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

Abstract

PurposeFour 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 MethodsFrom 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.ResultsClinical 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.ConclusionThe clinical discrimination between scimitar vein anomaly with and without cardiovascular and bronchopulmonary malformations is crucial for the outcomes vary.

Left-sided Scimitar Vein Causing Cyanosis after Fontan Operation: Successful Transcatheter Device Occlusion Using Magnetic Resonance Imaging X-ray Fusion

“…This type of isolated infradiaphragmatic drainage of the lower lobe pulmonary vein, when present on the right side, suggests the diagnosis of scimitar syndrome. Those patients who lack the associated features of lung hypoplasia or bronchopulmonary sequestration are best described as having isolated scimitar vein anomaly . Scimitar vein of the left lung is much less common, with only sporadic reports in patients with normal visceral situs .…”

“…Those patients who lack the associated features of lung hypoplasia or bronchopulmonary sequestration are best described as having isolated scimitar vein anomaly. 5 Scimitar vein of the left lung is much less common, with only sporadic reports in patients with normal visceral situs. 6 It might seem intuitive that left-sided scimitar vein could occur in heterotaxy patients with left-sided morphologic right lungs.…”

Scimitar Syndrome: Late Presentation and Conservative Management

“…There are four types of PAPVR that have been described as supracardiac, infracardiac, cardiac or mixed [5]. Infracardiac PAPVR involves most of the right lung draining into the inferior vena cava just above or below the right hemidiaphragm via the anomalous vein which appears as a Turkish curved sword on a chest X-ray (the eponymous Scimitar) [6]. This syndrome as originally described by Cooper in 1836 involved other coexisting cardiovascular and pulmonary malformations [7].…”

Clinics in diagnostic imaging (173)

“…(5) A meandering pulmonary vein occurs when one of the pulmonary veins draining into the left atrium has an anomalous pulmonary course. Another mimic of the scimitar sign is an anomalous single unilateral pulmonary vein, which is a single pulmonary trunk draining all the bronchopulmonary segments to the left atrium.…”