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12세 여아에서 운동 중 발생한 흉통 및 실신 - 왼쪽 주 관상동맥의 이상 기시의 진단 및 수술적 치료 1례

Anomalous origin of left coronary artery arising from the right coronary cusp presenting with chest discomfort and syncope on physical exercise

  • 백란 (연세대학교 의과대학 소아과학교실 소아심장과) ;
  • 김남균 (연세대학교 의과대학 소아과학교실 소아심장과) ;
  • 박한기 (연세대학교 의과대학 흉부외과학교실 심장혈관외과) ;
  • 박영환 (연세대학교 의과대학 흉부외과학교실 심장혈관외과) ;
  • 유병원 (연세대학교 의과대학 소아과학교실 소아심장과) ;
  • 최재영 (연세대학교 의과대학 소아과학교실 소아심장과)
  • Baik, Ran (Division of Pediatric Cardiology, Severance Cardiovascular Hospital, Yonsei University Health System) ;
  • Kim, Nam Kyun (Division of Pediatric Cardiology, Severance Cardiovascular Hospital, Yonsei University Health System) ;
  • Park, Han Ki (Division of Cardiovascular Surgery, Severance Cardiovascular Hospital, Yonsei University Health System) ;
  • Park, Young Hwan (Division of Cardiovascular Surgery, Severance Cardiovascular Hospital, Yonsei University Health System) ;
  • Yoo, Byung Won (Division of Pediatric Cardiology, Severance Cardiovascular Hospital, Yonsei University Health System) ;
  • Choi, Jae Young (Division of Pediatric Cardiology, Severance Cardiovascular Hospital, Yonsei University Health System)
  • 투고 : 2009.08.31
  • 심사 : 2009.09.21
  • 발행 : 2010.02.15

초록

관상동맥의 이상 기시는 소아에서 드문 질환이다. 이러한 이상 기시들은 대동맥과 폐동맥간과의 해부학적인 관계에 의해 3가지 형태로 분류할 수 있다. 우리 환자의 경우와 같이 기형 동맥이 대동맥과 폐동맥사이로 주행하는 경우 젊은 연령에서 급사의 위험도가 증가하기 때문에 빠른 진단과 치료가 필요하다. 관상동맥의 이상 기시를 교정하는 방법으로는 잘 알려진 re-implantation, 관상동맥 우회술과 unroofing의 세가지 방식이 있고, 아직까지 좌 관상동맥의 이상 기시의 치료에 대해서는 많은 견해들이 있으나 일단 수술적 교정이 되면 좋은 결과를 보인다. 우리는 운동 중 발생한 흉통과 실신을 주소로 내원한 12세 여아에서 심초음파와 관상동맥 전상화 단층촬영을 통하여 좌 관상동맥의 우 관상동맥동으로부터의 이상 기시를 진단받고 re-implantation, 관상동맥 우회술, 그리고 unroofig 방법을 통해 치료받은 1례를 경험하여 이를 보고하고자 한다.

Anomalous origins of coronary arteries are a rare type of disease among children. These anomalies can be categorized into 3 types according to the anatomical relationship of the aorta and pulmonary trunks. Among these types, the interarterial type, as observed in our case, needs early diagnosis and treatment, because it can increase the risk for the patient, causing sudden cardiac death in young individuals. Although there are controversies concerning the management of anomalous origins of the left coronary artery (LCA) in children, the result can be very beneficial, if treated accurately. Three well-known methods for correction of anomalous origins of LCA are re-implantation, coronary arterial bypass grafting (CABG), and unroofing. We report on the case of a 12-year-old girl who had chest discomfort and syncope with physical exercise and was later diagnosed with an anomalous origin of LCA by transthoracic echocardiography (TTE) and heart computed tomography (CT). She underwent a corrective operation by re-implantation, CABG, and unroofing.

키워드

참고문헌

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피인용 문헌

  1. Coronary Artery Anomalies: Current Recognition and Treatment Strategies. Update on Recent Progress vol.8, pp.9, 2014, https://doi.org/10.1007/s12170-014-0395-4
  2. Sudden Cardiac Death Associated with Anomalous Origin of the Left Main Coronary Artery from the Right Sinus, with an Intramural Course vol.42, pp.6, 2010, https://doi.org/10.14503/thij-14-4249