Zhang Xin, Zheng Mingxia, Li Xingjian, Yan Yejun, Yang Caixia, Zhao Zhengang, Feng Yuan, Chen Mao
Department of Cardiology, West China Hospital, Sichuan University, Chengdu.
Department of Cardiology.
Medicine (Baltimore). 2019 Jan;98(2):e13922. doi: 10.1097/MD.0000000000013922.
Complex pulmonary arteriovenous fistula (PAVF) is unusual, and even rarer in 2 members of a family. PAVF may not appear on chest X-ray or computed tomography imaging, especially in asymptomatic patients, and therapy is limited. Herein, PAVFs occurring in a mother and daughter are described, with the current standard methods of diagnosis and treatment of PAVF.
A 34-year-old woman and her 13-year-old daughter presented with light cyanosis of the nail beds but were otherwise asymptomatic, and physical examination was unremarkable. Their arterial oxygen saturation levels were low (80-85%).
Angiography led to a diagnosis of PAVF involving the bilateral lung in both women.
The combined use of coils with occluder (patent ductus arteriosus, or PDA) to obstruct the fistula.
After interventional treatment, the patients' arterial oxygen saturation improved rapidly (90-95%). At 6-month follow-up, the patients' symptoms and oxygen saturation were normal.
PAVF is an autosomal dominant disease. Here, the characteristics of the 2 patients were very similar. Using detachable coils and then a PDA occluder is a highly efficient method for treating complex PAVFs.
复杂性肺动静脉瘘(PAVF)较为罕见,在一个家族的两名成员中发病则更为罕见。PAVF可能在胸部X线或计算机断层扫描成像中不显示,尤其是在无症状患者中,且治疗方法有限。本文描述了一对母女发生的PAVF,以及目前PAVF的标准诊断和治疗方法。
一名34岁女性及其13岁女儿表现为甲床轻度发绀,但无其他症状,体格检查无异常。她们的动脉血氧饱和度水平较低(80 - 85%)。
血管造影显示两名女性均患有累及双侧肺的PAVF。
联合使用线圈和封堵器(动脉导管未闭封堵器,即PDA)阻塞瘘管。
介入治疗后,患者的动脉血氧饱和度迅速改善(90 - 95%)。在6个月的随访中,患者的症状和血氧饱和度均正常。
PAVF是一种常染色体显性疾病。在此,两名患者的特征非常相似。使用可分离线圈然后使用PDA封堵器是治疗复杂性PAVF的高效方法。