Pulmonary thromboendarterectomy (PTE) is a curative resolution for patients with antiphospholipid syndrome (APS) related to chronic thromboembolic pulmonary hypertension (CTEPH), according to the results of a retrospective cohort study conducted at the Peking Union Medical College Hospital in Beijing, China. Findings from the analysis were published in Lupus.

Investigators sought to assess the efficacy and potential risks of using PTE in patients with APS-associated CTEPH who had been diagnosed between January 2012 and September 2017. They obtained data regarding clinical manifestations of disease, antiphospholipid antibody profiles, the use of pulmonary arterial hypertension-targeted medications, and patient demographics. Deterioration of cardiac function and death served as the study end points to evaluate the effect of PTE on short-term and long-term prognoses (ie, based on change in cardiac function after treatment and cardiac deterioration or death in the follow-up period, respectively).

A total of 20 participants who had APS-associated CTEPH were enrolled in the study; 8 of these individuals were undergoing PTE. The median patient age was 29 years (range, 19-51 years); 65.0% (13 of 20) of the patients were women. Median duration of APS from the onset of disease-specific symptoms to diagnosis was 36 months (range, 1-252 months).

Based on the results of chi-square testing (P =.01) and Kaplan-Meier curves (log rank test, P =.04), statistically significant differences were observed with respect to both short- and long-term prognoses between participants who underwent PTE vs those who did not undergo the procedure.


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The investigators concluded that the study results provide strong evidence in favor of PTE use in patients with APS-associated CTEPH. Once a full specialized and multidisciplinary risk-to-benefit assessment has been performed to limit the risk for thrombosis or bleeding associated with PTE and to manage the possible development of thrombocytopenia, PTE can be considered at least a temporal curative resolution in patients with CTEPH that is complicated by the presence of APS.

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Reference

Li C, Zhao J, Liu S, et al. Pulmonary thromboendarterectomy is a curative resolution for chronic thromboembolic pulmonary hypertension associated with antiphospholipid syndrome: a retrospective cohort study. Lupus. 2018;27(14):2206-2214.