肺动脉高压少见病因的临床分析研究

目的探讨肺动脉高压(PH)的少见病因,提高对PH的认识.方法分析2002年10月至2004年12月我院诊断为PH并排除常见疾病的36例患者.结果36例患者具体病因为肺栓塞17例(47.2%);自身免疫系统疾病8例(22.2%);血液病2例(5.6%);特发性肺纤维化2例(5.6%);感染性心内膜炎、肺动脉瘤样扩张、肺动脉缺如伴狭窄各1例(各占2.8%);原发性PH 2例(5.6%)、原因不明2例(5.6%).结论在引起PH的少见病中,肺栓塞为主要原因,其次为免疫系统疾病,临床医师加强对肺动脉高压少见病的认识,有助于明确肺动脉高压的病因.

Clinical analysis of uncommon origin of pulmonary hypertension

Objective: To investigate the causes and improve the identification of pulmonary hypertension (PH) of uncommon origin. Methods: The clinical data in 36 cases of PH admitted to the First Affiliated Hospital of Suzhou University from Oct, 2002 to Dec, 2004 were analyzed. Results:The diagnosis was finally made in 36 cases, of which pulmonary embolism was found in 17 cases (47. 2% ) and the autoimmune diseases in 8 cases (22. 2% ) , idiopathic pulmonary fibrosis in 2 cases (5.6%), haemal diseases in 2 cases (5. 6% ). One patient with endocarditis (2. 8% ) , 1 patient with dilatated pulmonary artery (2.8%) and 1 patient with absence of pulmonary artery associated with pulmonary stenosis (2. 8% ) were founded. The final diagnosis was made as primary PH in 2 cases (5. 6% ). The cause still remained obscure in 2 cases (5.6% ). Conclusion : The main cause of uncommon origin is pulmonary embolism and the secondary one is the autoimmune disease. The clinicians must be familiar with some uncommon causes of uncommon origin to improve the identification of pulmonary hypertension.