Chronic thromboembolic pulmonary hypertension
Chronic thromboembolic pulmonary hypertension
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DOI：
10.1183/09031936.04.00079704
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发表时间：
2004-04-01
影响因子：
24.3
通讯作者：
Simonneau, G
中科院分区：
文献类型：
--
作者：
Dartevelle, P;Fadel, E;Simonneau, G
肺动脉高压是一种长期被忽视的严重疾病。然而，在过去的20年里，胸科医生、心脏病专家和胸科外科医生对这种疾病表现出越来越多的兴趣，因为新的治疗方法的发展改善了患者的预后和生活质量，包括肺移植和前列环素治疗。慢性血栓栓塞性肺动脉高压(CTEPH)可以通过复杂的外科手术：肺血栓内膜切除术治愈。肺血栓内膜切除术是在低温和全循环停搏的情况下进行的。由于50%的患者没有临床上明显的急性肺栓塞发作，诊断CTEPH可能很困难。肺核素扫描显示节段性不匹配的灌注缺损区是发现CTEPH的最佳诊断工具。肺血管造影术确认诊断，并根据病变的近端和远端的位置确定动脉内膜切除术的可行性。血管造影术必须是完美的，每个肺的整个动脉树都能在同一张照片上捕捉到。病变必须始于肺动脉干或叶动脉水平，才能找到动脉内膜切除术的方案。当血流动力学重力与闭塞程度一致时，肺血栓内膜切除术可以在围手术期死亡率最低的情况下进行，几乎在所有病例中都能提供明确的、极佳的功能结果。
Pulmonary arterial hypertension is a severe disease that has been ignored for a long time. However, over the past 20 yrs chest physicians, cardiologists and thoracic surgeons have shown increasing interest in this disease because of the development of new therapies, that have improved both the outcome and quality of life of patients, including pulmonary transplantation and prostacyclin therapy.Chronic thromboembolic pulmonary arterial hypertension (CTEPH) can be cured surgically through a complex surgical procedure: the pulmonary thromboendarterectomy. Pulmonary thromboendarterectomy is performed under hypothermia and total circulatory arrest.Due to clinically evident acute-pulmonary embolism episodes being absent in >50% of patients, the diagnosis of CTEPH can be difficult. Lung scintiscan showing segmental mismatched perfusion defects is the best diagnostic tool to detect CTEPH.Pulmonary angiography confirms the diagnosis and determines the feasability of endarterectomy according to the location of the disease, proximal versus distal. The technique of angiography must be perfect with the whole arterial tree captured on the same picture for each lung. The lesions must start at the level of the pulmonary artery trunk, or at the level of the lobar arteries, in order to find a plan for the endarterectomy.When the haemodynamic gravity corresponds to the degree of obliteration, pulmonary thromboendarterectomy can be performed with minimal perioperative mortality, providing definitive, excellent functional results in almost all cases.