Yonsei Med J.  2007 Dec;48(6):973-980. 10.3349/ymj.2007.48.6.973.

Isolated and Complex Scimitar Vein Anomalies and Their Differentiation from the Meandering Right Pulmonary Vein

• Lee ML

• KMID: 1786212
• DOI: http://doi.org/10.3349/ymj.2007.48.6.973

Abstract

PURPOSE: Four pediatric patients with isolated (the adult form) and complex (the infantile form) scimitar vein anomalies were reviewed and compared with patients with meandering right pulmonary veins. MATERIALS AND METHODS: From January 1990 to December 2006, 4 female patients, aged 2 days to 3.5 years, with isolated and complex scimitar vein anomalies were retrospectively studied. The clinical features, chest radiographs, echocardiography, magnetic resonance imaging, magnetic resonance angiography, computer tomography, bronchography, cardiac catheterization with angiography, surgery, and autopsy were reviewed to substantiate the diagnosis of isolated and complex scimitar vein anomalies. RESULTS: Clinical manifestations were lung infections with radiographic scimitar signs in 4 patients (3 right; 1 left), respiratory distress, congestive heart failure, pulmonary hypertension and/or cyanosis in 3 patients, dextrocardia, heterotaxy, and/or right atrial isomerism in 2 patients, as well as dextroversion, right bronchial isomerism, bronchial stenosis, and/or sepsis in 1 patient. Two patients with right atrial isomerism expired. CONCLUSION: The clinical discrimination between scimitar vein anomaly with and without cardiovascular and bronchopulmonary malformations is crucial for the outcomes vary.

Keyword

Scimitar vein anomaly; cardiovascular; bronchopulmonary; malformation; heterotaxy; right atrial isomerism

MeSH Terms

Angiography
Child, Preschool
Diagnosis, Differential
Echocardiography
Female
Heart Catheterization
Humans
Infant
Infant, Newborn
Magnetic Resonance Angiography
Magnetic Resonance Imaging
Pulmonary Veins/*abnormalities
Retrospective Studies
Scimitar Syndrome/*pathology
Tomography, X-Ray Computed

Figure

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  Fig. 1 Patient 3, a 4-day-old female baby. (A) Plain chest radiograph showed a positive scimitar sign (white star) that was left-sided. (B) Echocardiography with Doppler showed drainage of this left-sided scimitar vein (sv) into the hepatic vein (hv).

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  Fig. 2 Patient 4, a 3.5-year-old girl. (A) Plain chest radiograph showed loss of cardiac silhouette at the right lower cardiac border with a positive scimitar sign (SS). (B) Two-dimensional echocardiography showed drainage of the scimitar vein (SV) into the right atrium (RA). (C and D) Chest computer tomography showed two abnormal vessels draining the right upper lobe (SVRUL) and the right lower lobe (SVRLL), and converging as a right-sided scimitar vein. This vessel drained to the junction of the inferior vena cava (IVC) and the right atrium (RA) above the diaphragm (black arrow). RPA denotes right pulmonary artery. The black open star indicates that one set of the right pulmonary veins was draining normally to the left atrium.

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