Burke C M, Safai C, Nelson D P, Raffin T A
Am Rev Respir Dis. 1986 Aug;134(2):334-9. doi: 10.1164/arrd.1986.134.2.334.
Although first reported 90 yr ago, the natural history of pulmonary arteriovenous malformations (PAVM) is still incompletely understood. We review historical perspectives and pathologic classification of this rare disease and identify etiologic factors and clinical features in the 450 reported cases. The many different diagnostic modalities currently available are described, and a logical sequence of investigations is suggested. Pulmonary angiography remains mandatory prior to definitive treatment. The available evidence strongly suggests that morbidity and mortality in untreated PAVM exceeds that associated with either surgical resection or embolic occlusion. Thus, definitive treatment should be offered in all cases, with the possible exception of asymptomatic patients with small lesions who have no evidence of Osler-Weber-Rendu disease. Embolotherapy is now the treatment of choice in institutions with the necessary expertise and facilities, notwithstanding the absence of long-term follow-up for patients treated this way. It is suggested that increased documentation of long-term morbidity and mortality in both treated and untreated patients with PAVM will lead to greater understanding and more effective management of this enigmatic condition.
尽管肺动静脉畸形（PAVM）90年前就首次被报道，但对其自然病史仍未完全了解。我们回顾了这种罕见疾病的历史观点和病理分类，并在450例报告病例中确定了病因和临床特征。描述了目前可用的多种不同诊断方法，并提出了合理的检查顺序。在进行确定性治疗之前，肺血管造影仍然是必需的。现有证据有力地表明，未经治疗的PAVM的发病率和死亡率超过了与手术切除或栓塞闭塞相关的发病率和死亡率。因此，除了无症状的小病灶患者且无奥斯勒-韦伯-伦杜病证据外，所有病例均应进行确定性治疗。尽管缺乏对接受这种治疗的患者的长期随访，但在具备必要专业知识和设施的机构中，栓塞治疗现在是首选治疗方法。建议增加对接受治疗和未接受治疗的PAVM患者的长期发病率和死亡率的记录，这将有助于更好地了解并更有效地管理这种神秘疾病。
