Background and aims Pulmonary hypertension is one of the most debilitating and fatal complications of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). These patients are prone to chronic thromboembolic pulmonary hypertension (CTEPH), for which the treatment of choice is pulmonary endarterectomy (PEA). It is a complex surgical procedure with removal of obstructive thromboembolic material from the pulmonary arteries in order to reduce pulmonary vascular resistance, relieve pulmonary hypertension (PH) and alleviate right ventricular dysfunction. Hereby, we share our clinical experience of PEA for CTEPH in SLE and APS patients.

Methods Data were collected prospectively for consecutive patients with APS and SLE who underwent PEA over a 5 year period [2011–2016]. Case selection was made by consensus of a team consist of a cardiologist, pulmonologist, rheumatologist and thoracic surgeon. All the operations were performed by the same surgical team.

Results We identified 22 patients (5 male, 17 female) with APS and SLE. Mean age was 35 (range=7 to 57). Median NYHA score was III (II to IV). Mean pulmonary artery pressure (mPAP) of the patients fell immediately from 77.4±30.8 mmHg to 28.8±8.9 mmHg right after surgery, and 31.2±7.5 mmHg on discharge. One (5.9%) patient developed acute respiratory distress syndrome and died on postoperative day 10. Mean follow-up duration was 31 months, with no additional mortality.

Conclusions Patients with SLE and/or APS should be screened for CTEPH, since they are more susceptible to intravascular thrombosis. PEA is the treatment of choice for CTEPH patients, with its low morbidity and high success rates.