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Case 74: Right-sided Superior Vena Cava Draining into Left Atrium in a Patient with Persistent Left-sided Superior Vena Cava¹

¹ From the Department of Radiology, The Churchill Hospital, Oxford, England. Received September 3, 2002; revision requested October 31; revision received March 19, 2003; accepted April 14. Address correspondence to P.M.P., Department of Neuroradiology, The Radcliffe Infirmary, Woodstock Rd, Oxford OX2 6HE, England (e-mail: pretoriuspieter@hotmail.com).

Index terms: Diagnosis Please • Venae cavae, abnormalities, 566.1591, 566.1599, 946.142

	HISTORY

TOP HISTORY IMAGING FINDINGS DISCUSSION REFERENCES

 
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 (^99mTc) 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, ^99mTc-MAA was injected into a left arm vein.

	IMAGING FINDINGS

TOP HISTORY IMAGING FINDINGS DISCUSSION REFERENCES

 
The first perfusion lung scintigram, obtained after 90 MBq of ^99mTc-MAA was injected into a right arm vein, shows activity predominantly in the systemic arterial perfusion territory—particularly 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.

View larger version (190K): [in this window] [in a new window] [Download PPT slide]   Figure 1. Anterior and posterior views of the first perfusion lung scintigram obtained after injection of radiotracer into a right arm vein. Activity is predominantly seen in a systemic distribution, particularly in the kidneys (arrowheads on the posterior image), spleen (arrow on the posterior image), brain, thyroid, bowel (arrowheads on the anterior image), and myocardium (arrow on the anterior image).

View larger version (100K): [in this window] [in a new window] [Download PPT slide]   Figure 2a. Chest CT scans acquired 20 seconds after the start of intravenous injection of contrast material into a right arm vein. Images are shown at the (a) level of the aortic arch, (b) bifurcation of the main pulmonary artery, and (c) cardiac chambers. In a, contrast material is present in the right-sided SVC (straight arrow) and aortic arch. Note the unopacified left-sided SVC (curved arrow). In b, the opacified right-sided SVC (straight arrow) and ascending and descending aorta (large arrowheads) are demonstrated. No contrast material is present in the main pulmonary artery (small arrowhead). The left-sided SVC (curved arrow) lies adjacent to the left pulmonary artery. In c, opacified blood from the right-sided SVC (arrow) enters the left atrium (arrowheads).

View larger version (92K): [in this window] [in a new window] [Download PPT slide]   Figure 2b. Chest CT scans acquired 20 seconds after the start of intravenous injection of contrast material into a right arm vein. Images are shown at the (a) level of the aortic arch, (b) bifurcation of the main pulmonary artery, and (c) cardiac chambers. In a, contrast material is present in the right-sided SVC (straight arrow) and aortic arch. Note the unopacified left-sided SVC (curved arrow). In b, the opacified right-sided SVC (straight arrow) and ascending and descending aorta (large arrowheads) are demonstrated. No contrast material is present in the main pulmonary artery (small arrowhead). The left-sided SVC (curved arrow) lies adjacent to the left pulmonary artery. In c, opacified blood from the right-sided SVC (arrow) enters the left atrium (arrowheads).

View larger version (136K): [in this window] [in a new window] [Download PPT slide]   Figure 2c. Chest CT scans acquired 20 seconds after the start of intravenous injection of contrast material into a right arm vein. Images are shown at the (a) level of the aortic arch, (b) bifurcation of the main pulmonary artery, and (c) cardiac chambers. In a, contrast material is present in the right-sided SVC (straight arrow) and aortic arch. Note the unopacified left-sided SVC (curved arrow). In b, the opacified right-sided SVC (straight arrow) and ascending and descending aorta (large arrowheads) are demonstrated. No contrast material is present in the main pulmonary artery (small arrowhead). The left-sided SVC (curved arrow) lies adjacent to the left pulmonary artery. In c, opacified blood from the right-sided SVC (arrow) enters the left atrium (arrowheads).

View larger version (85K): [in this window] [in a new window] [Download PPT slide]   Figure 3. Anterior and posterior views of a perfusion lung scintigram obtained 19 days after the first scintigram was obtained. Radiotracer was injected into a left arm vein.

	DISCUSSION

TOP HISTORY IMAGING FINDINGS DISCUSSION REFERENCES

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 high-grade 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 bronchial arterial supply to the lungs. In addition, the presence of a persistent left-sided SVC on CT scans explains the normal appearance of the lung perfusion scintigram after radiotracer was injected into a left-arm vein (Fig 3), since the persistent left-sided SVC drains into the right atrium via the coronary sinus.

After injection of ^99mTc-MAA in healthy subjects, the particles undergo embolization in pulmonary arterioles and capillaries in accordance with pulmonary arterial blood flow. Approximately 2% of particles are transfered to the systemic circulation via healthy intrapulmonary shunts (1). Activity is therefore detected in the systemic circulation of healthy subjects only if radiopharmaceutical problems have occurred, such as degradation of particles to submicron size (2). Right-to-left shunts of 10%–15% or more are apparent on perfusion lung scintigrams because of activity in organs with high systemic blood flow, such as the kidneys, brain, thyroid, and spleen (3). Depiction of the myocardium, as in this patient, requires an even larger (>39%) shunt (4).

Anomalous systemic venous connection with the left atrium is an unusual congenital cause of a right-to-left shunt. It is most commonly due to a persistent left-sided SVC draining into the left atrium (5). Less commonly, the left atrium may connect with the right-sided SVC, the inferior vena cava, the coronary sinus, the azygos vein, or a hepatic vein (6–8). Rare instances of total anomalous systemic venous connections to the left atrium have also been described (6,8).

A left-sided SVC occurs in approximately 0.3%–0.5% of healthy individuals and 4.4% of patients with congenital heart disease (9,10). The left-sided SVC represents a persistent left anterior cardinal vein and drains into the coronary sinus in 92% of patients. In the remaining 8%, the SVC drains into the left atrium (11). A right-sided SVC is present in 82%–90% of patients with a persistent left-sided SVC; in 25%–35% of these patients, the left innominate vein is present, which allows some cross-flow between the two SVC drainage territories (12). When the left or right SVC connects with the left atrium, the two SVCs may or may not communicate via such an innominate connection. Cyanosis may or may not be present, depending on the volume and direction of flow through this connection. In this patient, the left innominate vein is absent.

A right-sided SVC that drains into the left atrium is a very rare congenital abnormality, and reports of only seven cases could be found in the English-language medical literature (13–19). The SVC may drain into or receive drainage from one or more pulmonary veins before entering the left atrium (8).

At least six cases have been reported in which the right-sided SVC has connections with both atria (20). The embryologic abnormalities underpinning a connection between the SVC and the left atrium are not well understood. Some mechanisms have been proposed to explain the wide range of anomalies described in the literature (8,21).

Anomalous systemic venous connections are rare congenital anomalies that should be suspected when a right-to-left shunt is detected with perfusion lung scintigraphy in a patient without a history of congenital cardiac disease. The diagnosis is confirmed if a perfusion lung scintigram with a normal appearance is obtained after a radiotracer has been injected at another site (1,22). Contrast-enhanced chest CT is a readily available method of delineating central venous anatomy in such patients.

	FOOTNOTES

 
Authors stated no financial relationship to disclose.

Part 1 of this case appeared 4 months previously and may contain larger images.

One individual submitted the highest number of most likely diagnoses for cases 61–72. The name of this person will be announced in the October issue of the Journal.

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TOP HISTORY IMAGING FINDINGS DISCUSSION REFERENCES

 

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