Journal of Chest Surgery

The Korean Society for Thoracic and Cardiovascular Surgery (대한심장혈관흉부외과학회)
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Absent Pulmonary Valve with Intact Ventricular Septum, PDA. ASD

온전한 심실중격을 가진 폐동맥판막무형성증, 동맥관개존, 심방중격결손의 수술치험 1례

  • 유지훈 (성균관대학교 의과대학 삼성서울병원 흉부외과) ;
  • 박계현 (성균관대학교 의과대학 삼성서울병원 흉부외과) ;
  • 이영탁 (성균관대학교 의과대학 삼성서울병원 흉부외과) ;
  • 박표원 (성균관대학교 의과대학 삼성서울병원 흉부외과) ;
  • 전태국 (성균관대학교 의과대학 삼성서울병원 흉부외과)
  • Published : 2002.08.01
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Abstract

Absent pulmonary valve syndrome with intact ventricular septum(APVS with IVS) is a rare congenital anomaly. The severe form of this syndrome, characterized by severe respiratory distress presented soon after birth, has been attributed to the compression of the airways caused by aneurysmal dilatation of pulmonary artery. Several operative treatments such as pulmonary valve insertion, or reductive angioplasty of pulmonary artery have been applied. We present a 3-day-old male who showed improvement after PDA ligation, reductive angioplasty of pulmonary artery, pulmonary valve reconstruction, and ASD closure.

온전한 심실중격을 가진 폐동맥판막무형성증(absent pulmonary valve syndrome with intact ventricular septum, APVS with IVS)은 매우 드문 선천성 심장 질환이다. 신생아의 경우 확장된 폐동맥에 의한 기도폐쇄와 이로 인한 심한 호흡곤란증으로 높은 사망률을 유발한다. 이에 대한 해결책으로 여러 약물치료 및 여러 고식적 수술, 폐동맥 판막 삽입, 폐동맥 축소술(reductive angioplasty of pulmonary artery) 등이 사용되었다. 본원에서는 동맥관 결찰술, 폐동맥 축소술, 폐동맥 판막 재건술(pulmonary valve reconstruction) 및 심방중격결손 봉합술을 시행하여 좋은 결과를 얻었다.

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References

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