Hemodynamics: Venous flow from right lung returns to systemic veins → L-to-R shunt
IMAGING
General Features
Radiographic Findings
Fluoroscopic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Masrani A et al: Anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR. J Cardiovasc Comput Tomogr. 12(4):286-9, 2018
Wang H et al: Scimitar syndrome in children and adults: natural history, outcomes, and risk analysis. Ann Thorac Surg. 105(2):592-98, 2018
Wasilewska E et al: Unilateral hyperlucent lung in children. AJR Am J Roentgenol. 198(5):W400-14, 2012
Korkmaz AA et al: Scimitar syndrome: a complex form of anomalous pulmonary venous return. J Card Surg. 26(5):529-34, 2011
Biyyam DR et al: Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 30(6):1721-38, 2010
Rajiah P et al: Computed tomography of pulmonary venous variants and anomalies. J Cardiovasc Comput Tomogr. 4(3):155-63, 2010
Ho ML et al: MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging. 24(2):89-95, 2009
Navas Lobato MA et al: Scimitar syndrome. Clin Cardiol. 32(7):E15-6, 2009
Gavazzi E et al: Scimitar syndrome: comprehensive, noninvasive assessment with cardiovascular magnetic resonance imaging. Circulation. 118(3):e63-4, 2008
Tjang YS et al: Scimitar syndrome presenting in adults. J Card Surg. 23(1):71-2, 2008
Wang CC et al: Scimitar syndrome: incidence, treatment, and prognosis. Eur J Pediatr. 167(2):155-60, 2008
Tsitouridis I et al: Scimitar syndrome versus meandering pulmonary vein: evaluation with three-dimensional computed tomography. Acta Radiol. 47(9):927-32, 2006
Yoo SJ et al: The relationship between scimitar syndrome, so-called scimitar variant, meandering right pulmonary vein, horseshoe lung and pulmonary arterial sling. Cardiol Young. 16(3):300-4, 2006
Khan MA et al: Usefulness of magnetic resonance angiography for diagnosis of scimitar syndrome in early infancy. Am J Cardiol. 96(9):1313-6, 2005
Berrocal T et al: Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24(1):e17, 2004
Sinha R et al: Scimitar syndrome: imaging by magnetic resonance angiography and Doppler echocardiography. Indian J Chest Dis Allied Sci. 46(4):283-6, 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
Kramer U et al: Scimitar syndrome: morphological diagnosis and assessment of hemodynamic significance by magnetic resonance imaging. Eur Radiol. 13 Suppl 4:L147-50, 2003
Marco de Lucas E et al: Scimitar syndrome: complete anatomical and functional diagnosis with gadolinium-enhanced and velocity-encoded cine MRI. Pediatr Radiol. 33(10):716-8, 2003
Vanderheyden M et al: Partial anomalous pulmonary venous connection or scimitar syndrome. Heart. 89(7):761, 2003
Reddy R et al: Scimitar syndrome: a rare cause of haemoptysis. Eur J Cardiothorac Surg. 22(5):821, 2002
Vaes MF et al: Scimitar syndrome. JBR-BTR. 85(3):160-1, 2002
Zylak CJ et al: Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 22 Spec No:S25-43, 2002
Do KH et al: Systemic arterial supply to the lungs in adults: spiral CT findings. Radiographics. 21(2):387-402, 2001
Gilkeson RC et al: Gadolinium-enhanced magnetic resonance angiography in scimitar syndrome: diagnosis and postoperative evaluation. Tex Heart Inst J. 27(3):309-11, 2000
Huddleston CB et al: Scimitar syndrome presenting in infancy. Ann Thorac Surg. 67(1):154-9; discussion 160, 1999
Henk CB et al: Scimitar syndrome: MR assessment of hemodynamic significance. J Comput Assist Tomogr. 21(4):628-30, 1997
Baran R et al: Scimitar syndrome: confirmation of diagnosis by a noninvasive technique (MRI). Eur Radiol. 6(1):92-4, 1996
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
Boothroyd AE et al: Shoe, scimitar or sequestration: a shifting spectrum. Pediatr Radiol. 25(8):652-3, 1995
Figa FH et al: Horseshoe lung--a case report with unusual bronchial and pleural anomalies and a proposed new classification. Pediatr Radiol. 23(1):44-7, 1993
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Synonyms
Hypogenetic lung/pulmonary venolobar syndrome
Definitions
Right lung hypoplasia with anomalous right pulmonary venous connection to inferior vena cava (IVC)
