Congenital pulmonary venolobar syndrome

Congenital pulmonary venolobar syndrome is a condition comprising a rare group of cardiac and pulmonary congenital abnormalities occurring variably in combination. The abnormalities include:

  • can present with shortness of breath and exercise intolerance due to the degree of left-to-right cardiopulmonary shunt causing pulmonary hypertension with right heart failure
  • recurrent pulmonary infection/lower lobe pneumonia and/or hemoptysis due to sequestration
  • frequently asymptomatic, picked up incidentally on plain chest radiograph or echocardiography

Non-specific and may show:

  • an enlarged right heart
  • small right hemithorax with mediastinal shift to the right
  • right sided scimitar vein
  • recurrent lower lobe consolidation

May show:

  • right ventricular dilation and hypertrophy
  • anomalous pulmonary veins, e.g. scimitar vein, left vertical vein
  • sequestration with systemic arterial supply from the upper abdominal aorta, celiac trunk or SMA
  • small right lung with mediastinal shift to the right
  • horseshoe lung - right lung extends posteriorly to the heart, contiguous with the left lung

In addition to CT, MRI allows the radiologist to:

  • confirm anomalous pulmonary venous drainage
  • identify any additional congenital heart abnormalities, e.g. sinus venosus atrial septal defect (ASD)
  • measure the degree of left-to-right shunt via phase contrast flow volumes
  • perform quantitation of right ventricular volumes and systolic function
  • identify systemic-pulmonary arterial supply with differential pulmonary perfusion

Identification of the systemic arterial supply to the lung in these patients is important in the context of planning for surgical repair of the anomalous venous drainage, in order to avoid injury and bleeding from this vessel.

  • in patients with a large left-to-right shunt at risk of developing pulmonary hypertension and right heart failure, corrective surgery is a consideration
  • surgical resection of the sequestration in cases of recurrent hemoptysis and/or infection
  • conservative management with serial cine MRI (cMRI) to monitor right heart progress

The term was initially coined by Dr Benjamin Felson.

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Article information

rID: 44469
System: Chest, Cardiac
Section: Anatomy, Syndromes
Synonyms or Alternate Spellings:

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Cases and figures

  • Case 1: with horseshoe lung
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    Case 2
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  • Features of Scimi...
    Case 3
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