Abstract：
Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct form of precapillary pulmonary hypertension classified as group 4 by the World Symposium on Pulmonary Hypertension (WSPH) and should be excluded during an episode of acute pulmonary embolism (PE). Patients presenting to emergency departments with sudden onset of signs and symptoms of acute PE may already have a pre-existing CTEPH condition decompensated by the new PE episode. Identifying an underlying and undiagnosed CTEPH during acute PE, while challenging, is an important consideration as it will alter the patients\' acute and long-term management. Differential diagnosis and evaluation require an interdisciplinary expert team. Analysis of the clinical condition, the CT angiogram, and the hemodynamic situation are important considerations; patients with CTEPH usually have significantly higher sPAP at the time of index PE, which is unusual and unattainable in the context of acute PE and a naïve right ventricle. The imaging may reveal signs of chronic disease such as right ventricle hypertrophy bronchial collaterals and atypical morphology of the thrombus. There is no standard for the management of acute on chronic CTEPH. Herein, we provide a diagnostic and management algorithm informed by several case descriptions and a review of the literature.
摘要：
慢性血栓栓塞性肺动脉高压（CTEPH）是一种独特的脉络膜前肺动脉高压，被世界肺动脉高压研讨会（WSPH）归类为第4组，应在急性肺栓塞（PE）发作期间排除。到急诊科就诊的患者突然出现急性PE的体征和症状，可能已经存在因新的PE发作而代偿失调的预先存在的CTEPH状况。在急性PE期间确定潜在的和未诊断的CTEPH，在挑战的同时，是一个重要的考虑因素，因为它将改变患者的急性和长期管理。鉴别诊断和评估需要一个跨学科的专家团队。临床情况分析，CT血管造影,和血流动力学情况是重要的考虑因素;CTEPH患者通常在指数PE时sPAP显著升高,这在急性PE和幼稚右心室的情况下是不寻常和无法实现的。影像学可能会显示出慢性疾病的体征，例如右心室肥大支气管络脉和血栓的非典型形态。尚无治疗慢性CTEPH的标准。在这里，我们通过几个病例描述和文献综述提供了一个诊断和管理算法.
