Major vessel chronic thromboembolic pulmonary hypertension is potentially remediable by thromboendarterectomy. The diagnosis often has been delayed because a lung perfusion scan demonstrated modest defects thought to be incompatible with severe vascular obstruction. To define the relationships between perfusion scan abnormalities, angiographic findings, and hemodynamic data, we analyzed 25 consecutive patients with chronic major vessel thromboembolic pulmonary hypertension who subsequently underwent thromboendarterectomy. We found that the perfusion lung scan consistently caused us to underestimate the severity of pulmonary arterial obstruction as defined by pulmonary angiography. Furthermore, there was no significant correlation between the severity of hemodynamic compromise and the extent of obstruction defined by perfusion scan or angiogram. These findings suggest that, when pulmonary hypertension is known or suspected, the diagnosis of correctable, chronic major vessel obstruction should be pursued by angiography and hemodynamic assessment even though the perfusion scan may demonstrate only two segmental defects.