Journal of Chest Surgery

The Korean Society for Thoracic and Cardiovascular Surgery (대한심장혈관흉부외과학회)
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Right Ventricle Exclusion in Severe Neonatal Ebstein's Anomaly

증상이 심한 신생아 엡스타인 기형에서의 우심실 제외

  • Min, Sun-Kyung (Department of Thoracic and Cardiovascular Surgery, Seoul Paik Hospital, Inje University School of Medicine) ;
  • Kim, Woong-Han (Department of Thoracic and Cardiovascular Surgery, College of Medicine, Seoul National University) ;
  • Lee, Young-Ok (Department of Thoracic and Cardiovascular Surgery, College of Medicine, Seoul National University) ;
  • Seong, Yong-Won (Department of Thoracic and Cardiovascular Surgery, College of Medicine, Seoul National University) ;
  • Park, Sung-Joon (Department of Thoracic and Cardiovascular Surgery, College of Medicine, Seoul National University) ;
  • Choi, Jin-Ho (Department of Thoracic and Cardiovascular Surgery, College of Medicine, Seoul National University)
  • 민선경 (인제대학교 서울백병원 흉부외과) ;
  • 김웅한 (서울대학교 의과대학 흉부외과학교실) ;
  • 이영옥 (서울대학교 의과대학 흉부외과학교실) ;
  • 성용원 (서울대학교 의과대학 흉부외과학교실) ;
  • 박성준 (서울대학교 의과대학 흉부외과학교실) ;
  • 최진호 (서울대학교 의과대학 흉부외과학교실)
  • Received : 2010.04.26
  • Accepted : 20201615
  • Published : 2010.10.05
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Abstract

A one-day-old baby was transferred for cyanosis and heart murmur. Echocardiographic evaluation revealed that he had severe neonatal Ebstein's anomaly (Carpentier type C), pulmonary atresia, and pulmonary circulation via patent ductus arteriosus. Because the wall of the atrialized right ventricle was very thin, showed decreased contractility, and the small right ventricle showed pulmonary atresia, we decided that a two-ventricular repair was impossible. When the patient was one-month-old, he underwent right atrium reduction-plasty, a right ventricular exclusion procedure (including atrialized right ventricle resection and functional right ventricle plication), and right modified Blalock-Taussig shunt. He was discharged without specific problems. He received a bidirectional cavopulmonary shunt successfully at 4 months later.

생후 1일된 환아가 청색증과 심잡음을 주소로 전원되었다. 심장 초음파 검사를 시행한 결과, 폐동맥 폐쇄가 동반되어 동맥관 의존성 폐혈류를 보이는 증상이 심한 엡스타인 기형(Carpentier type C)이었다. 심방화된 우심실의 벽은 매우 얇고 수축력이 저하되어 있었으며 기능적 우심실의 크기가 매우 작고 폐동맥 폐쇄가 동반되어 양심실 교정이 불가능하다고 판단하였다. 생후 1개월 째 우심방 절제 성형술, 심방화된 우심실의 광범위한 절제 후 봉합 폐색, 변형 Blalock-Taussig 단락술을 시행하였다. 환아는 별다른 문제 없이 퇴원하였으며 생후 5개월에 양방향성 상대정맥-폐동맥 단락술을 시행받았다. 저자 등은 심한 증상을 나타내는 신생아 엡스타인 기형에서 우심실 제외 수술을 시행하여 좋은 결과를 얻었기에 보고하는 바이다.

Keywords

References

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