Ende-Verhaar Yvonne M, Huisman Menno V, Klok Frederikus A
Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands.
Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands.
Thromb Res. 2017 Mar;151:1-7. doi: 10.1016/j.thromres.2016.12.026. Epub 2017 Jan 5.
Chronic thromboembolic pulmonary hypertension (CTEPH) is the most severe long term complication of acute pulmonary embolism (PE). Untreated, CTEPH is associated with a very poor prognosis and high risk of mortality, although curation can be achieved by surgical removal of the obstructive endothelialised thromboemboli from the pulmonary arteries. Early CTEPH diagnosis may improve surgical possibilities and patients outcome. Currently, early diagnosis of CTEPH is a major challenge as demonstrated by an unacceptable median diagnostic delay of over a year and as a result, surgery is impossible in 40% of patients. Most important reasons for this delay are the non-specific clinical presentation of CTEPH and lack of guideline recommendations with regard to the optimal follow-up of patients with acute PE. Despite compelling reasons to diagnose CTEPH earlier, acute PE is not classified among the conditions that warrant screening for pulmonary hypertension. Meaningful screening programs improve the patients' prognosis, and screening tools should be simple, widely available, non-invasive and acceptable to patients. In this review, we discuss current knowledge of available screening instruments for CTEPH, provide recommendations for clinical practice and expand on future developments of this particular subject.
慢性血栓栓塞性肺动脉高压（CTEPH）是急性肺栓塞（PE）最严重的长期并发症。未经治疗的CTEPH预后很差且死亡风险高，不过通过手术清除肺动脉中阻塞性内皮化血栓栓子可实现治愈。早期CTEPH诊断可能会改善手术可能性及患者预后。目前，CTEPH的早期诊断是一项重大挑战，超过一年的不可接受的中位诊断延迟就证明了这一点，结果40%的患者无法进行手术。这种延迟的最重要原因是CTEPH的临床表现不具特异性，以及缺乏关于急性PE患者最佳随访的指南建议。尽管有充分理由更早诊断CTEPH，但急性PE并未被列为需要筛查肺动脉高压的疾病。有意义的筛查项目可改善患者预后，且筛查工具应简单、广泛可用、非侵入性且为患者所接受。在本综述中，我们讨论了目前关于CTEPH现有筛查工具的知识，为临床实践提供建议，并阐述该特定主题的未来发展。
