Circulation. 2004;110:e540-e541
doi: 10.1161/01.CIR.0000150399.03263.11
(Circulation. 2004;110:e540-e541.)
© 2004 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Scimitar Syndrome With Esophageal Varices
Magnetic Resonance Angiography Detects Anomalous Pulmonary Venous Return
Ming-Ting Wu, MD;
Ruay-Sheng Lai, MD;
Yi-Luan Huang, MD;
Shih-Hung Hsiao, MD
From the Departments of Radiology (M.-T.W., Y.-L.H.) and Internal Medicine (R.-S.L., S.-H.H.), Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan; and the Faculty of Medicine, School of Medicine, National Yang Ming University, Taipei, Taiwan (M.-T.W., R.-S.L., Y.-L.H., S.-H.H.).
Correspondence to Ming-Ting Wu, MD, Department of Radiology, Kaohsiung Veterans General Hospital, 386 Ta-chung 1st Rd, Kaohsiung, 813, Taiwan. E-mail mtwu{at}isca.vghks.gov.tw or mingtin.wu@msa.hinet.net
A 17-year-old man was admitted to the emergency department complaining of a mouth full of blood that he had spit out twice since the previous day. A chest radiogram revealed a small right lung and dextroposition of the cardiomediastinal structure (Figure 1). After the man’s condition was stabilized, he underwent an MRI to evaluate a suspected congenital anomaly. After a contrast agent was injected intravenously, 3-dimensional magnetic resonance angiography (MRA) was performed with a 1.5-T scanner while the man held his breath for 10 seconds. Oblique-coronal reconstruction revealed a small, hypoplastic right pulmonary artery. Also noted was a large anomalous pulmonary venous return toward the inferior vena cava just at the level of the diaphragm (Figure 2A). Taken together, these findings indicated scimitar syndrome. Interestingly, the MRA showed that the anomalous pulmonary vein did not drain into the inferior vena cava. Instead, it serpiginously communicated to the azygos esophageal venous complex, causing severe esophageal varices (Figure 2B). Esophagoscopy confirmed the severely engorged varices with cherry red–colored mucosa (Figure 3). Presurgical catheter angiography confirmed the diagnosis of scimitar syndrome with anomalous venous return into esophageal varices, and a blind end to the inferior vena cava (Figure 4). This unusual pathway was identical to what was depicted by MRA. After surgical reimplantation of the pulmonary vein into the left atrium, the esophageal varices diminished, and the patient no longer experiences bleeding symptoms.
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Figure 1. Chest radiograph showed small right lung with dextroposition of cardiomediastinal structures. An abnormal linear opacity (arrows) was found, suggesting anomalous pulmonary venous return.
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Figure 2. A, Oblique-coronal reconstruction of contrast-enhanced 3-dimensional MRA showed hypogenetic right pulmonary artery (black arrowhead) and large abnormal pulmonary venous return (white arrow) toward inferior vena cava (black arrow) at supradiaphragmatic level with a blind end (asterisk) to the inferior vena cava. MPA indicates main pulmonary artery; LA, left atrium. B, Oblique-coronal plan 10 mm posterior to A. The abnormal pulmonary venous return (white arrow) drains into azygos-esophageal venous serpiginously (curved white arrow) with a blind end (*) to the inferior vena cava. The esophageal varices (white arrowheads) appeared severely engorged and drained cephalically. LPV indicates left pulmonary vein; LPA, left pulmonary artery.
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Figure 3. Endoscopy revealed severely engorged esophageal varices with cherry-reddish bleeding spots on the mucosa.
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Figure 4. Digital subtraction angiogram by super-selective injection from the hypoplastic right pulmonary artery. Note the anomalous pulmonary venous return (white arrow and curved black arrow) drains into esophageal varices (black arrowheads) with a blind end (*) to the inferior vena cava, identical to that shown by MR (Figure 2).
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Acknowledgments
This study was completed at Kaohsiung Veterans General Hospital under grant VGHKS93-80.
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Circulation 2004 110: 3743.
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