Objectives: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis. Chronic thromboembolism is one of the known established pathogenesis of pulmonary hypertension, known as chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary endarterectomy (PEA) is the treatment of choice for CTEPH. The aim of this study is to evaluate the efficacy and risk of PEA in patients with APS-associated CTEPH.
Method: Data were prospectively collected and retrospectively analyzed, for patients who underwent PEA between March 2011 and March 2020.
Results: Seventeen patients (4 male,13 female) were identified. Thirteen patients had primary APS and four had secondary APS. The mean age was 34.82±10.07 years and the mean time interval between the diagnosis and surgery was 26.94±17.35 months. Dyspnea on exertion was the main symptom in all patients. Seven patients had previous deep vein thrombosis, five patients had a history of recurrent abortions and two patients had hemoptysis. Following surgery, mean pulmonary artery pressure decreased from 47.82±13.11 mmHg to 22.24±4.56 mmHg (p<0.001), and pulmonary vascular resistance improved from 756.50±393.91 dyn/s/cm-5 to 298.31±132.84 dyn/s/cm-5 (p<0.001). There was no in-hospital mortality with a mean follow-up of 75.29±40.21 months. The functional capacity of all patients improved from 269.46±111.7 meters to 490±105.34 meters on 6-minute walking test.
Conclusions: Pulmonary endarterectomy is a safe and curative treatment in patients with APS-associated CTEPH. It has a favorable outcome by increasing the quality of life. A multidisciplinary experienced CTEPH team is critical in the management of these unique patients.