Internal Medicine Department, Centro Hospitalar Universitario de Santo Antonio, Porto, Portugal
Internal Medicine Department; Pulmonary Vascular Disease Unit, Centro Hospitalar Universitario de Santo Antonio, Porto, Portugal.
BMJ Case Rep. 2024 Feb 6;17(2):e257942. doi: 10.1136/bcr-2023-257942.
Unlike acute pulmonary embolism (PE), the resolution of thrombi is ineffective in chronic thromboembolic pulmonary hypertension (CTEPH), leading to reorganisation and fibrotic changes within the pulmonary arteries. The authors report the case of a man in his 60s with polycythemia vera, under warfarin, following an acute PE. He was admitted a year later with right heart failure and haemodynamic instability. Acute over chronic PE caused this severe presentation, confirmed by right heart catheterisation and pulmonary scintigraphy. The challenging diagnosis and management involved transfer to a centre specialised in pulmonary vascular disease. Normalisation of functional and haemodynamic parameters, sustained in 10-year follow-up, was achieved with anticoagulation and triple therapy with prostanoids as a bridge to pulmonary thromboendarterectomy. Targeted medical therapy, not standard at that time, was crucial to recovering conditions for transfer. An individualised approach, integrating multidisciplinary pulmonary hypertension expertise, provides the basis for the best care for CTEPH.
与急性肺栓塞（PE）不同，慢性血栓栓塞性肺动脉高压（CTEPH）中的血栓溶解效果不佳，导致肺动脉内出现重构和纤维化改变。作者报告了一例 60 多岁的男性病例，该患者患有真性红细胞增多症，正在服用华法林，曾因急性 PE 住院。一年后，他因右心衰竭和血流动力学不稳定再次入院。急性 PE 加重慢性 PE 导致了这种严重的表现，这一诊断在右心导管检查和肺闪烁扫描中得到了证实。由于涉及到转移到专门治疗肺血管疾病的中心，因此诊断和管理具有挑战性。在 10 年的随访中，通过抗凝和前列腺素类药物三联治疗作为肺血栓内膜切除术的桥梁，实现了功能和血流动力学参数的正常化，并得以维持。当时并未采用标准治疗方法的靶向药物治疗对于病情的恢复和转移至关重要。个体化的治疗方法，整合了多学科肺动脉高压专业知识，为 CTEPH 的最佳治疗提供了基础。
