Abstract
Background Behçet's syndrome (BS) is a multisystem disorder and is not known as a risk factor
for chronic thromboembolic pulmonary hypertension (CTEPH), for which the treatment
of choice is pulmonary endarterectomy (PEA). The aim of this study was to review our
experience in the surgical treatment of CTEPH in patients with BS.
Methods Data were collected prospectively for consecutive patients with BS who underwent
PEA over a 6-year period.
Results We identified nine patients (seven males, two females, mean age: 34.7 ± 9.9 years)
with BS. The mean disease duration before PEA was 88.0 ± 70.2 months. All patients
but one received immunosuppressive therapy before the surgery. Exercise-induced dyspnea
presented symptoms in six patients. One patient had associated intracardiac thrombosis.
PEA was bilateral in five patients, unilateral in three, and lobar in one. No perioperative
mortality was observed; however, one patient died four weeks after PEA due to massive
hemoptysis. Morbidity was observed in two patients. The systolic pulmonary artery
pressure fell significantly from 59.0 ± 22.7 mm Hg to 30.0 ± 6.5 mm Hg after surgery
(p = 0.031). Pulmonary vascular resistance also improved significantly from 611.8 ± 300.2
to 234.7 ± 94.9 dyn/s/cm5 (p = 0.031). After a median follow-up of 29.4 months, all patients improved to the New
York Heart Association (NYHA) functional class I and II.
Conclusion Patients with BS may suffer recurrent pulmonary embolism and develop CTEPH. In patients
who do not respond to anticoagulation or immunosuppressive therapy, PEA may be a therapeutic
option when thrombotic lesions are surgically accessible. Due to the high risk of
perioperative mortality, the procedure should be undertaken in centers with experience.
Keywords
Behçet's syndrome - pulmonary endarterectomy - mortality - hemoptysis - outcome
