肺动脉内膜肉瘤致原发性肺动脉高压1例

Pulmonary artery intimal sarcoma as a rare cause of pulmonary hypertension:a case report

肺动脉内膜肉瘤(PAIS)是一种罕见疾病,其误诊率高。由于发病时易与其他心血管疾病相混淆,一旦误诊或漏诊,导致病情进一步发展,错过最佳治疗时机。为提高临床对该疾病的诊疗水平,正确制订诊疗方案,本文汇报分析1例就诊于广东省中医院的PAIS患者。该患者首发症状为进行性胸闷和气喘,通过术前查体、影像学检查及结合以往病史,考虑为PAIS;在体外循环下行肺动脉肿瘤切除术,术中见灰白色肿物沿肺动脉主干侵犯至血管全层,完整切除肿瘤,术后病理检查证实为PAIS。患者术后1个月因多器官功能衰竭去世。通过回顾性分析国内外文献,多数认为一旦确诊PAIS,应积极手术治疗和同步化放疗;同时通过对疾病的症状、临床表现、影像特征等进行文献复习,提高对该疾病的认识,进而减少误诊或漏诊。

Pulmonary artery intimal sarcoma(PAIS)is a rare disease with a high misdiagnosis rate.Because the onset is easy to be confused with other cardiovascular diseases,once misdiagnosed or missed diagnosis,resulting in further development of the disease,miss the best treatment opportunity.In order to improve the level of clinical diagnosis and treatment of this disease and make correct diagnosis and treatment plan,a case of PAIS was reported and analyzed in Guangdong Provincial Hospital of Chinese Medicine.The first symptoms of the patient were progressive chest tightness and asthma.The patient was considered to be PAIS through preoperative physical examination,imaging examination,and combined with previous medical history.The pulmonary artery tumor was excised under cardiopulmonary bypass,during which gray and white masses were found to invade the whole layer of blood vessels along the main pulmonary artery.The tumor was completely resected,and PAIS was confirmed by postoperative pathological examination.The patient died of multiple organ failure one month after surgery.Through retrospective analysis of domestic and foreign literature,most of them believe that once PAIS is diagnosed,surgery and concurrent chemorotherapy should be actively treated;at the same time,literature review was carried out on the symptoms,clinical manifestations,imaging features of the disease to improve the understanding of the disease,so as to reduce misdiagnosis or missed diagnosis.