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 grade Ⅳright-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.