Combined atresia of the right pulmonary artery and one of the pulmonary veins

Case contributed by Dr Parthena D. Ioannidi

Presentation

Two episodes of hemoptysis in the last 24 hours with a history of surgical resection of a right lung cyst at the age of 5 months and ongoing sporadic episodes of hemoptysis.

Patient Data

Age: 20 years
Gender: Male
  • redused pulmonary volume on the right side and mediastinal shifting ipsilaterally
  • middle - low opacification of the right lung

Thorax CT

Pulmonary window:

  • hyperventilated left lung, reduced volume of right lung with mediastinal shifting to the right
  • alveolar opacities at the middle and lower left lobe
  • bronchiectasis at the upper right lobe

Mediastinal window + C (arterial phase)

  • multiple vascular anomalies: absence of right pulmonary artery and one of right pulmonary veins

  • tortuous bronchial, intercostals and inferior phrenic arteries with rich collateral net connected to descending thoracic aorta

3D bone reconstruction:

  • additional congenital abnormalities - two bifid right ribs 

Perfusion Scintigraphy:

  • no participation of right lung in gas exchange, which confirmed the CT findings

Case Discussion

The absence of one pulmonary artery combined with partial obliteration of one of the pulmonary veins is a rare condition that concerns the developmental failure of the 6th brachial arch during the period of the 6th -16th weeks of gestation and failed connection of the common pulmonary vein to the left atrium.

About 20% of young patients with hemoptysis may present vascular abnormalities; one of the most significant is atresia of the pulmonary artery. There is no general agreement about treatment. The asymptomatic population must be followed up to monitor for pulmonary hypertension.

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