Multimodal Approach of Isolated Pulmonary Vasculitis: A Single-Institution Experience
| Autor: | Sehnaz Olgun Yildizeli, Halil Atas, G. Nural Bekiroğlu, Ahmet Zengin, Nevsun Inanc, Atakan Erkılınç, Emine Bozkurtlar, Haner Direskeneli, Fatma Alibaz-Oner, Bu¨lent Mutlu, Bedrettin Yildizeli, Mehmed Yanartaş, Cagatay Cimsit, Serpil Taş, Ayşe Zehra Karakoç |
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| Rok vydání: | 2022 |
| Předmět: |
Vasculitis
Pulmonary and Respiratory Medicine medicine.medical_specialty Hypertension Pulmonary Endarterectomy Disease Pulmonary Artery Pulmonary endarterectomy medicine.artery medicine Humans Pulmonary artery stenosis business.industry Multimodal therapy Middle Aged medicine.disease Surgery Chronic Disease Pulmonary artery Etiology Female Chronic thromboembolic pulmonary hypertension Pulmonary Embolism Cardiology and Cardiovascular Medicine business |
| Zdroj: | The Annals of Thoracic Surgery. 114:1253-1261 |
| ISSN: | 0003-4975 |
| Popis: | Background Isolated pulmonary vasculitis (IPV) is a single-organ vasculitis of unknown etiology and may mimic chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to review our clinical experience with pulmonary endarterectomy in patients with CTEPH secondary to IPV. Methods Data were collected prospectively for consecutive patients who underwent pulmonary endarterectomy and had a diagnosis of IPV at or after surgery. Results We identified nine patients (six female, median age 48 (23–55) years) with IPV. The diagnosis was confirmed after histopathological examination of all surgical materials. The mean duration of disease before surgery was 88.0 ±70.2 months. Exercise-induced dyspnea was the presenting symptom in all patients. Pulmonary endarterectomy was bilateral in six patients and unilateral in three. No mortality was observed, however, one patient had pulmonary artery stenosis and stent implantation was performed. All patients received immunosuppressive therapies after surgery. Mean pulmonary artery pressure decreased significantly from 30(19–67) mm Hg to 21(15–49) mm Hg after surgery (p Conclusions Isolated pulmonary vasculitis can mimic CTEPH, and these patients can be diagnosed with pulmonary endarterectomy. Furthermore, surgery has not only diagnostic but also therapeutic value for IPV when stenotic and/or thrombotic lesions are surgically accessible. A multidisciplinary experienced CTEPH team is critical for management of these unique patients. |
| Databáze: | OpenAIRE |
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