Curative resolution of chronic thromboembolic pulmonary hypertension with pulmonary thromboendarterectomy in primary antiphospholipid syndrome

Abstract

Rationale:Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe complication of antiphospholipid syndrome (APS). Once diagnosed, the recommendation for the treatment of CTEPH is long-term anticoagulation and pulmonary thromboendarterectomy (PTE). However, cardiac surgeons apply PTE cautiously for these patients, as there is an increased risk of perioperative complications. Here, we present the curative case of a patient with severe APS-associated CTEPH treated with PTE.Patient concerns:A 29-year-old man presented with chest pain, decreased exercise capacity, dyspnoea, and haemoptysis.Diagnoses:He was triple positive for antiphospholipid antibodies. Computed tomography pulmonary angiography revealed multiple, recurrent pulmonary embolisms and complete obstruction of the left pulmonary artery. He was diagnosed with APS and CTEPH.Interventions:After balancing the risk of thrombosis and haemorrhage, the patient underwent PTE.Outcomes:The patient experienced symptom relief after PTE, and electrocardiography at a six-month follow-up showed a recovery of cardiac structure and pulmonary arterial pressure.Lessons:After evaluating the thrombosis risk at an experienced treatment centre and the application of standard anticoagulation treatment, PTE may be a curative resolution for APS-associated CTEPH.