肺动脉高压原因不明44例临床分析

A Retrospective Analysis of 44 Cases with Pulmonary Hypertension of Unknown Origin

目的：探讨不明原因肺动脉高压（ＰＨ）的病因。 方法：回顾性总结分析１９９０年１月～２０００年６月我院诊断ＰＨ而原因不明的患者４４例。 结果：４４例患者最终明确诊断者３８例。病因：自身免疫病１２例（３１．６％）；肺栓塞１０例（２６．３％）；其它肺部疾病６例（１５．８％），其中包括肺泡低通气综合征和（或）阻塞性睡眠呼吸暂停综合征３例；感染性心内膜炎及肝硬化、多发性骨髓瘤各１例（２．６％）；原发性ＰＨ７例（１８．４％）。原因仍不明者６例，占总数的１３．６％，包括绒毛膜癌３例，慢性淋巴细胞性白血病１例。 结论：自身免疫病和肺栓塞仍然是ＰＨ的主要原因，需要加强对一些少见病因的认识，多发微小瘤栓栓塞应该作为ＰＨ一项重要的鉴别诊断。

Objective: To investigate the causes of pulmonary hypertension (PH)of unknown origin. Methods: The clinical data in 44 cases of PH admitted to Peking Union Medical College Hospital from Jan, 1990 to June, 2000 were analyzed retrospectively. Eligible criteria were as follows: ① admitted to hospital with PH of unknown origin. ② PH was found in hospital stay which could not be explained with the existing disease. Results: The diagnosis was finally made in 38 cases, of them the autoimmune diseases were found in 12 cases (13.6% ), and the pulmonary embolism in 10 cases (26.3% ). The other pulmonary diseases in 6 cases (15.8% )including 3 patients with alveolar hypoventilation syndrome and/or obstructive sleep apnea syndrome. One patient (2.6% )with cirrhosis and 1 patient (2.6% )with multiple myeloma. The final diagnosis was made as primary PH in 7 cases (18.4% ). The cause still remained obscure in 6 cases included 3 chariocarcinoma and 1 chronic lymphocyte leukemia. Conclusion: Autoimmune disease and pulmonary embolism are still the main cause of PH of unknown origin. It is neces- sary to pay more attentions to some uncommon causes. It is important to consider the tumor microembolism in the differential diagnosis of PH.