We have located links that may give you full text access.
Outcomes of Patients with Behçet's Syndrome after Pulmonary Endarterectomy.
Thoracic and Cardiovascular Surgeon 2018 March
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.
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.
Full text links
Related Resources
Trending Papers
Challenges in Septic Shock: From New Hemodynamics to Blood Purification Therapies.Journal of Personalized Medicine 2024 Februrary 4
Molecular Targets of Novel Therapeutics for Diabetic Kidney Disease: A New Era of Nephroprotection.International Journal of Molecular Sciences 2024 April 4
The 'Ten Commandments' for the 2023 European Society of Cardiology guidelines for the management of endocarditis.European Heart Journal 2024 April 18
A Guide to the Use of Vasopressors and Inotropes for Patients in Shock.Journal of Intensive Care Medicine 2024 April 14
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app
All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.
By using this service, you agree to our terms of use and privacy policy.
Your Privacy Choices
You can now claim free CME credits for this literature searchClaim now
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app