Ference B A, Shannon T M, White R I, Zawin M, Burdge C M
Department of Diagnostic Radiology, Yale University School of Medicine, New Haven, Conn 06520-8042.
Chest. 1994 Nov;106(5):1387-90. doi: 10.1378/chest.106.5.1387.
The occurrence of significant pulmonary hemorrhage associated with pulmonary arteriovenous malformations (PAVMs) and hereditary hemorrhagic telangiectasia (HHT) and the incidence of PAVMs in family members of patients with PAVMs and HHT are poorly defined. We reviewed our experience in 143 patients with PAVMs and HHT. Eleven (8 percent) of the 143 patients with HHT and PAVMs had a history of either massive hemoptysis or of hemothorax which required hospitalization. One patient died directly related to the pulmonary hemorrhage. There were four men and seven women. Three of the seven women experienced pulmonary hemorrhage during pregnancy. Seven of the 11 families participated in screening for PAVMs. Thirty-six (80 percent) of the 45 screened family members were found to have HHT. Thirteen (36 percent) of the 36 family members with HHT were proven to have PAVMs by pulmonary angiography. Pulmonary hemorrhage due to spontaneous rupture of the PAVM is a potentially life-threatening complication that should be treated aggressively with transcatheter embolotherapy. It occurs more frequently than previously recognized in patients with PAVMs and HHT. In addition, because of the increased incidence of PAVMs in family members of patients with HHT and PAVM, screening of family members with HHT is recommended especially in women of childbearing age.
与肺动静脉畸形（PAVM）和遗传性出血性毛细血管扩张症（HHT）相关的严重肺出血的发生率，以及PAVM患者和HHT患者家庭成员中PAVM的发病率，目前尚不清楚。我们回顾了143例PAVM和HHT患者的治疗经验。143例HHT和PAVM患者中有11例（8%）有大量咯血或血胸病史，需要住院治疗。1例患者直接死于肺出血。其中男性4例，女性7例。7名女性中有3名在怀孕期间发生肺出血。11个家庭中有7个参与了PAVM筛查。在45名接受筛查的家庭成员中，有36名（80%）被发现患有HHT。36名患有HHT的家庭成员中有13名（36%）经肺血管造影证实患有PAVM。PAVM自发性破裂导致的肺出血是一种潜在的危及生命的并发症，应积极采用经导管栓塞治疗。它在PAVM和HHT患者中的发生率比以前认识到的更高。此外，由于HHT和PAVM患者家庭成员中PAVM的发病率增加，建议对HHT家庭成员进行筛查，尤其是育龄妇女。