Often associated: Anomalous systemic arterial supply
Hemodynamics: Venous flow from right lung returns to systemic veins → L-to-R shunt
IMAGING
General Features
Radiographic Findings
Fluoroscopic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Masrani A et al: Anatomical associations and radiological characteristics of Scimitar syndrome on CT and MR. J Cardiovasc Comput Tomogr. 12(4):286-9, 2018
Wang H et al: Scimitar syndrome in children and adults: natural history, outcomes, and risk analysis. Ann Thorac Surg. 105(2):592-98, 2018
Wasilewska E et al: Unilateral hyperlucent lung in children. AJR Am J Roentgenol. 198(5):W400-14, 2012
Korkmaz AA et al: Scimitar syndrome: a complex form of anomalous pulmonary venous return. J Card Surg. 26(5):529-34, 2011
Biyyam DR et al: Congenital lung abnormalities: embryologic features, prenatal diagnosis, and postnatal radiologic-pathologic correlation. Radiographics. 30(6):1721-38, 2010
Rajiah P et al: Computed tomography of pulmonary venous variants and anomalies. J Cardiovasc Comput Tomogr. 4(3):155-63, 2010
Ho ML et al: MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging. 24(2):89-95, 2009
Navas Lobato MA et al: Scimitar syndrome. Clin Cardiol. 32(7):E15-6, 2009
Gavazzi E et al: Scimitar syndrome: comprehensive, noninvasive assessment with cardiovascular magnetic resonance imaging. Circulation. 118(3):e63-4, 2008
Tjang YS et al: Scimitar syndrome presenting in adults. J Card Surg. 23(1):71-2, 2008
Wang CC et al: Scimitar syndrome: incidence, treatment, and prognosis. Eur J Pediatr. 167(2):155-60, 2008
Tsitouridis I et al: Scimitar syndrome versus meandering pulmonary vein: evaluation with three-dimensional computed tomography. Acta Radiol. 47(9):927-32, 2006
Yoo SJ et al: The relationship between scimitar syndrome, so-called scimitar variant, meandering right pulmonary vein, horseshoe lung and pulmonary arterial sling. Cardiol Young. 16(3):300-4, 2006
Khan MA et al: Usefulness of magnetic resonance angiography for diagnosis of scimitar syndrome in early infancy. Am J Cardiol. 96(9):1313-6, 2005
Berrocal T et al: Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24(1):e17, 2004
Sinha R et al: Scimitar syndrome: imaging by magnetic resonance angiography and Doppler echocardiography. Indian J Chest Dis Allied Sci. 46(4):283-6, 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
Kramer U et al: Scimitar syndrome: morphological diagnosis and assessment of hemodynamic significance by magnetic resonance imaging. Eur Radiol. 13 Suppl 4:L147-50, 2003
Marco de Lucas E et al: Scimitar syndrome: complete anatomical and functional diagnosis with gadolinium-enhanced and velocity-encoded cine MRI. Pediatr Radiol. 33(10):716-8, 2003
Vanderheyden M et al: Partial anomalous pulmonary venous connection or scimitar syndrome. Heart. 89(7):761, 2003
Reddy R et al: Scimitar syndrome: a rare cause of haemoptysis. Eur J Cardiothorac Surg. 22(5):821, 2002
Vaes MF et al: Scimitar syndrome. JBR-BTR. 85(3):160-1, 2002
Zylak CJ et al: Developmental lung anomalies in the adult: radiologic-pathologic correlation. Radiographics. 22 Spec No:S25-43, 2002
Do KH et al: Systemic arterial supply to the lungs in adults: spiral CT findings. Radiographics. 21(2):387-402, 2001
Gilkeson RC et al: Gadolinium-enhanced magnetic resonance angiography in scimitar syndrome: diagnosis and postoperative evaluation. Tex Heart Inst J. 27(3):309-11, 2000
Huddleston CB et al: Scimitar syndrome presenting in infancy. Ann Thorac Surg. 67(1):154-9; discussion 160, 1999
Henk CB et al: Scimitar syndrome: MR assessment of hemodynamic significance. J Comput Assist Tomogr. 21(4):628-30, 1997
Baran R et al: Scimitar syndrome: confirmation of diagnosis by a noninvasive technique (MRI). Eur Radiol. 6(1):92-4, 1996
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
Boothroyd AE et al: Shoe, scimitar or sequestration: a shifting spectrum. Pediatr Radiol. 25(8):652-3, 1995
Figa FH et al: Horseshoe lung--a case report with unusual bronchial and pleural anomalies and a proposed new classification. Pediatr Radiol. 23(1):44-7, 1993
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