Correale Michele, Tarantino Nicola, Ieva Riccardo, Gravina Matteo, Casavecchia Grazia, Strazzella Caterina, Di Biase Matteo, Brunetti Natale Daniele
1 Cardiology Department, Ospedali Riuniti University Hospital, Foggia, Italy.
2 Department of Medical & Surgical Sciences, University of Foggia, Foggia, Italy.
Pulm Circ. 2018 Jul-Sep;8(3):2045894018785047. doi: 10.1177/2045894018785047. Epub 2018 Jun 6.
We report the case of an incomplete diagnosis of chronic thromboembolic pulmonary hypertension, with relevant prognostic implications, missing the presence of a primary pulmonary artery angiosarcoma. After the late neoplasm diagnosis, the patient, treated for months with riociguat, was considered inoperable and died soon after. This case highlights the need to manage patients with suspected pulmonary arterial hypertension by expert referral centers with specific and multi-professional expertise (heart and thoracic imaging) in order to avoid incomplete or delayed diagnoses.
我们报告了一例慢性血栓栓塞性肺动脉高压诊断不完整的病例，这具有相关的预后意义，漏诊了原发性肺动脉血管肉瘤的存在。在肿瘤诊断较晚之后，该患者接受了数月的利奥西呱治疗，被认为无法进行手术，不久后死亡。该病例凸显了由具有特定和多专业专长（心脏和胸部影像学）的专家转诊中心对疑似肺动脉高压患者进行管理的必要性，以避免诊断不完整或延迟。
