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(Circulation. 1998;98:1583-1584.)
© 1998 American Heart Association, Inc.

Images in Cardiovascular Medicine

Scimitar Syndrome

Victor A. Ferrari, MD; Muredach P. Reilly, MB; Leon Axel, PhD, MD; ; Martin G. St. John Sutton, MBBS, FRCP

From the Cardiac Noninvasive Imaging Laboratory and the Adult Congenital Heart Clinic, Cardiovascular Division, Department of Medicine (V.A.F., M.P.R., M.G.S.S.), and the Department of Radiology (L.A.), University of Pennsylvania Medical Center, Philadelphia, Pa.

Correspondence to Victor A. Ferrari, MD, Associate Director, Cardiac Noninvasive Imaging Laboratory, University of Pennsylvania Medical Center, 9014 E Gates Pavilion, 3400 Spruce St, Philadelphia, PA 19104. E-mail ferrariv@mail.med.upenn.edu

A 27-year-old male truck driver presented with recurrent rapid palpitations associated with near-syncope for 6 months. He was noted to have had "isolated" dextrocardia soon after birth. There was no family history of congenital heart disease. Before a herniorrhaphy at age 4 years, a chest radiograph demonstrated anomalous venous drainage of the right lung. He was otherwise asymptomatic, with a normal exercise tolerance.

On examination, he was acyanotic, in sinus rhythm, with a blood pressure of 120/80 mm Hg. His apical impulse was not palpable, and heart sounds were best heard to the right of the sternum. The first heart sound was normal, and the second sound was widely split, with a holosystolic murmur of tricuspid regurgitation.

A chest radiograph showed dextrocardia and a small right lung with a prominent anomalous right pulmonary vein. Two-dimensional echocardiography demonstrated an enlarged right heart but an intact atrial septum by contrast injection. Cardiac-gated MRI showed dextrocardia, a dilated and hypertrophied right ventricle, and a large pulmonary vein that joined the inferior vena cava (IVC) below the level of the diaphragm (Fig 1). Three-dimensional (3D) MR angiography with gadolinium contrast enhancement unequivocally demonstrated that the venous drainage of the whole of the diminutive right lung was by an anomalous vein whose entire course to the suprahepatic IVC was visualized. The arterial supply of the right lung was seen to originate from a normal right pulmonary artery (Figs 2 and 3). A 24-hour ECG monitor revealed recurrent long R-P narrow complex tachycardia, with . . . [Full Text of this Article]

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