특발성 폐동맥고혈압과 만성혈전색전성 폐고혈압의 임상상 비교
Comparison of Clinical Reatures between Idiopathic Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension
심태선 (울산대학교)
고윤석 (울산대학교)
김동순 (울산대학교)
김원동 (울산대학교)
임채만 (울산대학교)
박용범 (한림대학교)
홍상범 (울산대학교)
오연목 (울산대학교)
김우성 (울산대학교)
이상도 (울산대학교)
나주옥 (순천향대학교)
안종준 (울산대학교)
임재민 (울산대학교)
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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.
- keywords
- Idiopathic pulmonary arterial hypertension, Chronic thromboembolic pulmonary hypertension, Perfusion lung scan, Differential diagnosis, Idiopathic pulmonary arterial hypertension, Chronic thromboembolic pulmonary hypertension, Perfusion lung scan, Differential diagnosis
Abstract
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. (Tuberc Respir Dis 2005; 59: 170-178)
- keywords
- Idiopathic pulmonary arterial hypertension, Chronic thromboembolic pulmonary hypertension, Perfusion lung scan, Differential diagnosis, Idiopathic pulmonary arterial hypertension, Chronic thromboembolic pulmonary hypertension, Perfusion lung scan, Differential diagnosis
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