Thromboendarterectomy as Treatment in the Antiphospholipid Syndrome

Suzette W. Peng, MD; Jeanne P. Mitchell, MD

In This Article

Abstract and Introduction

Antiphospholipid antibody syndrome (APS) may lead to pulmonary hypertension, a lethal complication of chronic pulmonary thromboembolism. There are few viable treatment options for pulmonary hypertension, but pulmonary thromboendarterectomy (PTE) has been used to successfully treat this condition in many patient populations. Following is a description of a patient with APS and systemic lupus erythematosus (SLE) who presented with severe pulmonary hypertension and underwent lifesaving surgery with pulmonary thromboendarterectomy. Successful intervention with thromboendarterectomy for the treatment of severe pulmonary hypertension is possible in patients with the antiphospholipid antibody syndrome, and surgical referral of these patients is warranted.

APS is defined by the occurrence of arterial or venous thromboses in the presence of antiphospholipid antibodies (aPL), namely anticardiolipin antibodies (aCL) or lupus anticoagulant (LA). The risk of thrombosis in patients with aPL is unknown and varies depending on other patient characteristics.[1] Asymptomatic patients incidentally found to have aPL have less than 1% risk per year of developing venous thromboembolism (VTE).[1,2] Conversely, up to 69% of patients with aPL and a history of thrombosis will develop recurrent thromboses.[1,3]

The pulmonary manifestations of APS include thromboembolism, pulmonary hypertension, and, less commonly, microvascular thromboses, pulmonary capillaritis, and alveolar hemorrhage. Chronic thromboembolism is a frequent cause of progressive pulmonary hypertension in APS and has a poor prognosis. The medical treatment of chronic thromboembolic pulmonary hypertension (CTEPH) is not effective and surgery provides the only potential for a cure at present.[4,5] The following is a report of a patient with severe CTEPH secondary to APS and SLE who underwent PTE and had a dramatic improvement in her hemodynamic status.


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