We present the case of a 22-year-old female with a history of systemic lupus erythematosus and antiphospholipid syndrome, complicated by recurrent deep vein thrombosis of the lower extremities and pulmonary embolism, who subsequently developed chronic thromboembolic pulmonary hypertension. The patient presented with progressive dyspnea, ultimately limiting daily activities, and was managed with long-term anticoagulation and pulmonary vasodilator therapy. Diagnostic workup, including imaging and hemodynamic assessment, revealed multiple bilateral segmental perfusion defects, right heart dilation, and significantly elevated pulmonary pressures with increased pulmonary vascular resistance, consistent with operable chronic thromboembolic disease. The patient underwent pulmonary endarterectomy via median sternotomy under cardiopulmonary bypass with deep hypothermia and intermittent total circulatory arrest. Two periods of circulatory arrest, of 19 and 18 minutes, were utilized during the procedure. Organized thromboembolic material was successfully removed from both pulmonary arteries, achieving adequate endarterectomy planes without major intraoperative complications. In the immediate postoperative period, the patient remained hemodynamically stable, with expected improvement in right ventricular afterload and pulmonary hemodynamics. This case highlights the importance of recognizing chronic thromboembolic pulmonary hypertension in young patients with autoimmune disease and recurrent thrombotic events, as well as the role of pulmonary endarterectomy as a potentially curative treatment when performed in specialized centers.
Ponce et al. (Sun,) studied this question.