THE SCIMITAR

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THE SCIMITAR

• Dr Chaitanya Vemuri

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• A 40 yr old male presented with fever and
  thrombocytopenia
• Patient gave history saying that his heart is on
  right side.
• So, CXR was taken....

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SCIMITAR SYNDROME
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Scimitar Syndrome

• 1-3 per 1,oo,ooo births
• Hypogenetic lung syndrome /
  Congenital pulmonary venolobar syndrome
• FELSON coined the term CPVS
• WOODRING et al extended the term CPVS to cover
  a range of anomalies including pulmonary
  hypoplasia pulmonary
  sequestration

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• An anomalous vein connects between the pulmonary
  venous circulation and systemic venous
  circulation. This anomalous pulmonary venous
  return can be either partial (PAPVR) or total
  (TAPVR). The syndrome associated with PAPVR is
  more commonly known as "Scimitar syndrome"

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Components

• Total or partial anomalous connection of the
  pulmonary veins in one lung to the inferior vena
  cava. This connection can be above or below the
  level of the diaphragm. Right lung is commonly
  involved
• Variable degree of hypoplasia (small-size) and
  malformation of the pulmonary arteries and lung
  in the same side.
• Often presence of aortic-pulmonary artery
  collateral arteries to the lung that is small.
  Pulmonary hypertension is present if these
  arteries are large

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• Bronchial tree on involved side is stunted
  arterial supply to lungs derive in greater
  proportion than normal from SYSTEMIC ARTERIES.
• Syndrome derives its name from anomalous
  pulmonary vein that descends vertically in lung
  before curving medially to enter inf.vena cava
  above / below diaphragm

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• The vein broadens as it curves downward,
  resulting in configuration resembling TURKISH
  SWORD, SCIMITAR

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• Occasionally, there are 2 or more veins
• Anomalous venous drainage may involve entire /
  part of lung.
• The anomalous vein can also drain into right
  atrium, portal vein.

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• About 25 of patients have associated congenital
  heart disease
• Mostly Atrial septal defect
• When isolated, is compatible with normal life,
  the consequent LT to RT shunt being small
• Associated with Bronchiectasis
• Tracheal
  Diverticula
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  Eventeration RT diaphragm
• Horse- shoe
  lung

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Radiographic features

• Enlarged curved vascular structure coursing
  medially toward the rt diaphragm this structure
  enlarges in diameter as it approaches diaphragm
• Dextroposition of the heart
• Hypoplasia of right lung

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Differential diagnosis

• Scimitar sign is simulated radiographically by
  hypoplasia of Rt lung with aplasia of pulmonary
  artery
• Abnormal artery / arteries can simulate scimitar
  vein
• Abnormal meandering vein draining a hypoplastic
  Rt lung to Lt atrium can simulate scimitar vein.

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• CT also has advantage that tracheobronchial
  anomalies are better delineated
• MRI estimate the degree of Lt to Rt shunt by
  means of velocity encoded cine MR.

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TREATMENT

• There is a wide clinical spectrum for patients
  with scimitar syndrome. The decision to intervene
  medically depends on the hemodynamic burden.
• If the amount of blood flowing to the inferior
  vena cava from the anomalous pulmonary veins is
  small, no therapy is required.
• If the anomalous pulmonary venous drainage is
  associated with a significant left-ro-right
  shunt, surgical correction is warranted.
• Aortopulmonary collateral arteries can be closed
  either in the cardiac catheterization laboratory
  by placement of occluding coils or other devices,
  or surgically.

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Thank You !