특발성 폐동맥고혈압과 만성혈전색전성 폐고혈압의 임상상 비교

Comparison of Clinical Features between Idiopathic Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

  • 김현국 (울산대학교 의과대학 서울아산병원 내과학교실) ;
  • 나주옥 (순천향대학교 의과대학 내과학교실) ;
  • 안종준 (울산대학교 의과대학 울산대학교병원 내과학교실) ;
  • 박용범 (한림대학교 의과대학 내과학교실) ;
  • 임재민 (울산대학교 의과대학 서울아산병원 내과학교실) ;
  • 홍상범 (울산대학교 의과대학 서울아산병원 내과학교실) ;
  • 오연목 (울산대학교 의과대학 서울아산병원 내과학교실) ;
  • 심태선 (울산대학교 의과대학 서울아산병원 내과학교실) ;
  • 임채만 (울산대학교 의과대학 서울아산병원 내과학교실) ;
  • 고윤석 (울산대학교 의과대학 서울아산병원 내과학교실) ;
  • 김우성 (울산대학교 의과대학 서울아산병원 내과학교실) ;
  • 김동순 (울산대학교 의과대학 서울아산병원 내과학교실) ;
  • 김원동 (울산대학교 의과대학 서울아산병원 내과학교실) ;
  • 이상도 (울산대학교 의과대학 서울아산병원 내과학교실)
  • Kim, Hyun Kuk (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Na, Joo Ock (Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Ahn, Jong Joon (Department of Internal Medicine, University of Ulsan college of medicine, Ulsan University Hospital) ;
  • Park, Yong Bum (Department of Internal Medicine, Hallym University College of Internal Medicine) ;
  • Lim, Jae Min (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Hong, Sang-Bum (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Oh, Yeon-Mok (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Shim, Tae Sun (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Lim, Chae-Man (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Koh, Younsuck (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Kim, Woo Sung (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Kim, Dong Soon (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Kim, Won Dong (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center) ;
  • Lee, Sang-Do (Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center)
  • 투고 : 2005.06.09
  • 심사 : 2005.07.30
  • 발행 : 2005.08.30

초록

배 경 : 특발성 폐동맥고혈압(idiopathic pulmonary arterial hypertension, IPAH)과 만성혈전색전성 폐고혈압(chronic thromboembolic pulmonary hypertension, CTEPH)은 드문 질환이지만 만성적인 폐동맥 고혈압을 유발하는 중요한 질환이다. 두 질환은 임상소견 및 검사소견이 유사한 질환으로 감별이 어렵지만 치료의 차이 때문에 감별이 꼭 필요한 질환이다. 한 대학병원에서 경험한 두 질환의 임상상의 유사점과 차이점을 분석하고자 하였다. 대상 및 방법 : 서울아산병원에서 1995년부터 2002년까지 IPAH로 진단 받은 환자 33명과 CTEPH으로 진단 받은 환자 22명에 대해서 작성된 프로토콜과 의무기록을 검토하여 증상, 신체검사, 심전도, 흉부단순촬영, 폐기능검사, 심초음파, 핵의학검사, 심도자검사 등의 임상소견을 비교, 분석하였다. 결 과 : 나이의 중앙값은 IPAH군에서 33세(6~70세)로 CTEPH군의 52세(27~78세)보다 적었다. 성별은 IPAH군은 33명중 여자가 25명(76%)으로 남자보다 많았고 CTEPH군은 22명중 남자가 12명(55%)으로 성비에 차이가 없었다. 흉부단순촬영상 계측치, 심전도에서 계측치, 폐기능검사에서 폐활량과 폐확산능 그리고 심초음파에서 삼첨판최고역류속도는 두 군간에 차이가 없었다. 폐관류스캔에서는 IPAH군의 28.1%의 환자가 정상소견을 보였고 71.9%의 환자가 폐색전증의 낮은 가능성 소견을 보였다. 이에 반해 CTEPH군에서는 22명 모든 환자에서 폐색전의 높은 가능성소견이 관찰되었다. 결 론 : CTEPH과 IPAH는 임상증상이나 일반적인 검사소견이 유사한 질환이지만, 나이와 성별분포 및 폐관류 스캔소견에 차이를 보여 이러한 차이점들이 두 질환의 감별에 도움을 줄 것으로 사료된다.

Background : Idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare but significantly imperative in inducing chronic pulmonary hypertension. Clinically, it is difficult to distinguish between IPAH and CTEPH. However, the treatment of pulmonary hypertension is different depending on the disease. The present study was performed to analyze the similarities and differences in clinical features between IPAH and CTEPH. Methods : During a nine-year period, thirty-three patients with IPAH and twenty-two patients with CTEPH were enrolled. Symptoms, physical findings, chest radiograph, electrocardiograph, pulmonary function test, echocardiograph, perfusion lung scan, right heart catheterization results were analyzed between both the groups. Results : The median age of IPAH group was 33 (6~70) years that was lower than that (52(27~80) years) of CTEPH group. Amongst the IPAH patients, there was female predominance (76 %) and there was no sex difference between the patients with CTEPH. Both the groups exhibited similarity in the results of chest radiograph, electrocardiograph, pulmonary function test, and echocardiograph. In the perfusion lung scan, all IPAH patients exhibited findings with normal (28%) or low probability (72%) of pulmonary embolism and all CTEPH patients exhibited findings with high probability of pulmonary embolism. Conclusion : Although IPAH and CTEPH bear similarities in terms of symptoms, physical signs and general investigation results, there were differences in age distribution, sex predominance and results of perfusion lung scan.

키워드

참고문헌

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