A 61-year-old man with hypogammaglobulinemia presented with recent onset of shortness of breath and pleuritic chest pain. He was slightly cyanotic. Results of an examination of the chest and cardiovascular system were unremarkable. There was no history of cardiac disease. A chest radiograph showed a few small areas of peripheral airspace opacity. Perfusion lung scintigraphy was performed to assist in the evaluation of pulmonary embolism after injection of technetium 99m ( 99m Tc) macroaggregated albumin (MAA) into a right arm vein. Given the findings of this examination, contrast material-enhanced computed tomography (CT) of the chest was performed on the same day. A 100-mL dose of iopamidol 300 was injected into a right arm vein at a rate of 4 mL/sec, and image acquisition commenced 20 seconds after the start of the injection. Another episode of pleuritic chest pain prompted another perfusion examination 19 days later. This time, 99m Tc-MAA was injected into a left arm vein.
IMAGING FINDINGSThe first perfusion lung scintigram, obtained after 90 MBq of 99m Tc-MAA was injected into a right arm vein, shows activity predominantly in the systemic arterial perfusion territoryparticularly in the brain, kidneys, spleen, thyroid, bowel, and myocardium-which indicates a right-to-left shunt (Fig 1). A chest CT scan obtained after contrast material was injected into a right arm vein confirms a right-to-left shunt caused by the right-sided superior vena cava (SVC) draining into the left atrium (Fig 2). In addition, this CT scan shows a persistent left-sided SVC.A second perfusion lung scintigram was obtained 19 days after the first scintigram was obtained, with injection of a radiotracer into a left arm vein. The appearance of the second scintigram was normal, with no evidence of pulmonary embolism or systemic shunting (Fig 3).
DISCUSSIONThe key to establishing a diagnosis lies in reconciling the discordant findings on the two perfusion lung scintigrams by identifying and interpreting two vascular anomalies on the CT scan.The first perfusion lung scintigram (Fig 1) was obtained by injecting the radiotracer into a right arm vein, and the systemic distribution of activity indicates a right-to-left shunt. Visual estimation of the amount of activity in the systemic circulation versus the pulmonary circulation suggests a highgrade shunt, which is at odds with the patient's mild cyanosis and asymptomatic survival into middle age.The contrast-enhanced chest CT scan (Fig 2), which was obtained by injecting contrast material into a right arm vein, confirmed a right-to-left shunt, which was caused by the anomalous drainage of the right-sided SVC into the left atrium. This results in complete right-to-left shunting of the venous return from the right arm and right jugular territories. This shunt explains the systemic distribution of radiotracer that is seen on the first perfusion lung scintigram and the marked paucity of activity in the lungs. The small amount of activity observed in the lungs can be explained on the basis of the bronc...