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(Circulation. 2006;114:e373-e375.)
© 2006 American Heart Association, Inc.

Images in Cardiovascular Medicine

Scimitar Syndrome

Complete Diagnosis by Transthoracic Echocardiography

Rowlens M. Melduni, MD; Farouk Mookadam, MBChB, FRCPC; Martina Mookadam, MD; Krishnaswamy Chandrasekaran, MD; Fletcher A. Miller, MD; Jae K. Oh, MD; A. Jamil Tajik, MD

From the Division of Cardiovascular Diseases and Department of Internal Medicine, Mayo Clinic, Rochester, Minn, and Division of Cardiovascular Diseases, Mayo Clinic, Scottsdale, Ariz.

Correspondence to Farouk Mookadam, MBChB, Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905. E-mail mookadam.farouk{at}mayo.edu

A 56-year-old woman who underwent secundum atrial septal defect closure at the age of 25 years was referred to the Mayo Clinic for exertional dyspnea and symptomatic atrial fibrillation. On examination, she was acyanotic, with an irregularly irregular heart rate of 86 beats per minute and a blood pressure of 140/80 mm Hg. She had a fixed S₂ split with a normal apical impulse.

During the course of her work-up, transthoracic echocardiography demonstrated anomalous pulmonary venous return of the right upper and lower pulmonary veins to the inferior vena cava just above the diaphragm (Scimitar syndrome). The right-sided chambers of the heart and pulmonary arteries were dilated because of left-to-right shunting. The left and right middle pulmonary veins entered the left atrium normally. The calculated pulmonary-to-systolic flow ratio was 2.5:1 (Figures 1 through 3 and Movies I through IV). Contrast-enhanced computed tomography scan and cardiac catheterization to delineate the anatomy of the vascular and bronchial anatomy also confirmed our findings and revealed a pulmonary arterial pressure of 46/31 mm Hg and pulmonary vascular resistance of 3.26 Woods unit · m² (Figure 4 and Movies V through VII). The patient was referred for surgical correction.

View larger version (21K): [in this window] [in a new window]   Figure 1. Subcostal view with 2-dimensional and color Doppler showing the scimitar vein (SV) draining into the dilated inferior vena cava (IVC) just above the diaphragm. LA indicates left atrium; RA, right atrium.

View larger version (28K): [in this window] [in a new window]   Figure 2. Pulse wave Doppler image of the anomalous vein showing the typical monophasic flow pattern of the Scimitar vein into the inferior vena and extending throughout the cardiac cycle with an abnormally high peak velocity.

View larger version (32K): [in this window] [in a new window]   Figure 3. "Crab view" with 2-dimensional and color Doppler (obtained with the transducer in the suprasternal notch position) demonstrating the absence of the right upper and right lower pulmonary veins. The right middle (RMPV), left upper (LUPV), and left lower (LLPV) pulmonary veins drain normally into the left atrium (LA). Note the dilated right pulmonary artery (RPA) caused by left-to-right shunting. Ao indicates aorta.

View larger version (25K): [in this window] [in a new window]   Figure 4. A, Anterior view; B, posterior view. Three-dimensional computed tomography reconstructed images showing a large scimitar vein that drains most of the right lung into the inferior vena cava just above the diaphragm. IVC indicates inferior vena cava; LA, left atrium; LLPV, left lower pulmonary vein; LUPV, left upper pulmonary vein; RMPV, right middle pulmonary vein; and Scim.V, scimitar vein.

When anomalous pulmonary venous drainage is suspected because of the characteristic radiographic curvilinear appearance of vein shaped like a "scimitar" or Turkish sword (scimitar sign) or increased caval flow into the right atrium, the diagnosis can be made easily by transthoracic echocardiography delineating the abnormal anatomy with the use of the "crab view" coupled with subcostal imaging to demonstrate anomalous pulmonary venous flow into the inferior vena cava below or just above the diaphragm.

	Acknowledgments

 
The authors thank Mark Zangs for his expert technical assistance.

Disclosures

None.

	Footnotes

 
The online-only Data Supplement, which includes 7 movies, is available at http://circ.ahajournals.org/cgi/content/full/114/10/e373/DC1.

This Article Full Text (PDF) Data Supplement 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 Melduni, R. M. Articles by Tajik, A. J. Search for Related Content PubMed PubMed Citation Articles by Melduni, R. M. Articles by Tajik, A. J. Related Collections Cardiovascular imaging agents/Techniques Pulmonary circulation and disease Echocardiography Pediatric and congenital heart disease, including cardiovascular surgery