Abstract:
BACKGROUND:Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and life-threatening condition with poor prognosis in patients with antiphospholipid syndrome (APS). Pulmonary thromboendarterectomy (PTE) is the optimal surgical option for CTEPH. OBJECTIVES:This retrospective cohort study aimed to evaluate the efficacy and risk of PTE in patients with APS-associated CTEPH. METHODS:Consecutive patients with APS-associated CTEPH diagnosed between January 2012 and September 2017 at Peking Union Medical College Hospital were retrospectively evaluated. Demographics, clinical manifestations, antiphospholipid antibody (aPL) profiles, and pulmonary arterial hypertension-targeted medications were collected. Deterioration of cardiac function and death were chosen as the endpoints, in order to assess the effect of PTE on short-term and long-term prognoses (evaluated by the change of cardiac function after treatment and cardiac deterioration or death in the follow-up, respectively). RESULTS:A total of 20 patients with APS-associated CTEPH were enrolled, and eight patients underwent PTE. Chi-square test ( p = 0.01) and Kaplan-Meier curves (log rank test, p = 0.04) showed that there were statistically significant differences in both short-term and long-term prognoses between patients with and without PTE. CONCLUSION:These results provide strong evidence that PTE is a curative resolution in patients with APS-associated CTEPH. Following a full specialized and multidisciplinary risk-benefit evaluation to limit the risk of thrombosis or bleeding and to manage possible thrombocytopenia, PTE is at least a temporal curative resolution for CTEPH complicated with APS.
journal_name
Lupusjournal_title
Lupusauthors
Li C,Zhao J,Liu S,Song W,Zhu J,Hua L,Wang Q,Li M,Zeng X,Zhao Ydoi
10.1177/0961203318810427subject
Has Abstractpub_date
2018-12-01 00:00:00pages
2206-2214issue
14eissn
0961-2033issn
1477-0962journal_volume
27pub_type
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