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 (I2 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.
Keywords: Antiphospholipid antibodies; Chronic thromboembolic pulmonary hypertension; Inherited thrombophilia.