Isolated pulmonary arteriovenous fistula without Rendu-Osler-Weber disease as a cause of cryptogenic stroke.

There has been uncertainty as to whether a right to left shunt through an isolated pulmonary arteriovenous fistula (P-AVF) without Rendu-Osler-Weber (ROW) disease can cause paradoxical brain embolism. A population of 747 acute ischaemic stroke patients was examined to determine the frequency and clinical characteristics of those patients who had an isolated P-AVF. The presence of a P-AVF was determined as follows. On patients with a stroke of undetermined cause, both transoesophageal echocardiography and transcranial Doppler with saline contrast medium was performed to detect a right to left shunt. If a P-AVF was then suspected, selective pulmonary angiography and enhanced chest CT was performed to confirm the presence of the P-AVF. Four patients (0.5%) were diagnosed as having a stroke associated with an isolated P-AVF. All the patients were middle-aged women (mean age 61 years). In all these patients, the P-AVF could not have been suspected on physical findings or chest x ray. The P-AVF was always single and located in the lower lobe. All the patients had asymptomatic deep venous thrombosis, and three patients developed pulmonary embolism. As D-dimer and thrombin-antithrombin complex were elevated in all patients, this indicated an activation of both fibrinolytic and thrombin activity. Our results show that an isolated P-AVF without ROW disease can cause paradoxical brain embolism. Thus, the existence of an isolated P-AVF as a right to left shunt in patients with a stroke of unknown origin should not be overlooked, even if a P-AVF is not suggested by the initial physical findings or chest x ray.