Postoperative Pulmonary Vein Stenosis (PVS) in Patients with TAPVR

전 폐정맥 환류 이상의 수술 후 폐정맥 협착에 대한 분석

  • Jung Sung-Ho (Division of Pediatric Cardiac Surgery, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Park Jeong-Jun (Division of Pediatric Cardiac Surgery, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Yun Tae-Jin (Division of Pediatric Cardiac Surgery, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jhang Won-Kyoung (Division of Pediatric Cardiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim Young-Hwue (Division of Pediatric Cardiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Ko Jae-Kon (Division of Pediatric Cardiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Park In-Sook (Division of Pediatric Cardiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Seo Dong-Man (Division of Pediatric Cardiac Surgery, Asan Medical Center, University of Ulsan College of Medicine)
  • 정성호 (울산대학교 의과대학 서울아산병원 소아심장외과분과) ;
  • 박정준 (울산대학교 의과대학 서울아산병원 소아심장외과분과) ;
  • 윤태진 (울산대학교 의과대학 서울아산병원 소아심장외과분과) ;
  • 장원경 (울산대학교 의과대학 서울아산병원 소아심장분과) ;
  • 김영휘 (울산대학교 의과대학 서울아산병원 소아심장분과) ;
  • 고재곤 (울산대학교 의과대학 서울아산병원 소아심장분과) ;
  • 박인숙 (울산대학교 의과대학 서울아산병원 소아심장분과) ;
  • 서동만 (울산대학교 의과대학 서울아산병원 소아심장외과분과)
  • Published : 2006.05.01

Abstract

Background: Despite recent advances in surgical technique and perioperative care of total anomalous pulmonary venous return (TAPVR), post-repair pulmonary vein stenosis (PVS) remains as a serious complication. We thought that the most important factors of TAPVR repair to prevent PVS were good exposure, proper alignment, and sufficient stoma size. We analyzed our experience retrospectively. Material and Method: Between Jan. 1995 and Feb. 2005, we studied 74 patients diagnosed with TAPVR suitable for biventricular repair. Supra-cardiac type (n=41, 55.4%) was the most common. Mean CPB time, ACC time, and TCA (40.5%, 30/74) time were $92.1{\pm}25.9\;min,\;39.1{\pm}10.6\;min$, and $30.2{\pm}10.7\;min$, respectively. Mean follow-up duration was $41.4{\pm}29.1$ months and follow-up was possible in all patients. Result: The median age and body weight at operation were 28.5 days ($0{\sim}478$ days) and 3.4 kg $(1.4{\sim}9\;kg)$. Early mortality was 4.1% (3/74). Causes of death were pulmonary hypertensive crisis, sepsis, and sudden death. There was PR-PVS in 2 patients (early: 1, late: 1). Both patients were cardiac type TAPVR drained to coronary sinus. Re-operations were done but only one patient survived. Cumulative survival rate in 5 year and percent freedom from PVS were $94.5{\pm}2.7%\;and\;97.2{\pm}2.0%$, respectively. Conclusion: There was no PVS in patients who underwent extra-cardiac anatomosis between LA and CPVC. Therefore it could be said that our principle might be effective in preventing PR-PVS in patients suitable two-ventricle.

배경: 전 폐정맥 환류 이상의 치료성적은 과거에 비해 많이 향상되었으나, 폐정맥 협착은 아직까지 수술 후에 발생하는 심각한 합병증으로 남아있다. 폐정맥 협착의 발생을 감소시키기 위하여 본원에서는 좋은 시야 확보, 적절한 배치, 충분한 개구부의 크기가 중요하다고 생각하고 이러한 원칙하에서 시행한 본원의 경험을 후향적으로 분석하였다. 대상 및 방법: 1995년 1월부터 2005년 2월까지 전 폐정맥 환류 이상으로 진단 받고, 양심실 교정이 가능한 환자 74명을 대상으로 하였다. 상 심장형이 41명(55.4%)으로 가장 많았다. 평균 심폐기 가동시간은 $92.1{\pm}25.9$분, 대동맥 차단 시간은 $39.1{\pm}10.6$분이었고, 순환 정지를 시행한 환자는 30명(40.5%)이었고 평균 시간은 $30.2{\pm}10.7$분이었다. 평균 추적관찰기간은 $41.4{\pm}29.1$개월이었고, 모든 환자에서 추적관찰이 가능하였다. 결과: 수술 당시 나이의 중간값은 28.5일($0{\sim}478$일), 체중은 3.4 kg $(1.4{\sim}9kg)$이었다. 조기사망은 3명(4.1%)이었고, 원인은 폐동맥 고혈압, 패혈증, 돌연사(sudden death)가 각각 1명씩 있었다. 수술 후 폐정맥 폐쇄는 2명(2.7%)으로 조기가 1명, 만기가 1명이었다. 모두 관상 정맥동으로 연결되는 심장형 전폐정맥 환류 이상을 가진 환자들이었고, 심장외부에서 공통 폐정맥과 좌심방의 후벽을 연결하는 재수술을 시행 받았지만 그 중 한 명은 지속적인 폐정맥 협착으로 사망하였다. 5년 생존율과 5년간 폐정맥 협착을 피할 확률은 각각 $94.5{\pm}2.7%$$97.2{\pm}2.0%$였다. 결론: 본원에서 시행한 양심실 교정이 가능한 전 폐정맥 환류 이상 환자의 수술에서 심장외부에서 좌심방의 후벽과 공통 폐정맥을 연결한 환자들에서는 폐정맥의 협착이 발생하지 않았다. 따라서 본원에서 가지고 있는 원칙이 폐정맥 협착을 예방하는 데 효과적인 방법이라고 생각된다.

Keywords

References

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