Patients with pulmonary arteriovenous malformations (PAVMs) are at risk for multiple complications and require close follow-up. We investigated the reproducibility of the 6-minute walk test (6MWT) and exercise stress test (EST) for the evaluation of low oxygen saturation in patients with PAVMs. Twenty-two patients with PAVMs, most of whom had hereditary hemorrhagic telangiectasia (HHT), participated in a Human Investigations Committee-approved protocol. Patients ranged from 9 to 74 years of age (mean 28) and had a broad spectrum of anatomic subtypes of PAVMs, including focal and diffuse. Standard 6MWT and cycle ergometry EST were both performed twice with adequate rest between tests. Heart rate (HR) and oxygen saturation were measured at the beginning and end of each test. Distance walked and maximum resistance was also recorded. The intraclass correlation coefficients (r(i)) at the end of 6MWT were as follows: HR (r(i) = 0.940; 95% confidence interval [CI] 0.863-0.975), oxygen saturation (r(i) = 0.973; 95% CI 0.933-0.989), and distance (r(i) = 0.942; 95% CI 0.867-0.975). The r(i)s at the end of EST were as follows: HR (r(i) = 0.941; 95% CI 0.865-0.975), oxygen saturation (r(i) = 0.993; 95% CI 0.982-0.997), and maximum resistance (r(i) = 0.941; 95% CI 0.864-0.975). 6MWT and EST were reproducible measures of exercise capacity and oxygen saturation and are potential adjunct tests in the follow-up assessment for patients with PAVMs.