Idiopathic isolated pulmonary arteritis with chronic cor pulmonale

Multiple obstructions of the proximal pulmonary arteries were found in a patient with pulmonary hypertension and chronic cor pulmonale without any systemic arterial lesions. An operation to relieve severe stenosis at the right main pulmonary artery provided great clinical improvement. Pathologic examination of the biopsied specimen revealed a nonspecific panarteritis with giant cells consistent with idiopathic isolated pulmonary arteritis.