Scimitar syndrome triad: Right lung hypoplasia, anomalous right pulmonary venous connection to inferior vena cava (IVC), & anomalous systemic arterial supply to right lower lobe
Scimitar vein represents form of partial anomalous pulmonary venous return (PAPVR)
Hemodynamics: Venous flow from right lung returns to right atrium → volume overload of right heart [atrial septal defect (ASD)-type physiology]
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
Diaz-Frias J et al: Scimitar syndrome. StatPearls, 2021
Mounir R et al: Adults forms of scimitar syndrome. J Card Surg. 35(7):1697-9, 2020
Trimech T et al: Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment. Ann Cardiol Angeiol (Paris). 69(5):317-22, 2020
Han F et al: The case of the missing pulmonary vein: a focused update on anomalous pulmonary venous connection in congenital cardiovascular disease. Echocardiography. 36(10):1930-5, 2019
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
O'Byrne ML et al: Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood. Cardiol Young. 28(2):329-33, 2018
Althomali SA et al: Uncommon presentation of adult-form scimitar syndrome associated with single left pulmonary vein in a pregnant woman. BMJ Case Rep. 2017
Bernal Garnes N et al: Magnetic resonance imaging in the assessment of anomalous pulmonary venous connections. Radiologia. 58(2):111-9, 2015
Nawrocki P et al: Scimitar syndrome associated with abnormal hepatic venous drainage. World J Pediatr Congenit Heart Surg. 6(3):474-6, 2015
Yamakawa H et al: Transcatheter embolization for hemoptysis associated with anomalous systemic artery in a patient with scimitar syndrome. Springerplus. 4:422, 2015
Ciçek S et al: Scimitar syndrome: the curved Turkish sabre. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 17(1):56-61, 2014
Dyer KT et al: Imaging in congenital pulmonary vein anomalies: the role of computed tomography. Pediatr Radiol. 44(9):1158-68; quiz 1155-7, 2014
Ganesan S et al: Prenatal findings in total anomalous pulmonary venous return: a diagnostic road map starts with obstetric screening views. J Ultrasound Med. 33(7):1193-207, 2014
Goudarzi M et al: Anomalous unilateral single pulmonary vein mimicking pulmonary nodules on HRCT of the lungs and scimitar syndrome on chest radiograph: multidetector CT findings. J Thorac Imaging. 24(2):142-6, 2009
Gavazzi E et al: Scimitar syndrome: comprehensive, noninvasive assessment with cardiovascular magnetic resonance imaging. Circulation. 118(3):e63-4, 2008
Lee ML: Isolated and complex scimitar vein anomalies and their differentiation from the meandering right pulmonary vein. Yonsei Med J. 48(6):973-80, 2007
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
Ramseyer L et al: The scimitar syndrome: demonstration with magnetic resonance imaging. J Okla State Med Assoc. 89(9):324-5, 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
Gao YA et al: Scimitar syndrome in infancy. J Am Coll Cardiol. 22(3):873-82, 1993
Baxter R et al: Scimitar syndrome: cine magnetic resonance imaging demonstration of anomalous pulmonary venous drainage. Ann Thorac Surg. 50(1):121-3, 1990
Partridge JB et al: Scimitar etcetera--the dysmorphic right lung. Clin Radiol. 39(1):11-9, 1988
Scimitar syndrome triad: Right lung hypoplasia, anomalous right pulmonary venous connection to inferior vena cava (IVC), & anomalous systemic arterial supply to right lower lobe
Scimitar vein represents form of partial anomalous pulmonary venous return (PAPVR)
Hemodynamics: Venous flow from right lung returns to right atrium → volume overload of right heart [atrial septal defect (ASD)-type physiology]
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
Diaz-Frias J et al: Scimitar syndrome. StatPearls, 2021
Mounir R et al: Adults forms of scimitar syndrome. J Card Surg. 35(7):1697-9, 2020
Trimech T et al: Scimitar syndrome with bicuspid aortic valve. A case report of cross-sectional non- invasive imaging allowing a complete anatomical and functional assessment. Ann Cardiol Angeiol (Paris). 69(5):317-22, 2020
Han F et al: The case of the missing pulmonary vein: a focused update on anomalous pulmonary venous connection in congenital cardiovascular disease. Echocardiography. 36(10):1930-5, 2019
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
O'Byrne ML et al: Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood. Cardiol Young. 28(2):329-33, 2018
Althomali SA et al: Uncommon presentation of adult-form scimitar syndrome associated with single left pulmonary vein in a pregnant woman. BMJ Case Rep. 2017
Bernal Garnes N et al: Magnetic resonance imaging in the assessment of anomalous pulmonary venous connections. Radiologia. 58(2):111-9, 2015
Nawrocki P et al: Scimitar syndrome associated with abnormal hepatic venous drainage. World J Pediatr Congenit Heart Surg. 6(3):474-6, 2015
Yamakawa H et al: Transcatheter embolization for hemoptysis associated with anomalous systemic artery in a patient with scimitar syndrome. Springerplus. 4:422, 2015
Ciçek S et al: Scimitar syndrome: the curved Turkish sabre. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 17(1):56-61, 2014
Dyer KT et al: Imaging in congenital pulmonary vein anomalies: the role of computed tomography. Pediatr Radiol. 44(9):1158-68; quiz 1155-7, 2014
Ganesan S et al: Prenatal findings in total anomalous pulmonary venous return: a diagnostic road map starts with obstetric screening views. J Ultrasound Med. 33(7):1193-207, 2014
Goudarzi M et al: Anomalous unilateral single pulmonary vein mimicking pulmonary nodules on HRCT of the lungs and scimitar syndrome on chest radiograph: multidetector CT findings. J Thorac Imaging. 24(2):142-6, 2009
Gavazzi E et al: Scimitar syndrome: comprehensive, noninvasive assessment with cardiovascular magnetic resonance imaging. Circulation. 118(3):e63-4, 2008
Lee ML: Isolated and complex scimitar vein anomalies and their differentiation from the meandering right pulmonary vein. Yonsei Med J. 48(6):973-80, 2007
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
Ramseyer L et al: The scimitar syndrome: demonstration with magnetic resonance imaging. J Okla State Med Assoc. 89(9):324-5, 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
Gao YA et al: Scimitar syndrome in infancy. J Am Coll Cardiol. 22(3):873-82, 1993
Baxter R et al: Scimitar syndrome: cine magnetic resonance imaging demonstration of anomalous pulmonary venous drainage. Ann Thorac Surg. 50(1):121-3, 1990
Partridge JB et al: Scimitar etcetera--the dysmorphic right lung. Clin Radiol. 39(1):11-9, 1988
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