A patient with chronic fibrous mediastinitis presented with bronchial, superior vena caval, and pulmonary venous obstruction, pulmonary hypertension, and pulmonary fibrosis. Pulmonary venous obstruction was confirmed at surgery and explains the hemodynamic findings of elevated pulmonary artery and pulmonary wedge pressures despite low left atrial pressure. Pulmonary function studies showed marked alterations in ventilation-distribution, diffusion, work of breathing, and fixed airway resistance. It is concluded that the pulmonary arterial hypertension was due, in large part, to the pulmonary venous hypertension caused by pulmonary venous obstruction. Unsuccessful surgical intervention has been followed by 10 months of observation during which aldosterone inhibition together with use of chlorothiazide has prevented recurrence of heart failure and pleural effusion. The relationship between the physiological abnormalities and the patient's symptoms is discussed along with the possible relationship between pulmonary venous obstruction and pulmonary fibrosis.

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Thirty patients with primary cardiac compression due to constrictive pericarditis, lax effusion, or cardiac tamponade and an additional seven patients with spurious evidence of cardiac compression or with pericardial effusion playing an unimportant role in the circulatory disorder were studied. Rather stringently defined physical findings were sought which might allow discrimination between cardiac disorders. The following conclusions are drawn from the results. 1. Constrictive pericarditis is associated with venous and auscultatory phenomena which do not allow separation from other forms of heart disease causing congestive heart failure. Kussmaul's sign is present in less than 40%; pulsus paradoxus as classically defined is rare. 2. In lax pericardial effusion, Kussmaul's sign and Friedreich's sign, along with third heart sounds, are not present. Pulsus paradoxus is inconstant with tranquil breathing but is regularly induced by deep inspiration. There is inspiratory decrease in venous pressure and pericardial pressure. Cardiac index is normal and venous pressure is less than 12 mm Hg. Circulatory distress is not apparent. 3. Tamponade induces signs of circulatory distress and is regularly characterized by pulsus paradoxus but Friedreich's sign, a third heart sound, as well as Kussmaul's venous sign, are absent. The venous pressure exceeds 12 mm Hg. There is an inspiratory decrease in venous pressure and pericardial pressure. The low cardiac index is usually relieved by tap. When aortic stenosis is present, respiratory variation in left ventricular systolic pressure may not be reflected by clinical pulsus paradoxus. 4. Spurious signs of cardiac compression may be due to (1) respiratory disease, (2) severe myocardial disease and incidental effusion, or (3) obesity. In the respiratory disease pulsus paradoxus, normal cardiac index, low venous pressure, and venous and pericardial-pressure decrease with inspiration are present. The second group does not show pulsus paradoxus and the elevated venous pressure, diastolic dip, and third heart sounds are due to heart failure. Obesity may cause pulsus paradoxus and increased peripheral venous pressure, which does not reflect central venous pressure. These findings seem related to inspiratory collapse of extrathoracic vessels.

The role of venous passage of indicator from different venous injection sites on the genesis of right heart and pulmonary artery dilution curves was examined. Right heart and pulmonary artery thermodilution curves were recorded after injection of cool dye into commonly used portals—superior vena caval, right atrial, and inferior vena caval—and the contour compared with the subsequent femoral artery dye dilution curve. With superior vena caval or right atrial injection, the contour and disappearance slopes of the pulmonary artery curve bore an extremely variable relationship to those of the femoral artery curve. In sharp contrast, inferior vena caval injection yielded pulmonary artery curves with disappearance slopes which were highly correlated with the femoral artery slope ( r = .99). With inferior vena caval injection, considerable temporal dispersion and spatial dispersion of indicator is found at the right atrial level. With superior vena caval injection distribution mainly occurred beyond the right atrium and even beyond the pulmonary artery in eight out of ten animal studies. The geometry of the venous system may explain this difference. Inaccuracies in flow calculation from right heart dilution curves in dogs would seem to be minimized by inferior vena caval injection.

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