Department of Diagnostic Pathology, Tokyo Metropolitan Police Hospital.
Department of Cardiology, Tokyo Metropolitan Police Hospital.
Int Heart J. 2021 Jul 30;62(4):938-943. doi: 10.1536/ihj.20-572. Epub 2021 Jul 6.
A pulmonary artery (PA) aneurysm is an extremely rare condition that can be idiopathic or secondary. Only a few reports on giant PA aneurysms associated with chronic thromboembolic pulmonary hypertension (CTEPH) are available in the literature. Here, we present a case of CTEPH associated with a secondary giant PA aneurysm detected by autopsy. A 68-year-old woman was diagnosed with pulmonary hypertension (PH) and a PA aneurysm with a diameter of 7.5 cm 7 years before admission. CTEPH was suspected as the cause of PH; however, she refused to undergo surgical treatment. Although her condition improved temporarily with pulmonary vasodilators, she had recurrence of heart failure and died because of the deterioration of her general condition. An autopsy revealed a giant PA aneurysm without medial degeneration, suggesting a secondary PA aneurysm associated with PH. Histological findings indicated multiple organized thrombi with recanalization in the PA bilaterally, and CTEPH was diagnosed as the cause of PH. Although rare, when a PA aneurysm is detected, it is important to consider that CTEPH might be associated with a giant PA aneurysm. A better understanding of this condition is necessary to improve the therapeutic strategy.
肺动脉瘤是一种极罕见的疾病，可能是特发性的，也可能是继发性的。文献中仅有少数关于与慢性血栓栓塞性肺动脉高压（CTEPH）相关的巨大肺动脉瘤的报道。在这里，我们报告了一例由尸检发现的与继发性巨大肺动脉瘤相关的 CTEPH 病例。一名 68 岁女性在入院前 7 年被诊断为肺动脉高压（PH）和直径为 7.5cm 的肺动脉瘤。CTEPH 被怀疑是 PH 的原因；然而，她拒绝接受手术治疗。尽管她的病情在肺动脉扩张剂的治疗下暂时得到改善，但她再次出现心力衰竭，并因一般状况恶化而死亡。尸检显示巨大肺动脉瘤没有中膜变性，提示与 PH 相关的继发性肺动脉瘤。组织学检查显示双侧肺动脉内有多个有组织的血栓形成和再通，诊断为 PH 合并 CTEPH。虽然罕见，但当发现肺动脉瘤时，重要的是要考虑到 CTEPH 可能与巨大肺动脉瘤有关。需要更好地了解这种情况，以改善治疗策略。
