Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Clinical features and results of recent total anomalous pulmonary venous connection : Experience in a university hospital (Clinical study of total anomalous pulmonary venous connection)

최근 총폐정맥 환류이상의 임상 경과 및 수술 결과 : 단일 대학병원에서의 경험(총폐정맥 환류이상의 최근 결과)

  • Chu, Mi Ae (Department of Pediatrics, College of Medicine, Kyungpook National University) ;
  • Choi, Byung Ho (Department of Pediatrics, College of Medicine, Kyungpook National University) ;
  • Choi, Hee Joung (Department of Pediatrics, Pochon CHA University) ;
  • Kim, Yeo Hyang (Department of Pediatrics, College of Medicine, Keimyung University) ;
  • Kim, Gun Jik (Department of Thoracic and Cardiovascular Surgery, College of Medicine, Kyungpook National University) ;
  • Cho, Joon Yong (Department of Thoracic and Cardiovascular Surgery, College of Medicine, Kyungpook National University) ;
  • Hyeon, Myung Chul (Department of Pediatrics, College of Medicine, Kyungpook National University) ;
  • Lee, Sang Bum (Department of Pediatrics, College of Medicine, Kyungpook National University)
  • 추미애 (경북대학교 의과대학 소아과학교실) ;
  • 최병호 (경북대학교 의과대학 소아과학교실) ;
  • 최희정 (포천중문의과대학 소아과학교실) ;
  • 김여향 (계명대학교 의과대학 소아과학교실) ;
  • 김근직 (경북대학교 의과대학 흉부외과학교실) ;
  • 조준용 (경북대학교 의과대학 흉부외과학교실) ;
  • 현명철 (경북대학교 의과대학 소아과학교실) ;
  • 이상범 (경북대학교 의과대학 소아과학교실)
  • Received : 2008.08.04
  • Accepted : 2008.11.18
  • Published : 2009.02.15
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Abstract

Purpose : Active perioperative intervention and improvement on surgical technique has decreased the mortality rate of total anomalous pulmonary venous connection (TAPVC); however, when complicated with pulmonary venous obstruction, operative mortality is still high. The purpose of this study was to investigate the clinical course of TAPVC. Methods : Twenty-seven patients who were diagnosed with TAPVC (without other complex heart anomalies) by echocardiogram at Kyungpook National University Hospital from January 1994 to February 2008 were included. Results : Mean age at diagnosis was $28.1{\pm}33.4$ days (1-126 days). Sites of drainage were supracardiac type (15), cardiac (6), infracardiac (5), and mixed (1). Seven patients had pulmonary venous obstruction: 5 with supracardiac type, 1 with cardiac, and 1 with infracardiac. Intraoperative trans-esophageal echocardiograms were performed in 14 patients (58.3%). The operative mortality was 16.7% (4 of 24) and overall hospital mortality (including deaths without operation) was 22.2% (6 of 27). There were 5 postoperative pulmonary venous obstructions. The sites of obstruction were anastomotic in 3 of 5 (60%) patients, and ostial pulmonary vein in the other 2 (40%) patients. Three patients who presented with anastomotic pulmonary venous obstruction underwent reoperation, but all the patients were found to have pulmonary venous anastomotic obstruction. The other 2 patients with ostial pulmonary vein obstruction who had no significant symptoms were diagnosed by routine echocardiographic examination during follow-up. Conclusion : In TAPVC patients, early diagnosis and aggressive surgical management will improve prognosis, and we must pay attention to early and late pulmonary vein restenosis through intraoperative trans-esophageal echocardiogram and peri- and post-operative echocardiographic follow-up examinations.

목 적 : 총폐정맥 환류이상은 드문 선천성 심장질환이나, 진단 즉시 완전 교정 수술이 필요한 질환이며 폐정맥 협착 동반 유무에 따라 수술 사망률과 예후가 다르게 보고되고 있다. 이번 연구는 총폐정맥 환류이상 환아를 조사하여 이들의 임상적 특성과 수술 후 경과를 살펴보고자 시행하였다. 방 법 : 1994년 1월부터 2008년 2월까지 14년간 경북대학교병원에서 총폐정맥 환류이상으로 진단된 환아 27명의 의무 기록 정보를 후향적으로 분석 하였다. 결 과 : 총폐정맥 환류이상 환아 27명은 남아 17명, 여아 10명이었고, 진단 시 나이는 평균 $28.1{\pm}33.4$일(1-126일), 평균 체중은 $3.6{\pm}0.9$ kg (1.9-6.3 kg)였다. 병원을 방문한 주 증상으로 빈호흡, 저산소증 등의 호흡기 증상이 7명, 수유 시 청색증이나 전신 청색증이 13명, 심잡음이 7명이었다. 13명(48.1%)에서 심부전 소견을 보였다. 심초음파 검사로 진단된 해부학적 형태로 심상부형이 15명(55.6%), 심형이 6명(22.2%), 심하부형이 5명(18.5%)이고 혼합형은 1명(3.7%)있었다. 수술 전 폐정맥 협착이 동반된 경우는 7명으로 심상부형에서 5명, 심형과 심하부형에서 각각 1명이었다. 본원에서 수술 받은 환아는 총 24명으로 수술 중 경식도 초음파 검사는 14명(58.3%)에서 시행하였다. 수술 중 경식도 초음파 검사를 사용한 경우의 사망률은 0%이고 사용하지 않은 경우의 사망률 40%로 유의한 차이(P<0.05)를 보였다. 수술 사망은 4명(16.7%)으로, 수술하지 않고 사망한 환아 2명을 포함하여 전체 사망률은 22.2%였다. 그러나 수술 시기를 2000년을 기준으로 구분하여 살펴보면, 사망한 4명 모두 2000년 이전이었으며(사망률 50%, 4/8명), 2000년 이후에는 수술 후 사망이 한 명도 없었다(사망률 0%, 0/18명). 수술 후 폐정맥의 협착은 심초음파 검사로 진단하여 5명에서 발생하였다. 5명중 3명은 수술 부위의 협착이고 나머지 2명은 폐정맥 자체의 협착이었다. 수술 부위의 협착을 보인 3명은 재수술을 시행하였고(평균 기간 : 24일) 재수술 후 사망률은 0%이며, 현재 모두 약물치료는 시행하지 않으며 특별한 증상도 보이지 않으나 3명 모두 정기적으로 시행한 심초음파에서 후기 재 협착 소견을 보였다. 이들이 수술 부위의 후기 재 협착이 진단되기까지의 평균 기간은 31.3개월(17-54개월)이었다. 폐정맥 자체의 협착을 보인 환아는 2명으로, 교정 수술 후 심초음파 검사로 진단되기까지의 기간은 각각 3개월과 84개월이었다. 추적 관찰되었던 환아 20명에서 평균 관찰 기간은 $24.2{\pm}24.9$개월(2-80개월)이었다. 결 론 : 수술 후 폐정맥 협착 및 수술 부위의 재 협착이 발생할 수 있으므로 수술 중 경식도 초음파 검사의 적극적 이용과 수술 후 정기적이고 지속적인 관리가 필요할 것으로 생각된다.

Keywords

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