Cardiology Clinic, Thrombosis Centre, Department of Cardiac Thoracic and Vascular Sciences, University of Padua, Via Giustiniani 2, 35128, Padua, Italy.
Key Laboratory of Pulmonary Vascular Medicine & FuWai Hospital, State Key Laboratory of Cardiovascular Disease, Chinese Academy Medical Sciences & Peking Union Medical College, 167, Bei-li-shi Road, Beijing, 100037, People's Republic of China.
Intern Emerg Med. 2019 Jun;14(4):521-527. doi: 10.1007/s11739-018-02021-z. Epub 2019 Jan 2.
How thrombophilia may contribute to the development of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. We searched on PubMed and EMBASE (until 15 April 2018), studies on CTEPH reporting data on inherited or acquired thrombophilia. Starting from 367 articles mentioning the search terms, 347 were excluded mainly as duplicate articles or articles not in English. After reading the full text of remaining articles, ten were excluded for being reviews, editorials, letters or case reports, and two were further removed from the analysis because of the potential selection bias. All the eight considered studies provided the proportion of patients positive for antiphospholipid (aPL) antibodies. The crude rate of aPL in CTPEH patients is 11.8% (95% CI 10.09-13.8%). The meta-analysis considering the weighted mean proportion and 95% confidence intervals (CIs) yields a rate of aPL antibody-positive profile of 12.06% (95% CI 8.12-16.65%) among the patients with CTEPH in the random effects model (I 76.33%; 95% CI 52.75-88.14%, p = 0.0001). The sensibility analysis confirms the result. No predictors of heterogeneity are found in a meta-regression analysis. Our results suggest that aPL antibodies are frequently associated with CTEPH underlining the need to test for aPL antibodies in young patients with "idiopathic" and "provoked" PE caused by mild provoking risk factors.
血栓形成倾向如何导致慢性血栓栓塞性肺动脉高压（CTEPH）尚不清楚。我们在 PubMed 和 EMBASE 上进行了检索（截至 2018 年 4 月 15 日），检索了报道遗传性或获得性血栓形成倾向与 CTEPH 相关数据的研究。从提到搜索词的 367 篇文章中，排除了 347 篇主要是因为重复文章或非英文文章。在阅读了其余文章的全文后，又排除了 10 篇因是综述、社论、信件或病例报告，另外 2 篇因潜在的选择偏倚而被进一步排除在分析之外。所有 8 项考虑中的研究均提供了抗磷脂（aPL）抗体阳性患者的比例。CTEPH 患者 aPL 的粗率为 11.8%（95%CI 10.09-13.8%）。考虑加权平均比例和 95%置信区间（CI）的荟萃分析在随机效应模型中得出 CTEPH 患者 aPL 抗体阳性率为 12.06%（95%CI 8.12-16.65%）（I 76.33%；95%CI 52.75-88.14%，p=0.0001）。敏感性分析证实了这一结果。荟萃回归分析未发现异质性的预测因素。我们的结果表明，aPL 抗体经常与 CTEPH 相关，这突出表明需要对由轻度诱发危险因素引起的“特发性”和“诱发”PE 的年轻患者检测 aPL 抗体。
