Successful Thromboendarterectomy for Chronic Pulmonary Embolism in a Patient With Systemic Lupus Erythematosus and Antiphospholipid Syndrome

Abstract

Chronic pulmonary thromboembolism is known to be associated with poor prognosis with conservative medical treatment. Pulmonary thromboendarterectomy for chronic pulmonary thromboembolic disease is a potentially lifesaving procedure that prevents right-sided cardiac failure as a result of secondary pulmonary hypertension caused by pulmonary thromboembolism. We report a rare case of systemic lupus erythematosus with antiphospholipid syndrome in a patient who presented with pulmonary hypertension secondary to chronic proximal multiple pulmonary thromboembolism. To our knowledge, this is the first case report of chronic pulmonary thromboembolism complicated by systemic lupus erythematosus with antiphospholipid syndrome. Thromboendarterectomy was performed with satisfactory results.

Pulmonary Endarterectomy in Patients with Antiphospholipid Syndrome-Associated Chronic Thromboembolic Pulmonary Hypertension

“…To date, published data on the outcomes of PEA in APS mainly comprise case reports. 25 - 27 Nakajima et al 28 proposed PEA for patients with APS and high PVR depending on their promising early postoperative hemodynamic and functional improvement. Similarly, Elmogy et al 29 recommended PEA as a curative treatment to lower PVR in patients with the diagnosis of SLE and antiphospholipid syndrome.…”

A Chronic, Massive Thrombus in the Right Main Pulmonary Artery

“…Most case reports on main PA thrombi have focused on the diagnosis 2-4 and treatment [5][6][7] of this condition. Rittoo et al reported the case of a patient with a thrombus in the right PA after surgical closure of an atrial septal defect, as in our case.…”

“…1 There have been numerous case reports on this disorder. [2][3][4][5][6][7] Although most cases of pulmonary artery (PA) thrombosis are embolic in nature, in situ thrombosis has also been suggested. 8 Because pulmonary thrombi are expected to dissolve completely by intrinsic fibrinolysis, the pathogenesis of chronic, large thrombi in the main PAs is unclear.…”

Chronic Thromboembolic Pulmonary Hypertension and Pulmonary Thromboendarterectomy

“…An unexpected isolated prolongation of the activated partial thromboplastin time may indicate the presence of lupus anticoagulant or other antiphospholipid antibodies. 23,24 Chest radiography typically demonstrates clear lung fields with prominent hilar regions. There may be oligemia in the lung fields reflecting diminished pulmonary blood flow.…”