PMID- 28810271 OWN - NLM STAT- MEDLINE DCOM- 20181009 LR - 20181009 IS - 1439-1902 (Electronic) IS - 0171-6425 (Linking) VI - 66 IP - 2 DP - 2018 Mar TI - Outcomes of Patients with Behcet's Syndrome after Pulmonary Endarterectomy. PG - 187-192 LID - 10.1055/s-0037-1604411 [doi] AB - BACKGROUND: Behcet'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/cm(5) (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. CI - Georg Thieme Verlag KG Stuttgart . New York. FAU - Yildizeli, Sehnaz Olgun AU - Yildizeli SO AD - Department of Pulmonology and Intensive Care, Marmara University School of Medicine, Istanbul, Turkey. FAU - Yanartas, Mehmed AU - Yanartas M AD - Department of Cardiovascular Surgery, Kartal Kosuyolu Hospital, Istanbul, Turkey. FAU - Tas, Serpil AU - Tas S AD - Department of Cardiovascular Surgery, Kartal Kosuyolu Hospital, Istanbul, Turkey. FAU - Direskeneli, Haner AU - Direskeneli H AD - Department of Rheumatology, Marmara University School of Medicine, Istanbul, Turkey. FAU - Mutlu, Bulent AU - Mutlu B AD - Department of Cardiology, Marmara University School of Medicine, Istanbul, Turkey. FAU - Ceyhan, Berrin AU - Ceyhan B AD - Department of Pulmonology and Intensive Care, Marmara University School of Medicine, Istanbul, Turkey. FAU - Yildizeli, Bedrettin AU - Yildizeli B AD - Department of Thoracic Surgery, Marmara University School of Medicine, Istanbul, Turkey. LA - eng PT - Journal Article DEP - 20170815 PL - Germany TA - Thorac Cardiovasc Surg JT - The Thoracic and cardiovascular surgeon JID - 7903387 RN - 0 (Anticoagulants) RN - 0 (Immunosuppressive Agents) SB - IM MH - Adult MH - Anticoagulants/therapeutic use MH - Arterial Pressure MH - Behcet Syndrome/*complications/diagnosis/drug therapy MH - Chronic Disease MH - Computed Tomography Angiography MH - *Endarterectomy/adverse effects MH - Female MH - Humans MH - Hypertension, Pulmonary/diagnostic imaging/etiology/physiopathology/*surgery MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Middle Aged MH - Prospective Studies MH - Pulmonary Artery/diagnostic imaging/physiopathology/*surgery MH - Pulmonary Embolism/diagnostic imaging/etiology/physiopathology/*surgery MH - Recovery of Function MH - Risk Factors MH - Time Factors MH - Treatment Outcome MH - Vascular Resistance MH - Young Adult COIS- Disclosure The authors report no conflicts of interest in this work. EDAT- 2017/08/16 06:00 MHDA- 2018/10/10 06:00 CRDT- 2017/08/16 06:00 PHST- 2017/08/16 06:00 [pubmed] PHST- 2018/10/10 06:00 [medline] PHST- 2017/08/16 06:00 [entrez] AID - 10.1055/s-0037-1604411 [doi] PST - ppublish SO - Thorac Cardiovasc Surg. 2018 Mar;66(2):187-192. doi: 10.1055/s-0037-1604411. Epub 2017 Aug 15.