Aortic Root Replacement in Patient of Right Ventricular Hypoplasia with Annuloaortic Ectasia, Ventricular Septal Defect and Aortic Regurgitation - Report of 1 case -

대동맥판륜 확장증, 심실중격결손 및 대동맥판막 폐쇄부전과 동반된 우심실 저형성 환자에서 대동맥근부 치환술 -1예 보고

  • Baek, Man-Jong (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Na, Chan-Young (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Oh, Sam-Se (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Kim, Woong-Han (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Whang, Sung-Wook (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Lee, Cheol (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Chang, Yun-Hee (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Jo, Won-Min (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Kim, Jae-Hyun (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Seo, Hong-Ju (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Kang, Sang-Soo (Dept. of Anesthesiology, and Pain Clinic, College of Medicine, Hallym University) ;
  • Moon, Hyun-Soo (Dept. of Anesthesiology, and Pain Clinic, College of Medicine, Hallym University) ;
  • Park, Young-Kwan (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute) ;
  • Kim, Chong-Whan (Dept. of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute)
  • 백만종 (부천세종병원 흉부외과, 세종심장연구소) ;
  • 나찬영 (부천세종병원 흉부외과, 세종심장연구소) ;
  • 오삼세 (부천세종병원 흉부외과, 세종심장연구소) ;
  • 김웅한 (부천세종병원 흉부외과, 세종심장연구소) ;
  • 황성욱 (부천세종병원 흉부외과, 세종심장연구소) ;
  • 이철 (부천세종병원 흉부외과, 세종심장연구소) ;
  • 장윤희 (부천세종병원 흉부외과, 세종심장연구소) ;
  • 조원민 (부천세종병원 흉부외과, 세종심장연구소) ;
  • 김재현 (부천세종병원 흉부외과, 세종심장연구소) ;
  • 서홍주 (부천세종병원 흉부외과, 세종심장연구소) ;
  • 강상수 (한림대학교 의과대학 마취통증의학교실) ;
  • 문현수 (한림대학교 의과대학 마취통증의학교실) ;
  • 박영관 (부천세종병원 흉부외과, 세종심장연구소) ;
  • 김종환 (부천세종병원 흉부외과, 세종심장연구소)
  • Published : 2003.07.01

Abstract

Annuloaortic ectasia, aortic regurgitation(AR), and ventricular septal defect(VSD) in patients with right ventricular hypoplasia is a very rare condition. We report a patient who underwent aortic root replacement with a composite graft for annuloaortic ectasia associated with VSD and AR in right ventricular hypoplasia. The patient was a 19 year-old male. Transthoraic echocardiogram and cardiac catheterization revealed a perimembranous VSD (2 cm in diameter), severe AR, annuloaortic ectasia, bipartite right ventricle with hapoplasia, and hypoplastic tricuspid valve. Operative findings showed that free margins of the right and noncoronary cusps were markedly elongated, thickened, and retracted, and commissure between the right coronary cusp and the noncoronary cusp was fused and calcified. VSD was closed with an autologous pericardial patch and composite graft aortic root replacement using direct coronary button reimplantation was performed, and the hypertrophic muscle of the right ventricular outflow tract was resected. The patient had transient weaning failure of cardiopulmonary bypass and was discharged at the postoperative 14 days without any problems.

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