复杂型肺动静脉瘘介入封堵治疗一例并文献复习

Interventional embolization therapy for complex pulmonary arteriovenous fistula:case report and literature review

目的探讨复杂型肺动静脉瘘(PAVF)的临床特点及诊疗措施,丰富诊疗经验。方法对四川大学华西医院1例复杂型PAVF患者的发病过程、临床表现、影像学特点及诊治经过进行分析。以"complex pulmonary arteriovenous fistula(malformation)"在PubMed数据库进行检索,以"复杂型动静脉瘘"在中国知网及万方数据库中进行检索,检索时间为1997年1月至2018 4月年,筛选文献并进行文献复习。结果患者女性,47岁,因"反复鼻衄40年,间断咯血20年,头痛、头晕,伴胸痛、胸闷、乏力、左半身感觉障碍4年,加重3个月"于2018年1月23日就诊。胸部CT血管造影提示右肺中叶内外侧段、下叶外基底段及左肺下叶内前基底段、外基底段多发结节影,增强扫描见增粗肺动脉血管与上述病灶相连,边缘见伴行的粗大引流静脉(多为肺静脉)。肺部血管重建可见:复杂型PAVF异常的分支血管。诊断为:复杂型PAVF。入院后行PAVF介入封堵术治疗后好转出院,末次随访疗效满意。文献检索并经过严格筛选获得文献报道6篇,其中中文文献4篇,英文文献2篇,包含10例患者,其中男8例,女2例,平均年龄(9.7±7.0)岁,临床表现多为活动后气短、发绀、咯血,均经PAVF介入封堵术治疗后好转。结论复杂型PAVF是一种非常罕见的肺部血管发育畸形性疾病,临床表现主要有低氧血症、劳力性呼吸困难、咯血等,首选介入封堵术治疗,短期随访,临床效果满意。

Objective To explore the clinical characteristics and improve the knowledge of diagnosis and treatment of complex pulmonary arteriovenous fistula(PAVF) as well as enrich the experience of diagnosis and treatment of the disease. Methods A retrospective analysis of pathogenetic process clinical manifestations, imaging features and diagnosis and treatment was conducted on one case of complex PAVF. The literature review was carried out with'complex pulmonary arteryovenous fistula(malformation)' as the research terms in English and Chinese respectively in CNKI, WanFang and PubMed database. Search time ranged from January 1997 to April 2018, and the literature was screened and reviewed. Results The patient was a 47-year-old female complained of recurrent epistaxis for 40 years,intermittent hemoptysis for 20 years, headache, dizziness, chest pain, chest tightness for 4 years and the symptoms were aggravated by 3 months, visiting this hospital on January 23, 2018. Pulmonary CT angiography revealed multiple nodules in internal and external segment and outer basal segment of right lung, anterior basal segment and outer basal segment of left lung. CT enhanced scan showed that the thickened pulmonary artery was connected with the above lesion, and the edge was accompanied by large draining veins. Pulmonary artery revascularization showed complex PAVF abnormal branches. The diagnosis was complex PAVF, and interventional embolization therapy was carried out and curative effect was satisfactory during the follow-up. A total of 6 literatures were reviewed in above-mentioned databases, including4 Chinese literatures and 2 English literatures, containing 10 patients, including 8 males and 2 females, with an average age of(9.7±7.0) years. Most of the clinical manifestations were shortness of breath after exercise, cyanosis and hemoptysis and all patients were cured and discharged after interventional embolization treatment except for 1 patient refused treatment. Conclusions Complex PAVF is a very rare pulmonary vascular malformation. The clinical manifestations mainly include hypoxemia, dyspnea, hemoptysis, and the preferred treatment is interventional embolization, which has a satisfactory clinical effect at a short-term follow-up.