Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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가와사끼병 영아에서 발생한 혈전을 동반한 거대관상동맥류 1례

A Case of Multiple Giant Coronary Aneurysms with Large Mural Thrombus due to Kawasaki Disease in a Young Infant

  • 최은나 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 김정태 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 김유리아 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 유병원 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 최덕영 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 최재영 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 설준희 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 이승규 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 김동수 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 박영환 (연세대학교 의과대학 심장혈관병원 심장혈관외과)
  • Choi, Eun Na (Department of Pediatrics, Cardiovascular Center, Yonsei University College of Medicine) ;
  • Kim, Jeoung Tae (Department of Pediatrics, Cardiovascular Center, Yonsei University College of Medicine) ;
  • Kim, Yuria (Department of Pediatrics, Cardiovascular Center, Yonsei University College of Medicine) ;
  • Yoo, Byung Won (Department of Pediatrics, Cardiovascular Center, Yonsei University College of Medicine) ;
  • Choi, Deok Young (Department of Pediatrics, Cardiovascular Center, Yonsei University College of Medicine) ;
  • Choi, Jae Young (Department of Pediatrics, Cardiovascular Center, Yonsei University College of Medicine) ;
  • Sul, Jun Hee (Department of Pediatrics, Cardiovascular Center, Yonsei University College of Medicine) ;
  • Lee, Sung Kye (Department of Pediatrics, Cardiovascular Center, Yonsei University College of Medicine) ;
  • Kim, Dong Soo (Department of Pediatrics, Cardiovascular Center, Yonsei University College of Medicine) ;
  • Park, Young Hwan (Department of Cardiovascular Surgery, Cardiovascular Center, Yonsei University College of Medicine)
  • 투고 : 2004.09.07
  • 심사 : 2004.10.12
  • 발행 : 2005.03.15
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초록

가와사끼병은 주로 소아연령에서 발생하는 급성 열성 혈관염으로 관상동맥의 확장이 치명적 합병증으로 나타날 수 있다. 저자들은 3개월된 영아에서 반복적인 정맥용 면역글로불린, 스테로이드, 경구용 methotrexate의 치료에도 불구하고 진행되는 관상동맥 병변으로 다발성 거대 관상류, 협착 및 관상동맥 내 혈전을 보이며 심전도상 심근 허혈 소견과 함께 심장 자기공명영상에서 좌심실의 확장과 전벽 및 중격의 심한 운동저하를 나타낸 증례를 보고한다. 환아는 보존적 요법에 반응하지 않는 관상동맥병변으로 내원 88일째 수술적으로 우측 거대관상동맥류를 절개하고 Gore-tex tube를 삽입하여 각각의 측부순환과 연결하였으며, 좌측 관상동맥 협착부위는 세로로 절개한 후 심장막 반(pericardial patch)을 삽입, 확장하였으나, 수술 후 심폐부전으로 사망하였다.

Kawasaki disease is an acute systemic vasculitis of unknown origin. Giant coronary aneurysm is one of the most serious complications, although peripheral artery vasculitis can produce life-threatening events. Myocardial ischemia and infarction can be caused by coronary artery stenosis, aneurysm, and stagnation of blood flow in coronary arteries which triggers thromboembolism. Atypical presentation in young infants often interferes with prompt diagnosis and timely treatment, resulting in poor outcomes. We describe a 3-month-old infant with multiple giant coronary aneurysms with flow stagnation, stenosis and large mural thrombus due to Kawasaki disease. He presented with a prolonged course of severe coronary involvement in spite of all measures to reduce coronary complications. Finally, surgical intervention was tried because of the worsening coronary artery abnormalities. The patient died of acute cardiorespiratory failure shortly after weaning from cardiopulmonary bypass.

키워드

참고문헌

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