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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{at}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 rates of up to 230 bpm. Cardiac catheterization showed a pulmonary artery systolic pressure of 38 mm Hg and a left-to-right shunt ratio of 1.8:1.0.

View larger version (128K): [in this window] [in a new window]   Figure 1. Two-dimensional cardiac-gated MRI at level of top of diaphragm in transverse plane using a fast segmented k-space gradient recalled echo technique demonstrates dextrocardia and a dilated right ventricle (RV). Anomalous draining pulmonary vein (arrow) is seen adjacent to dilated inferior vena cava just superior to diaphragmatic hiatus.

View larger version (173K): [in this window] [in a new window]   Figure 2. Reformatted oblique section through 3D data set using gadolinium-DTPA contrast enhancement. In this coronal oblique view, superior and inferior pulmonary venous radicals are seen draining to anomalous (or scimitar) vein and into IVC (I) at diaphragmatic level. Proximal portion of right pulmonary artery is also seen adjacent to upper segment of anomalous vein. This 3D MR angiogram was acquired during a single breath-hold of 34 seconds.

View larger version (124K): [in this window] [in a new window]   Figure 3. A reformatted sagittal oblique view through contrast-enhanced 3D data set (spine to right of image) demonstrating course of right middle pulmonary vein, which drains into main body of anomalous vein. All lobes of right lung were thus seen to drain via the anomalous vein.

The patient underwent electrophysiological testing and atrioventricular nodal modification to control his supraventricular tachycardia. He is off all medications and feeling well, without recurrence of arrhythmia, 8 months after the procedure and is being conservatively managed with respect to his anomalous venous drainage.

Acknowledgments

The authors acknowledge the contributions of Jeffrey Goldman, MD, and Janice Dawson, RN, and the expert technical assistance of Norman Butler and Tanya Kurtz.

Footnotes

The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1–267, Houston, TX 77030.

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