Abstract Number: PB2223
Meeting: ISTH 2020 Congress
Background: As a hereditary thrombotic risk factor, the role of hereditary thrombophilia in the pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH) still remained unresolved, especially in East Asian patients. The previous studies have focused on two small Caucasian cohorts, ignoring the ethnic specificity of thrombophilia, which may have led to bias in their results.
Aims: We aimed to analyze the association between the clinical characteristics of CTEPH and hereditary thrombophilia.
Methods: We conducted a retrospective cohort study. Hereditary thrombophilia was diagnosed through the screening tests for protein C, protein S, antithrombin III, and factor V Leiden and prothrombin G20210A mutations. Data on clinical, angiographic and hemodynamic characteristics were collected.
Results: Between May 2013 to April 2019, 315 patients with CTEPH were enrolled. Thirty (9.5%) were diagnosed with hereditary thrombophilia, including 12 protein C deficiency (3.8%), 15 protein S deficiency (4.8%), 3 antithrombin III deficiency (1.0%). None factor V Leiden and prothrombin G20210A mutations was defined (Figure 1). Compared with the non-thrombophilia group, patients with hereditary anticoagulant deficiency occurred less likely among females (26.7% vs. 48.4%, p=0.023), more frequently among those with a history of deep vein thrombosis (66.7% vs. 45.3%, p=0.026), especially recurrent thrombosis (13.3% vs. 2.5%, p=0.002). The age and NYHA classification were similar between two groups. While for patients with hereditary anticoagulant deficiency, their exercise tolerance was better evaluated by six-minute walk distance (442 ± 146 vs. 381 ± 122, p=0.032), the same as the haemodynamic profiles.The pulmonary artery lesions in patients with hereditary anticoagulant deficiency were more proximal, especially in left pulmonary artery (Table 1).
Conclusions: The prevalence of hereditary anticoagulant deficiency in Chinese CTEPH patients was significantly high. This association may suggest the special role of vein thrombosis in CTEPH etiology.
To cite this abstract in AMA style:Lian T-, Zhu Y-, Zhou Y-, Yan X-, Wu T, Jiang X, Xu X-, Sun K, Hua L, Jing Z-. High Prevalence of the Hereditary Anticoagulant Deficiency in Chronic Thromboembolic Pulmonary Hypertension (CTEPH) [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/high-prevalence-of-the-hereditary-anticoagulant-deficiency-in-chronic-thromboembolic-pulmonary-hypertension-cteph/. Accessed November 30, 2021.
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