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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
Hypogenetic lung syndrome
Pulmonary venolobar syndrome
Definitions
Partial or total anomalous pulmonary venous return of right lung to the inferior vena cava above or below the diaphragm
Infantile and pediatric/adult variants
Associated abnormalities in descending frequency
Abnormal right lung lobation and right lung hypoplasia (~ 100%)
Dextroposition of heart; right pulmonary artery hypoplasia (60%)
Systemic arterialization of right lower lung (60%)
Secundum atrial septal defect (40% overall, 80-90% in infantile variant)
Right diaphragmatic hernia (15%)
Horseshoe lung
Infantile: Ventricular septal defect, patent ductus, hypoplastic aortic arch, coarctation, tetralogy of Fallot, anomalous origin of left coronary artery, truncus arteriosus
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Ali YZ et al: Scimitar syndrome. Eur Heart J Cardiovasc Imaging. 21(8):939, 2020
Diaz-Frias J et al: Scimitar Syndrome 2020
Manso PH et al: Scimitar sign in the absence of anomalous pulmonary venous drainage: a case report. Eur Heart J Case Rep. 3(2), 2019
Wang H et al: Scimitar syndrome in children and adults: natural history, outcomes, and risk analysis. Ann Thorac Surg. 105(2):592-98, 2018
Bo I et al: Variants of the scimitar syndrome. Cardiol Young. 26(5):941-7, 2016
Korkmaz AA et al: Scimitar syndrome: a complex form of anomalous pulmonary venous return. J Card Surg. 26(5):529-34, 2011
Midyat L et al: Eponym. Scimitar syndrome. Eur J Pediatr. 169(10):1171-7, 2010
Gudjonsson U et al: Scimitar syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 9(1):56-62, 2006
Agarwal PP et al: MDCT of anomalous unilateral single pulmonary vein. AJR Am J Roentgenol. 183(5):1241-3, 2004
Berrocal T et al: Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24(1):e17, 2004
Konen E et al: Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics. 23(5):1175-84, 2003
Kamijoh M et al: Horseshoe lung with bilateral vascular anomalies: a rare variant of hypogenetic lung syndrome (scimitar syndrome). Pediatr Int. 44(4):443-5, 2002
Zylak CJ et al: Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 22 Spec No(S25-43, 2002
Vrachliotis TG et al: Hypogenetic lung syndrome: functional and anatomic evaluation with magnetic resonance imaging and magnetic resonance angiography. J Magn Reson Imaging. 6(5):798-800, 1996
Cairns RA et al: Pediatric case of the day. Hypogenetic lung syndrome (scimitar syndrome) with right-sided congenital diaphragmatic hernia. Radiographics. 15(2):496-9, 1995
Dupuis C et al: "Infantile" form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol. 71(15):1326-30, 1993
Pomerantz SM et al: False pulmonary artery catheter measurements due to the scimitar (hypogenetic lung) syndrome. Potential for iatrogenic pulmonary edema. Chest. 103(6):1895-7, 1993
Dupuis C et al: The "adult" form of the scimitar syndrome. Am J Cardiol. 70(4):502-7, 1992
Heron CW et al: Anomalous systemic venous drainage occurring in association with the hypogenetic lung syndrome. Clin Radiol. 39(4):446-9, 1988
Gilman MJ et al: Hypoplastic right pulmonary artery in the hypogenetic lung syndrome. J Comput Assist Tomogr. 6(5):1015-8, 1982
Goodman LR et al: Meandering right pulmonary vein simulating the Scimitar syndrome. Chest. 62(4):510-2, 1972
Roehm JO Jr et al: Radiographic features of the scimitar syndrome. Radiology. 86(5):856-9, 1966
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
Hypogenetic lung syndrome
Pulmonary venolobar syndrome
Definitions
Partial or total anomalous pulmonary venous return of right lung to the inferior vena cava above or below the diaphragm
Infantile and pediatric/adult variants
Associated abnormalities in descending frequency
Abnormal right lung lobation and right lung hypoplasia (~ 100%)
Dextroposition of heart; right pulmonary artery hypoplasia (60%)
Systemic arterialization of right lower lung (60%)
Secundum atrial septal defect (40% overall, 80-90% in infantile variant)
Right diaphragmatic hernia (15%)
Horseshoe lung
Infantile: Ventricular septal defect, patent ductus, hypoplastic aortic arch, coarctation, tetralogy of Fallot, anomalous origin of left coronary artery, truncus arteriosus
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
Ali YZ et al: Scimitar syndrome. Eur Heart J Cardiovasc Imaging. 21(8):939, 2020
Diaz-Frias J et al: Scimitar Syndrome 2020
Manso PH et al: Scimitar sign in the absence of anomalous pulmonary venous drainage: a case report. Eur Heart J Case Rep. 3(2), 2019
Wang H et al: Scimitar syndrome in children and adults: natural history, outcomes, and risk analysis. Ann Thorac Surg. 105(2):592-98, 2018
Bo I et al: Variants of the scimitar syndrome. Cardiol Young. 26(5):941-7, 2016
Korkmaz AA et al: Scimitar syndrome: a complex form of anomalous pulmonary venous return. J Card Surg. 26(5):529-34, 2011
Midyat L et al: Eponym. Scimitar syndrome. Eur J Pediatr. 169(10):1171-7, 2010
Gudjonsson U et al: Scimitar syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 9(1):56-62, 2006
Agarwal PP et al: MDCT of anomalous unilateral single pulmonary vein. AJR Am J Roentgenol. 183(5):1241-3, 2004
Berrocal T et al: Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24(1):e17, 2004
Konen E et al: Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics. 23(5):1175-84, 2003
Kamijoh M et al: Horseshoe lung with bilateral vascular anomalies: a rare variant of hypogenetic lung syndrome (scimitar syndrome). Pediatr Int. 44(4):443-5, 2002
Zylak CJ et al: Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 22 Spec No(S25-43, 2002
Vrachliotis TG et al: Hypogenetic lung syndrome: functional and anatomic evaluation with magnetic resonance imaging and magnetic resonance angiography. J Magn Reson Imaging. 6(5):798-800, 1996
Cairns RA et al: Pediatric case of the day. Hypogenetic lung syndrome (scimitar syndrome) with right-sided congenital diaphragmatic hernia. Radiographics. 15(2):496-9, 1995
Dupuis C et al: "Infantile" form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol. 71(15):1326-30, 1993
Pomerantz SM et al: False pulmonary artery catheter measurements due to the scimitar (hypogenetic lung) syndrome. Potential for iatrogenic pulmonary edema. Chest. 103(6):1895-7, 1993
Dupuis C et al: The "adult" form of the scimitar syndrome. Am J Cardiol. 70(4):502-7, 1992
Heron CW et al: Anomalous systemic venous drainage occurring in association with the hypogenetic lung syndrome. Clin Radiol. 39(4):446-9, 1988
Gilman MJ et al: Hypoplastic right pulmonary artery in the hypogenetic lung syndrome. J Comput Assist Tomogr. 6(5):1015-8, 1982
Goodman LR et al: Meandering right pulmonary vein simulating the Scimitar syndrome. Chest. 62(4):510-2, 1972
Roehm JO Jr et al: Radiographic features of the scimitar syndrome. Radiology. 86(5):856-9, 1966
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