The syndrome of chronic thrombosis of the major pulmonary arteries

Abstract

Two cases of thrombosis of the major pulmonary arteries are presented. One, illustrating the onset and early development of the syndrome, presented as a case of pulmonary infarction with baffling cardiac signs. Sudden death ensued after a short course. The other ran a chronic course with the development of right ventricular failure, congestive cirrhosis and psychosis. Angiocardiography in the latter case led to the correct diagnosis prior to death. Clinical features which suggest the diagnosis are: (1) history of thromboembolic disease; (2) progressive marked right ventricular heart failure of obscure cause responding poorly to therapy; (3) dry lungs despite the presence of dyspnea, cyanosis, peripheral edema and ascites; (4) new or changing heart murmurs; (5) syncopal attacks or periods of mental confusion during the course of the illness. Roentgen studies are invaluable in making the diagnosis. Routine x-rays of the chest frequently demonstrate blunting or deformity of one or both pulmonary arteries associated with diminution in the vascular markings of the lung. Angiocardiography can be used to confirm the presence of occlusion of the pulmonary artery. There is reason to believe that this syndrome occurs more often than indicated by the number of cases reported. Only one patient is known to have been treated successfully. However, recent advances in cardiovascular surgery, the development of anticoagulants, and perhaps enzyme therapy should achieve effective treatment of more patients with this syndrome.