Chronic pulmonary thromboembolism with pulmonary hypertension is a rare but most unique syndrome in the broad spectrum of pulmonary embolism. This report describes a successful pulmonary thromboendarterectomy performed for a totally occluded right pulmonary artery on a 43 year old man who presented with positive cardiolipin antibodies. The surgery was performed through a median sternotomy with cardiopulmonary bypass and intermittent periods of deep hypothermic circulatory arrest. We are convinced that this method allows for complete removal of the thrombotic obstruction and should be the procedure of choice for patients with very proximal obstruction of a pulmonary artery.