Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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혈역학적으로 의미있는 선천성 심기형을 가진 극소 저체중 출생아의 임상경과 및 예후

Clinical course and prognosis of hemodynamically significant congenital heart defects in very low birth weight infants

  • 유혜수 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 김지은 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 박수경 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 서현주 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 정유진 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 최서희 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 정수인 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 김성훈 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 양지혁 (성균관대학교 의과대학 삼성서울병원 흉부외과학교실) ;
  • 허준 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 장윤실 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 전태국 (성균관대학교 의과대학 삼성서울병원 흉부외과학교실) ;
  • 강이석 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 박원순 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 박표원 (성균관대학교 의과대학 삼성서울병원 흉부외과학교실) ;
  • 이흥재 (성균관대학교 의과대학 삼성서울병원 소아과학교실)
  • Yoo, Hye Soo (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Ji Eun (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Park, Soo Kyoung (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Seo, Hyun Ju ;
  • Jeong, Yoo Jin (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Chio, Seo Heui (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Jeong, Soo In (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Sung Hoon (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Yang, Ji Hyuk (Department of thoracic and cardiovascular surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Huh, June (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Chang, Yun Sil (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Jun, Tae Gook (Department of thoracic and cardiovascular surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kang, I Seok (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Park, Won Soon (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Park, Pyo Won (Department of thoracic and cardiovascular surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Heung Jae (Department of pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 투고 : 2008.09.02
  • 심사 : 2008.11.09
  • 발행 : 2009.04.15
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초록

목 적 : 본 연구는 단일 기관에서 혈역학적으로 의미 있는 선천성 심기형을 가진 극소 저체중 출생아의 임상경과와 치료성적 및 예후 인자를 알아보고자 하였다. 방 법 : 1994년 11월부터 2007년 12월까지 13년간 삼성서울병원 신생아 집중치료실에 입원하였던 출생 체중 1,500 g 미만의 극소 저체중 출생아 1,098명을 대상으로 의무기록을 후향적으로 조사하였다. 선천성 심기형을 가진 대상아 33명의 임상경과, 합병증, 예후를 조사하였고 이들을 대조군 1,065명과 비교하였다. 결 과 : 총 극소 저체중 출생아 1,098명 중 33명(3%)에서 선천성 심기형이 있었으며, 이들 중 7명(21%)에서 선천기형 및 염색체 이상을 동반하였다. 심기형 종류로는 심실 중격 결손이 21명(64%)으로 가장 많았다. 자궁 내 성장 지연은 심기형군이 대조군에 비해 높았으나(34% vs. 20%), 재태기간, 출생체중, 신생아 호흡 곤란 증후군, 기관지폐 형성 이상, 괴사성 장염, 뇌실내 출혈, 그리고 낭종성 뇌실주위 백질 연화증의 빈도는 심기형군과 대조군 사이에 차이가 없었다. 심장수술은 단순 심기형에서 4명(19%), 복합 심기형에서 9명(75%)에게 시행되었고, 9명(69%)이 다단계 수술을, 10명(77%)은 조기 중재시술을 받았다. 심기형군의 사망률은 대조군에 비해 증가되어 있었고(27% vs. 16%), 특히 염색체 이상 및 다발성 기형을 동반한 경우에만 증가되었고(86% vs. 11%), 그 외 단순, 복합 심기형 여부(19% vs. 42%)와는 상관관계가 없었다. 결 론 : 극소 저체중 출생아에서 발생한 선천성 심기형의 예후는 심기형 자체의 복합성 보다는 동반된 선천 기형 및 염색체 이상이 결정한다.

Purpose : This study investigated the clinical course and prognostic factor of very low birth weight infants (VLBWI) with hemodynamically significant congenital heart defects (CHDs). Methods : Medical records of 1,098 VLBWI with birth weight <1,500 g who had been admitted to the neonatal intensive care unit of Samsung Medical Center from October 1994 to December 2007 were reviewed retrospectively. The data for these patients with hemodynamically significant CHD (n=33) were compared with those without CHD (n=1,065). Results : The incidence of CHD was 3.0% (33 patients) 7 patients (21%) had CHD combined with the congenital abnormalities or chromosomal disorders. The most common CHD was a ventricular septal defect. The incidence of intrauterine growth retardation was higher in patients with CHD than in patients without CHD (34% vs. 20%), but there were no significant differences in gestational age, birth weight, respiratory distress syndrome, bronchopulmonary dysplasia, necrotizing enterocolitis, severe intraventricular hemorrhage (${\geq}$Gr III), and periventricular leukomalacia. Cardiac surgery was performed on 13 patients (39%). Nine patients received staged operations, and 10 patients received early intervention. The overall mortality in patients who had CHD was higher than in the patients who did not have CHD (27% vs. 16%). In patients with CHD, congenital abnormalities or chromosomal disorders were more important factors for increased mortality (86% vs. 11%) than the degree of complexity of CHD (19% vs. 42%). Conclusion : The most important prognostic factors of VLBWI with CHD are the associated congenital abnormalities or chromosomal disorders.

키워드

과제정보

연구 과제 주관 기관 : In-Sung Foundation Medical Research

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