Azarian R, Brenot F, Sitbon O, Musset D, Grimon G, Boyer-Neumann C, Rain B, Dartevelle P, Simonneau G
Service de Pneumologie et de Réanimation respiratoire, Hôpital Antoine Béclère, Clamart.
Presse Med. 1994 Jun 11;23(22):1017-22.
Chronic thrombo-embolic pulmonary hypertension 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 70 patients with chronic thromboembolic pulmonary hypertension evaluated in our institution between 1984 and 1993, and discuss diagnostic clues and therapeutic approaches.
All patients complained of dyspnoea on exertion. A history of acute thrombo-embolic events and lung murmurs were found in 60% and 17% of patients respectively. Coagulation disorders were found in 30% of the patients tested; the most common abnormality was lupus anticoagulant. The key non-invasive study for diagnosis was the lung perfusion scan which showed at least one segmental or wider perfusion defects in all patients. Pulmonary angiography confirmed the diagnosis in all cases and, sometimes associated with intravascular ultrasound imaging, established the feasibility of thromboendarterectomy. Medical therapy included the use of long-term oral anticoagulant, and in 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 leading to dramatic functional and haemodynamic improvement.
Chronic thrombo-embolic pulmonary hypertension is a severe, sometimes fatal, disease which can be successfully treated by pulmonary thromboendartectomy and lung transplantation.
慢性血栓栓塞性肺动脉高压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70例慢性血栓栓塞性肺动脉高压患者的病例系列，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。分别有60%和17%的患者有急性血栓栓塞事件史和肺部杂音。在接受检测的患者中，30%发现有凝血障碍；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广泛的灌注缺损。肺血管造影在所有病例中均确诊，有时与血管内超声成像相结合，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患者，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例仍存活），11例患者接受了血栓内膜剥脱术，其中9例成功，导致功能和血流动力学显著改善。
慢性血栓栓塞性肺动脉高压是一种严重的、有时致命的疾病，可通过肺动脉血栓内膜剥脱术和肺移植成功治疗。
