69 years old female staged for colorectal cancer.
Conventional x-ray of the thorax (A) shows a curvilinear opacity on the right side of the lung extending from the hilum to the diaphragm. Computed tomography of the thorax postprocessed as thin slab maximum intensity projections (B, D) and volume rendering technique (C) show an abnormal course of the right upper lobe pulmonary vein draining into the inferior caval vein (red arrows). There are pulmonary veins draining regularly into the left atrium. Anatomy of pulmonary arteries is unremarkable.
Partial anomalous pulmonary venous return (PAPVR).
PAPVR is present when one or more, but not all, of the pulmonary veins drain into a systemic vein. This results in a left-to-right shunt. PAPVR is usually an isolated finding and is more frequently seen on the right side. Most patients are either mildly symptomatic or asymptomatic.
The anatomy present in this case may be called scimitar syndrome, because the radiographic appearance of the anomalous pulmonary vein resembles a type of turkish curved saber. However, in scimitar syndrome anomalous pulmonary venous return is usually associated with hypoplasia or aplasia of the right pulmonary artery and hypoplasia of the right lung.
In general, abnormalities that involve anomalous connections of the pulmonary parenchyma and the pulmonary and systemic vasculature are summarized under the term congenital pulmonary venolobar syndrome.
Konen et al.
Congenital Pulmonary Venolobar Syndrome: Spectrum of Helical CT Findings with Emphasis on Computerized Reformatting
Radiographics (2003) 23(5):1175
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