Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Late aortic root dilatation and aortic regurgitation in repaired tetralogy of fallot

팔로 4징의 완전 교정술 후 장기 추적 관찰상 나타나는 대동맥근의 확장과 대동맥판 폐쇄부전

  • Kim, Jeong Eun (Division of Pediatric Cardiology, Yonsei Cardiovascular Center Yonsei University College of Medicine) ;
  • Hur, Kyong (Division of Pediatric Cardiology, Yonsei Cardiovascular Center Yonsei University College of Medicine) ;
  • Kwon, Hae Sik (Division of Pediatric Cardiology, Yonsei Cardiovascular Center Yonsei University College of Medicine) ;
  • Yoo, Byung Won (Division of Pediatric Cardiology, Yonsei Cardiovascular Center Yonsei University College of Medicine) ;
  • Choi, Jae Young (Division of Pediatric Cardiology, Yonsei Cardiovascular Center Yonsei University College of Medicine) ;
  • Sul, Jun Hee (Division of Pediatric Cardiology, Yonsei Cardiovascular Center Yonsei University College of Medicine)
  • 김정은 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 허경 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 권해식 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 유병원 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 최재영 (연세대학교 의과대학 심장혈관병원 소아심장과) ;
  • 설준희 (연세대학교 의과대학 심장혈관병원 소아심장과)
  • Received : 2007.06.18
  • Accepted : 2007.08.16
  • Published : 2007.10.15
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Abstract

Purpose : Aortic valve or aortic root (AoRo) replacement is occasionally required because of AoRo dilatation and aortic regurgitation (AR) in repaired tetralogy of Fallot (TOF). We evaluated AoRo size and possible factors associated with its hemodynamic nature in patients with repaired TOF. Methods : We investigated 130 repaired TOF patients more than 15 years of age who followed-up by echocardiography from January 2002 to December 2003. Of 130 patients, we identified 17 patients with AoRo dilatation, which was defined as ratio of expected AoRo size by standard nomogram (AoRo ratio) >1.5 (dilator group), and 113 TOF controls, with AoRo ratio <1.5 (non-dilator group). Results : Mean indexed AoRo size ($mm/m^2$) in the first echo was $24{\pm}3.2$ in the dilator group and $18{\pm}3.4$ in the non-dilator group (P<0.0001). AoRo rate of change (mm/year) from the first to latest echo study was $1.6{\pm}3.8$ in dilator group and $0.05{\pm}1.6$ in the non dilator group (P=0.0021). Patients from the dilator group showed a higher prevalence of pulmonary atresia (P=0.031) and a history of aortopulmonary shunt before repair (P=0.048), moderate to severe AR (P=0.0065), and increased left ventricular end-diastolic dimensions (P=0.003). Conclusions : A subset of patients late after TOF repair may show progressive dilatation of AoRo. To identify and prevent long-term sequelae in this patient group, regular follow-up and speculation about AoRo after TOF repair is recommended.

목 적 : 팔로 4징 교정술 후 만기 추적 관찰 중인 청소년 및 성인환자에서 대동맥근 확장정도를 평가하고, 이를 통해 장기 추적 관찰 상 발생하는 대동맥근 확장과 대동맥판 폐쇄 부전의 관련 요인 및 환자군의 특성에 대해 알아보고자 하였다. 방 법 : 본원에서 활로 4징의 완전 교정술을 받은 후 장기 추적 관찰중인 15세 이상의 청소년 및 성인 총 130명의 환자를 대상으로 대동맥근 직경의 측정치와 기대치의 비가 1.5이상인 대동맥근 확장군(n=17)과 비확장군(n=113)으로 분류하여 임상적 특성과 교정술 시행 후 얻을 수 있는 첫 심초음파 검사 소견과 최근 추적관찰상의 대동맥근 직경 및 변화율의 차이를 조사하였다. 결 과 : 활로 4징의 완전 교정술을 받은 총 130명의 환자들 중 대동맥근 확장군은 17명(13%), 대동맥근 비확장군은 113명이었다. 동반된 폐동맥판 폐쇄의 빈도와 체폐 단락술의 백분율은 대동맥근 확장군에서 더 높았으나 교정술시의 연령, 추적관찰기간, 고식술과 교정술사이의 시간간격, 우측 대동맥궁의 빈도는 두 군 간의 유의한 통계적 차이는 없었다. 진단 당시와 최근의 대동맥근 직경 지수와 대동맥근 직경의 측정치/기대치 비는 모두 대동맥근 확장군에서 높았다. 또한 대동맥근 확장군에서 대동맥근 직경과 대동맥근 직경 지수의 변화율이 높았고 중등도 이상의 대동맥판 폐쇄부전이 더 많았으며 좌심실 확장기말 용적도 더 컸다. 결 론 : 팔로 4징의 교정술 후 장기 추적 관찰에 있어 일부 환자에서는 진행적인 대동맥 확장과 대동맥판 폐쇄 부전이 나타날 수 있어, 과거에 교정술을 시행받고 장기 추적 관찰 중인 성인 환자에서 더욱 중요하게 검토해야 할 요인으로 생각된다.

Keywords

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