arch with apical muscular ventricular septal defect associating esophageal atresia with tracheoesophageal fistula in a 3-day-old neonate weighing 2.6 kg. We repaired esophageal atresia through the right thoracotomy and subsequently performed extended end-to-end anastomosis of the aortic arch with pulmonary artery banding through the left thoracotomy at same operation. The apical muscular VSD was repaired 87 day after first operation. The patient required multiple additional interventions before closure of the apical muscular ventricular septal defect, such as pyloromyotomy for idiopathic hypertrophic pyloric stenosis, anterior aortopexy for airway obstruction, and balloon aortoplasty for residual coarctation. She is now doing well.

심내기형을 동반한 대동맥궁 단절은 매우 중한 자연경과를 갖고 있는 희귀한 선천성 심장 기형이다. 식도 폐쇄증과 기관 식도루를 동반한 심첨부 근육성 심실 중격 결손과 대동맥궁 단절을 생후 3일된 체중 2.6 kg의 신생아에서 단계적 수술법으로 치료한 경험을 보고한다. 1차 수술로서 우측 개흉술을 통한 식도 폐쇄증의 교정과 함께 좌측 개흉술에 의한 대동맥궁 광범위 단-단 문합술 및 폐동맥 교약술을 시행하였다. 1차 수술 후 87일째 정중 흉골 절개를 통해 심첨부 근육성 심실 중격 결손을 폐쇄하였다. 심실 중격 결손 폐쇄 전 유문부 근육절개술, 대동맥 전방고정술, 대동맥 풍선확장술 등의 추가 시술이 필요하였다. 최종 수술 후 3개월째 양호한 추적 결과를 경험하였기에 보고하는 바이다.

Keywords

References

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