Journal of Chest Surgery

The Korean Society for Thoracic and Cardiovascular Surgery (대한심장혈관흉부외과학회)
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Outcome of the Modified Norwood Procedure: 7 Years of Experience from a Single Institution

변형된 노우드(Norwood) 수술의 결과: 단일병원의 지난 7년간의 경험

  • Kim, Hyung-Tae (Department of Thoracic and Cardiovascular Surgery, Pusan National University Yangsan Hospital) ;
  • Sung, Si-Chan (Department of Thoracic and Cardiovascular Surgery, Pusan National University Yangsan Hospital) ;
  • Kim, Si-Ho (Department of Thoracic and Cardiovascular Surgery, Pusan National University Yangsan Hospital) ;
  • Bae, Mi-Ju (Department of Thoracic and Cardiovascular Surgery, Pusan National University Yangsan Hospital) ;
  • Lee, Hyoung-Doo (Department of Pediatrics, Pusan National University Yangsan Hospital) ;
  • Park, Ji-Ae (Department of Pediatrics, Pusan National University Yangsan Hospital) ;
  • Chang, Yun-Hee (Department of Thoracic and Cardiovascular Surgery, St. Mary’s Hospital, The Catholic University of Korea, School of Medicine)
  • 김형태 (양산부산대학교병원 흉부외과) ;
  • 성시찬 (양산부산대학교병원 흉부외과) ;
  • 김시호 (양산부산대학교병원 흉부외과) ;
  • 배미주 (양산부산대학교병원 흉부외과) ;
  • 이형두 (양산부산대학교병원 소아청소년과) ;
  • 박지애 (양산부산대학교병원 소아청소년과) ;
  • 장윤희 (가톨릭대학교 의과대학 서울성모병원 흉부외과학교실)
  • Received : 2010.02.08
  • Accepted : 2010.02.23
  • Published : 2010.08.05
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Abstract

Background: We assessed the early and mid-term results of the modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome (HLHS) and its variants to identify the risk factors for hospital mortality. Material and Method: Between March, 2003, and December, 2009, 23 patients (18 males and 5 females) with HLHS or variants underwent the modified Norwood procedure. The age at operation ranged from 3 to 60 days (mean, $11.7{\pm}13.2days$) and weight at operation ranged from 2.2 to 4.8 kg (mean, $3.17{\pm}0.52kg$). We used a modified technique that spared the anterior wall of the main pulmonary artery in 20 patients. The sources of pulmonary blood flow were RV-PA conduit in 15 patients (group I) and RMBTS in 8 (group II). Follow-up was completed in 19 patients (19/20, 95%) in our hospital (mean $26.0{\pm}22.8months$). Result: Early death occurred in 3 patients (3/23, 13%), of whom 2 had TAPVC. Fourteen patients underwent subsequent bidirectional cavopulmonary connection (BCPC, stage 2) and seven underwent the Fontan operation (stage 3). Three patients died between stages, 2 before stage 2 and one before stage 3. The estimated 1-year and 5-year survival rates were 78% and 69%, respectively. On multivariate regression analysis, aberrant right subclavian artery (RSCA) and associated total anomalous pulmonary venous connection (TAPVC) were risk factors for hospital mortality after stage 1 Norwood procedure. Conclusion: HLHS and its variants can be palliated by the modified Norwood procedure with low operative mortality. Total anomalous pulmonary venous connection adversely affects the survival after a stage 1 Norwood procedure, and interstage mortality rates need to be improved.

배경: 본 연구의 목적은 지난 7년 동안의 좌심형성부전증후군 및 그 변종에 대한 변형 노우드(Norwood) 수술의 조기 및 중기 결과를 알아보고, 조기 병원 사망에 영향을 주는 인자들을 확인하는 것이다. 대상 및 방법: 2003년 5월부터 2009년 12월까지 좌심형성부전증후군 및 그 변종으로 노우드 수술을 시행 받은 23명의 환자들을 대상으로 하였다. 수술 당시 나이는 3일에서 60일이었고, 체중은 2.2 kg에서 4.8 kg의 소견을 보였다. 주폐동맥의 전벽을 보존하는 변형된 술식을 20명의 환자에 있어서 적용하였다. 폐동맥혈류 유지는 15명에서 우심실-폐동맥 도관술, 8명에서 우측변형 브라록-타우시히 단락을 이용하였다. 19명(19/20, 95%)의 환자들에 대하여 평균 $26.0{\pm}22.8$개월 동안 추적관찰을 시행 하였다. 결과: 3명(3/23, 13%)의 조기 병원 사망이 있었다. 이중 2명은 폐정맥연결이상을 동반하고 있었다. 14명의 환자가 양방향성 상대정맥 폐동맥 단락술을 시행 받았고, 7명의 환자가 Fontan 수술을 시행 받았다. 양방향성 상대정맥 폐동맥 단락술 이전에 2명의 사망이 있었고, Fontan 수술이전에 1명의 사망이 있었다. 1년과 5년 장기 생존률은 각각 78%와 69%의 소견을 나타냈다. 이상 우측 쇄골하 동맥과 동반된 전 폐정맥연결이상이 병원 사망과 관련된 위험인자들로 나타났다. 결론: 좌심형성부전증후군 및 그 변종에 대한 변형 노우드(Norwood) 수술은 낮은 수술 사망률로 시행될 수 있었다. 전 폐정맥연결이상의 동반이 노우드 수술 후의 생존에 주요한 위험인자임을 알 수 있었다. 향후 중간단계 사망에 대한 성적향상의 노력이 필요할 것으로 판단된다.

Keywords

References

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