The bronchogram (Fig 3) was consistent with the findings of bronchoscopy and the tomograms. The left upper lobe bronchus was absent, leaving only a dimple at the usual site of its takeoff. There were two right upper bronchi, one from the trachea and the other from right main bronchus. Cytologic studies of bronchial lavage and sputum denied any malignancy.
Pulmonary angiography showed dilatation of the pulmonary arteries. There were no left upper branches, and all pulmonary veins perfused to the left atrium. A CT scan of the lung (Fig 4) demonstrated an unusual position of the heart, being displaced to the left and anterior to the chest wall just posterior to the sternum. The dilated pulmonary arteries and abnormal vessels could be seen. The azygos vein was dilated approximately up to the width of the spinal column. Intercommunication of both lungs could be seen. An abdominal CT scan demonstrated a dilated azygos vein, but the liver, gallbladder, and spleen were normal in number and were in the normal positions. On fluoroscopy the movement of the cardiac wall was unusual; and together with the findings on the chest x-ray film, a congenital defect of the left pericardium was suspected.
Table 1—Cardiac Catheterization
Sa02 (%) Pressure (mm Hg)
Right cardiac catheterization was first attempted through the saphenous vein, but the catheter stopped at the top of superior vena cava and could not get into the right atrium. Another attempt from the arm vein could guide the catheter into the right atrium. Data from cardiac catheterization showed normal pressure and normal Po2 levels in any parts of the heart examined (Table 1). The hepatic vein returned directly into the right atrium.
FIGURE 3. Bronchogram showing blind pouch of left upper bronchus and bronchus of right upper lobe.
Inferior venacavography demonstrated that opaque material injected from the right iliac vein flowed through the postrenal inferior vena cava, azygos vein, and superior vena cava into the right atrium. An anomalous inferior vena cava with azygos vein continuation was diagnosed.
FIGURE 4. A (top), Thoracic CT scan showing heart just behind sternum. В (bottom), Abdominal CT scan showing dilated azygos vein, inferior vena cava, and azygos vein.
There was neither malignant disease of the lungs nor heart disease, and the final diagnosis was a triad of congenital defects of the left pericardium, dysplasia of the left upper lobe, and anomalous inferior vena cava with azygos continuation (Fig 5).
FIGURE 5. Summary of anomalies of major veins and bronchial trees. SVC, Su¬perior vena cava; RA, right atrium; RY right ventricle; and VCI, inferior vena cava.
The patient has been quite well, and has returned for a checkup without any complaints in January 1989.