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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
- Woodring JH et al: Congenital pulmonary venolobar syndrome revisited. Radiographics. 14(2):349-69, 1994
- 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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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
- Woodring JH et al: Congenital pulmonary venolobar syndrome revisited. Radiographics. 14(2):349-69, 1994
- 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
