Clinical Characteristics Of Patients In Chronic Thromboembolic Pulmonary Hypertension With Antiphospholipid Syndrome

OBJECTIVE:Chronic thromboembolic pulmonary hypertension(CTEPH)is a complex cardiovascular disease,with unknown mechanism.Antiphospholipid syndrome(APS)is an acquired thrombophilic disease.Part of CTEPH patients are often combined with APS,in addition,the clinical features of CTEPH patients with APS and patients without APS are different from each other.The present study was designed to investigate the prevalence of APS in CTEPH patients and to analyze whether the effects of APS were combined with clinical features of CTEPH,aiming to lay the foundation of the partial elucidation of CTEPH mechanisms.METHODS:In this study,a total of 297 patients with CTEPH were retrospectively reviewed from thrombosis center of the FuWai Hospital of the Chinese Academy of Medical Sciences through May 2013 to December 2018.The clinical data of patients were collected and analyzed,including demographic statistics,medical history data,comorbidities,hemodynamics data tested by right heart catheterization,and pulmonary artery lesion distribution analyzed by expert using the CT and/or transcatheter pulmonary angiography.Detection and diagnosis of antiphospholipid antibodies were performed according to the international standards,multiple antiphospholipid antibody profiles were conducted on all patients,including:lupus anticoagulant(LAC),anticardiolipin antibody(aCL IgG/M)and β2GPI antibody(aβ2GPI IgG/M),Twelve weeks after the positive detection of the antiphospholipid antibody,the test was re-performed to confirm whether it was a long-term positive APS combined CTEPH patients.RESULTS:A total of 23 patients with CTEPH included were APS-positive(7.7%).Among them,17 APS-CTEPH patients(74%)had a triple positive antiphospholipid antibody profile.Compared with the APS-negative group,patients with APS(23 patients)were younger(30.0 ± 11.1 years vs.55.6 ± 12.9 years,p<0.0001),and more likely to harbor a history of pulmonary embolism(95.6%vs.65.7%,p=0.003).Autoimmune diseases were more common(43.5%vs.2.9%,p<0.0001),hemodynamic parameters were better;and mPAP were lower(44.4 ± 11.8 mmHg vs.50.5 ± 12.2 mmHg,p=0.03).In APS-positive patients,pulmonary angiography revealed pulmonary artery occlusion closer to the proximal pulmonary artery,with higher frequency of unilateral pulmonary occlusion.CTEPH patients with APS combined with SLE had lower systolic pulmonary artery pressure detected by RHC and NT-proBNP compared to APS-positive CTEPH patients without SLE.CONCLUSIONS:Our results clarify the incidence of APS with CTEPH in all CTEPH patients.In addition,CTEPH patients with APS have clear clinical and hemodynamic characteristics.