Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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심한 폐동맥 고혈압을 동반한 좌우 단락 질환 환자의 수술 후 경과

Surgical outcome of severe pulmonary arterial hypertension secondary to left-to-right shunt lesions

  • 이차곤 (성균관대학교 의과대학 삼성서울병원 소아청소년과) ;
  • 정수인 (성균관대학교 의과대학 삼성서울병원 소아청소년과) ;
  • 허준 (성균관대학교 의과대학 삼성서울병원 소아청소년과) ;
  • 강이석 (성균관대학교 의과대학 삼성서울병원 소아청소년과) ;
  • 이흥재 (성균관대학교 의과대학 삼성서울병원 소아청소년과) ;
  • 양지혁 (성균관대학교 의과대학 삼성서울병원 흉부외과) ;
  • 전태국 (성균관대학교 의과대학 삼성서울병원 흉부외과)
  • Lee, Cha Gon (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Jeong, Su-In (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Huh, June (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kang, I-Seok (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Heung Jae (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) ;
  • Jun, Tae Gook (Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 투고 : 2009.09.15
  • 심사 : 2009.12.11
  • 발행 : 2010.02.15
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초록

목 적 : 본 연구에서는 좌우 단락에 의한 심한 폐동맥 고혈압으로 수술 가능 여부가 불확실했던 환자들의 수술 후 중기 임상 경과를 보고하고자 한다. 방 법 : 1995년 이후 삼성서울병원에서 좌우 단락에 의한 폐동맥 고혈압으로 심도자술 후 수술 받은 환자들 중, 만 1세 이후, 폐혈관 저항 8 Wood unit 이상이었던 21명의 환자를 대상으로 이들의 수술 전후 및 추적 기간 중 임상 증상, 폐동맥 압력 변화를 후향적으로 검토하였다. 대상 환자의 수술 당시 평균 연령은 26 (1-58)세였다. 진단은 심방 중격 결손(n=11), 심실 중격 결손(n=4), 심실 중격 결손과 동맥관 개존(n=4), 그리고 동맥관개존(n=2)이었다. 수술 여부는 폐혈관 가역성에 기초하여 결정하였으며, 5명에서는 좌우 단락을 완전히 막았고, 16명의 환자는 작은 좌우 단락을 남겨 놓는 형태의 수술을 시행 받았다. 결 과 : 수술 직후 폐동맥에 삽입한 도관이나 심초음파로 추정한 폐동맥 압력은 모든 환자에서 수술 전보다 감소되었다. 또한 이러한 수술 직후의 폐동맥 압력은 수술 전 결손의 일시적 폐쇄 당시의 폐동맥 압력과 유사한 값을 보였다. 두 명의 환자를 제외한 대부분 환자에서 추적 기간 동안 폐동맥 압력이 감소추세를 유지하였다. 임상증상은 NYHA functional class 기준으로 한 명의 환자를 제외하고는 모두 호전을 보였다. 수술 직후뿐만 아니라 추적 관찰 기간 동안에도 사망이나, 수술과 관련된 특별한 합병증은 없었다. 결 론 : 본 연구에서는 좌우 단락에 의한 심한 폐동맥 고혈압 환자들을 폐동맥 고혈압 가역성에 근거하여 수술하였고, 큰 위험 없이 심장 수술이 가능하였다. 폐동맥 확장제 흡입에 반응을 보이지 않았던 환자들에서 결손의 일시적 폐쇄 상태에서는 반응을 보인 경우가 많았고, 이는 수술 직후 폐동맥 압력과 유사하여 유용한 정보를 얻을 수 있었다. 대부분의 환자에서 폐동맥 압력이 감소하고 임상 양상의 호전을 보였으나, 다시 증가하거나 정상까지 감소하지 않을 수 있으므로 세심한 관찰과 장기적인 추적이 필요하다.

Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity. Methods : We retrospectively reviewed 21 patients who underwent surgical closure of LRSL with severe PAH (${\geq}8$ Wood unit) from January 1995 to April 2009. The median age at operation was 26 years. Atrial septal defect, ventricular septal defect (VSD), VSD and patent ductus arteriosus (PDA), and PDA was present in 11, 4, 4, and 2 patients, respectively. Results : Operability was based on vasoreactivity of PAH. Of the 21 patients, 5 showed response to pulmonary vasodilator therapy and 8 showed vasoreactivity after balloon occlusion of defects. The remaining 8 patients were considered operable because of significant left-to-right shunt (Qp/Qs ${\geq}1.5$). Five patients underwent total closure of defects and 16 were left with small residual shunts. The median follow-up duration was 32 months. There was no significant postoperative mortality or morbidity. Systolic pulmonary artery pressure (PAP) decreased in all but 2 patients. All patients except 1 showed improvement of New York Heart Association functional class. Conclusion : Closure of LRSL in patients with severe PAH on the basis of pulmonary vasoreactivity seems reasonable. PAP and clinical symptoms improved in most patients. Further research is needed for the evaluation of long-term results.

키워드

참고문헌

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