Simonneau G, Azarian R, Brenot F, Dartevelle P G, Musset D, Duroux P
Service de Pneumologie et de Réanimation Respiratoire and Radiologie, Hôpital Antoine Béclère, Clamart, France.
Chest. 1995 Jan;107(1 Suppl):52S-55S. doi: 10.1378/chest.107.1_supplement.52s.
Chronic thromboembolic pulmonary hypertension (CT-E PH) is a rare and aberrant outcome of acute pulmonary embolism. Because it has become a potentially curable form of pulmonary hypertension, the frequency of recognized cases has increased. We report a case series of 72 patients with CT-E PH evaluated in our institution between 1984 and 1993, and discuss diagnostic clues and therapeutic approaches. All patients complained of dyspnea on exertion, a history of acute thromboembolic event, and lung murmurs were found in 60% and 17% of patients, respectively. The presence of a disorder of coagulation was found in 30% of the patients tested, the most common abnormality being lupus anticoagulant. The key noninvasive study for diagnosis was the lung perfusion scan which showed at least one segmental or larger perfusion defect in all patients. Pulmonary angiography confirmed the diagnosis in all cases, and sometimes associated to intravascular ultrasound imaging, established the feasibility of thromboendarterectomy. Medical therapy included the use of long-term oral anticoagulant, and in the case of lower limb venous thrombosis, inferior vena cava filtration. Finally two surgical procedures were discussed in selected patients: thromboendarterectomy and lung transplantation. Since 1988, eight patients have benefited from lung transplantation (six patients are still alive), and 11 patients underwent thromboendarterectomy which was successful in 9 patients with a dramatic functional and hemodynamic improvement.
慢性血栓栓塞性肺动脉高压（CT-E PH）是急性肺栓塞罕见且异常的后果。由于它已成为一种潜在可治愈的肺动脉高压形式，已确诊病例的数量有所增加。我们报告了1984年至1993年间在我们机构评估的72例CT-E PH患者的病例系列，并讨论诊断线索和治疗方法。所有患者均主诉劳力性呼吸困难，有急性血栓栓塞事件史，分别有60%和17%的患者发现肺部杂音。在30%接受检测的患者中发现存在凝血障碍，最常见的异常是狼疮抗凝物。诊断的关键非侵入性检查是肺灌注扫描，所有患者均显示至少一个节段性或更大的灌注缺损。肺血管造影在所有病例中均确诊，有时结合血管内超声成像，确定了血栓内膜剥脱术的可行性。药物治疗包括长期口服抗凝剂，对于下肢静脉血栓形成的病例，采用下腔静脉滤器。最后，对部分患者讨论了两种外科手术：血栓内膜剥脱术和肺移植。自1988年以来，8例患者受益于肺移植（6例患者仍存活），11例患者接受了血栓内膜剥脱术，其中9例成功，功能和血流动力学有显著改善。
