- AKA venolobar syndrome
- AKA hypogenetic lung syndrome
- Rare congenital cardiovascular anomaly involving the right lung.
- Ipsilateral anomalous pulmonary drainage of part or all of the right lung into the inferior vena cava
- Hypoplasia of the right lung
- Dextrorotation of the heart
- Hypoplasia or other malformation of the right pulmonary artery
- Anomalous systemic arterial supply to the lower lobe of the right lung from the subdiaphragmatic aorta or its main branches
- Connection to the bronchial tree distinguishes this entity from pulmonary sequestration
- Clinical significance and prognosis of scimitar sydrome depend to a large extent on the amount of the resulting left-right shunt; at least 40% of patients are asymptomatic.
- Clinical symptoms usually manifest in the 2nd to 3rd decade of life, with fatigue and dyspnea with exertion being most common and recurrent pulmonary infection less common.
 Imaging Findings
 See Also
- Pulmonary sequestration
- Partial anomalous pulmonary venous return
- Total anomalous pulmonary venous return
 External Links
Teresa Berrocal, Carmen Madrid, Susana Novo, Julia Gutiérrez, Antonia Arjonilla, and Nieves Gómez-León. Congenital Anomalies of the Tracheobronchial Tree, Lung, and Mediastinum: Embryology, Radiology, and Pathology. RadioGraphics 2004 24: 17e.