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(Circulation. 2001;103:e126.)
© 2001 American Heart Association, Inc.

Images in Cardiovascular Medicine

Familial Scimitar Syndrome

Three-Dimensional Visualization of Anomalous Pulmonary Vein in Young Sisters

Kinya Ashida, MD; Akira Itoh, MD; Takahiko Naruko, MD; Masato Otsuka, MD; Yuji Sakanoue, MD; Makoto Kobayashi, MD; Hajime Yamashita, MD; Michitaka Nagashima, MD; Takuro Shinsato, MD; Syuichiro Takanashi, MD; Yoshihiro Shimizu, MD; Kazuo Haze, MD

From the Departments of Cardiology and Cardiovascular Surgery (S.T., Y.S.), Osaka City General Hospital, Osaka, Japan.

Correspondence to Akira Itoh, MD, Department of Cardiology, Osaka City General Hospital, 2-13-22 Miyakojima hondori, Miyakojima-ku, Osaka 534-0021, Japan. E-mail akiraitoh{at}ocgh.hospital.city.osaka.jp

Scimitar syndrome is a rare congenital disorder characterized by an anomalous connection of the pulmonary vein with the inferior vena cava. The anomalous vein appears as a "scimitar"-like shadow on a chest x-ray. We recently encountered Scimitar syndrome in 2 sisters and demonstrated the 3D structure of the anomalous vein by computed tomography (CT).

Case 1

A 27-year-old woman was referred to our hospital for further evaluation of an abnormal shadow in the right lower lung field on chest x-ray (Figure 1). She was asymptomatic. The 3D CT clearly demonstrated an abnormal pulmonary vein that drained into the suprahepatic inferior vena cava (Figure 2). Cardiac catheterization showed a mean pulmonary artery pressure of 15 mm Hg, a pulmonary/systemic flow ratio of 1.86, and a left-to-right shunt ratio of 46%. Corrective surgery was not performed because of her lack of symptoms and relatively low shunt ratio.

View larger version (146K): [in this window] [in a new window]   Figure 1. Case 1. Chest x-ray showing a scimitar-like abnormal shadow in the right lower lung field (arrows).

View larger version (110K): [in this window] [in a new window]   Figure 2. Case 1. Top left, 3D CT in right anterior oblique view. An anomalous vein can be visualized unequivocally (arrows). Bottom left, 3D CT in posteroanterior view. Arrows indicate the anomalous vein. Right, Late phase of pulmonary arteriogram with digital subtraction showing the anomalous pulmonary vein (arrows). IVC indicates inferior vena cava; RV, right ventricle; and LV, left ventricle.

Case 2

This patient was the 31-year-old sister of the patient described in case 1. She also had an abnormal shadow in the right lower lung field on chest x-ray (Figure 3). She had mild dyspnea on exertion, which began at 20 years of age. The 3D CT demonstrated an abnormal pulmonary vein, which drained into the suprahepatic inferior vena cava (Figure 4). On cardiac catheterization, the mean pulmonary artery pressure was 16 mm Hg, the pulmonary/systemic flow ratio was 2.05, and the left-to-right shunt ratio was 53%. The patient subsequently underwent surgery to switch the anomalous pulmonary vein to the left atrium.

View larger version (136K): [in this window] [in a new window]   Figure 3. Case 2. Chest x-ray showing an abnormal shadow in the right lower lung field (arrows).

View larger version (59K): [in this window] [in a new window]   Figure 4. Case 2. Left, 3D CT in right anterior oblique view. Arrows indicate the anomalous vein. Center, 3D CT in posteroanterior view. Arrows indicate the anomalous vein. IVC indicates inferior vena cava; RV, right ventricle; RA, right atrium; LV, left ventricle; and Ao, descending aorta. Right, Late phase of pulmonary arteriogram showing the anomalous pulmonary vein (arrows).

This Article Extract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Ashida, K. Articles by Haze, K. Search for Related Content PubMed PubMed Citation Articles by Ashida, K. Articles by Haze, K. Related Collections Cardiovascular imaging agents/Techniques CT and MRI Pediatric and congenital heart disease, including cardiovascular surgery Cardiac development