A case report of Chronic Thromboembolic Pulmonary Hypertension associated with antiphospholipid syndrome

While pulmonary thromboembolism is common, thromboembolic pulmonary hypertension is very rare. The present case is a 35 year old woman with chronic thromboembolic pulmonary hypertension, who presented with severe dyspnea and leg edema, following an earlier thrombotic event of 10 years earlier, after her her second childbearing. She also had a history of the first childbearing complicated with congenital heart disease and death of her infant by the age of 5 months. Despite conventional treatments for pulmonary thromboembolism including anticoagulants, her condition developed to severe pulmonary hypertension. The investigations including spiral CT confirmed bilateral pulmonary artery thrombosis, positive antiphospholipid antibody and negative serologic tests for rheumatic disease. Besides, the patient showed no symptoms of systemic vasculitis. Therefore, it was diagnosed that the patient had primary antiphospholipid syndrome without underlying rheumatic disease. Thus, the patient was referred to a thoracic surgeon for endarterectomy because of severe pulmonary hypertension (105 mlHg). However, due to extensive bilateral thrombotic involvement of pulmonary artery, surgical treatment was refused. Treatment with prednisolone 60mg/d and warfarin with high dose was carried out. The clinical condition was improved and dyspnea, edema and general condition recovered. The probable role of this syndrome in the development of chronic thromboembolic pulmonary hypertension, neonatal congenital heart disease, and also therapeutic strategies will be discussed in this report.