肺动静脉瘘相关性脑梗死的临床特点

Clinical characteristics of pulmonary arteriovenous fistula related ischemic stroke

目的:分析肺动静脉瘘(PAVF)相关性脑梗死的临床特征和治疗方案,梳理PAVF相关性脑梗死的诊疗流程。方法:对2015年1月至2019年1月就诊于福建医科大学附属第一医院的6例PAVF相关性脑梗死患者的临床表现、颅脑磁共振成像、脑血管影像、反常性栓塞风险(RoPE)评分、增强经颅多普勒超声(cTCD)、增强经食道超声心动图(cTEE)、胸部CT和肺血管成像特点进行分析和总结,对PAVF患者行介入栓塞或药物保守治疗,随访1.5~5.5年。结果:入组6例患者均为中青年患者,男女比例1∶2,RoPE评分6~7分。1例为首发脑卒中,5例为复发性脑卒中。前后循环均可累及。2例为肯定的遗传性出血性毛细血管扩张症(HHT)合并PAVF,2例为可疑的HHT合并PAVF,2例为散发型PAVF。6例患者cTCD均呈Ⅳ级的大量右向左分流,cTEE表现为延迟的左心房大量气泡影,其中1例合并卵圆孔未闭(PFO)。6例患者均经肺部CT增强确诊,3例PAVF病灶位于左下肺,其余3例病灶分别位于左上肺、右下肺、右中肺,均为单纯型PAVF。对5例患者行经导管介入栓塞治疗,术后行cTCD复查右向左分流,结果4例阴性,1例合并PFO者术后仍有少量潜在分流。另外1例患者选择抗血小板药物保守治疗。对全部患者随访1.5~5.5年,均未再复发脑卒中。结论:PAVF相关性脑梗死患者再发脑梗死的风险很高,颅脑影像学无特异性表现。对于RoPE评分≥6分的隐源性脑卒中患者,应行cTCD筛查右向左分流,对阳性者进一步行cTEE筛查心内/心外分流,提示心外分流并疑诊PAVF者,需行肺部增强CT确诊。对确诊为PAVF者可选经导管栓塞或手术切除,并用cTCD评估治疗效果。

Objective To investigate the clinical characteristics,treatments and prognosis of pulmonary arteriovenous fistula(PAVF)related ischemic stroke,as well as summarize the diagnosis and treatment process to screen and confirm PAVF related ischemic stroke.Methods The clinical data of six PAVF related ischemic stroke patients in the First Affiliated Hospital of Fujian Medical University from January 2015 to January 2019,including clinical manifestations,brain magnetic resonance imaging,risk of paradoxical embolism(RoPE)score,contrast-enhanced transcranial Doppler(cTCD),contrast transesophageal echocardiography(cTEE),chest CT and pulmonary arteriography,were analyzed.PAVF patients were treated with interventional catheter embolization or conservative medication.All patients were followed up for 1.5 to 5.5 years.Results All the six PAVF related ischemic stroke patients were young and middle-aged.The ratio of men to women was 1∶2.RoPE scores ranged from 6 to 7 points.Recurrent stroke was seen in five patients,and only one patient had primary stroke.Both anterior and posterior cerebral circulation could be involved.Hereditary hemorrhagic telangiectasia(HHT)was common in PAVF patients.Of the six patients,two had definite HHT with PAVF,two had suspicious HHT with PAVF,and the other two had sporadic PAVF.In all the six cases,a gradeⅣright-to-left shunt(RLS)was observed on cTCD,and a mass of delayed microbubbles in the left atrium appeared on cTEE.One patient was diagnosed with patent foramen ovale(PFO).Lung enhanced CT was applied in all the patients.In three cases,PAVF was found located in left lower lobe.And in other three cases,lesions were located in left upper lobe,right lower lobe and right middle lung,separately.All cases were confirmed with simple type PAVF.Transcatheter embolization was performed to resolve PAVF in five patients.Of five post-operative patients,four cases showed no sign of RLS on cTCD.One patient combined with PFO still remained a small amount of potential RLS after embolization.One patient chose aspirin for conservative medication.All patients had no recurrence of ischemic stroke during the 1.5 to 5.5-year follow-up.Conclusions PAVF related ischemic stroke patients have a high recurrence rate of ischemic stroke.PAVF related ischemic stroke has no specific brain imaging characteristics.cTCD is recommended for cryptogenic stroke patients with a high RoPE score(≥6 points)to detect RLS.If a patient turns out to be positive for RLS,cTEE could help differentiate intracardiac shunt from extracardiac shunt.For patients with cTEE suggesting extracardiac shunt,lung enhanced CT scan is necessary to confirm PAVF diagnosis.Patients who are diagnosed as PAVF can choose transcatheter embolization or surgical resection.cTCD could be used to evaluate the treatment effect.