Isolated absence of the right pulmonary artery

Case contributed by Dr Yaïr Glick

Presentation

Cough, fever, shortness of breath.

Patient Data

Age: 17 years
Gender: Male

Small right lung with diminished vascular markings, ipsilateral mediastinal shift. Compensatory hyperinflation of left lung, with prominent left pulmonary artery and vascular markings. Increased retrosternal space on lateral film, filled by superior lingular segment.

Patient had had a chest NECT seven years previously at our institute, at the age of 10:

Absent right pulmonary artery. Small right lung with peripheral fibrotic and cystic changes. Numerous delicate linear opacities radiating from pleura into parenchyma of right lung, suggestive of collateral transpleural arteries (lung window). Hyperinflated left lung, particularly superior lingular segment, with prominent pulmonary arteries.

Case Discussion

Additional information garnered from patient's file reinforces diagnosis of IUAPA:

  • hypoplastic right lung with severely decreased ventilation and no perfusion, discovered incidentally on a V/Q scan performed at toddlerhood due to suspicion of foreign body aspiration
  • findings at report for chest CT performed at age 5 very similar to above exam
  • transthoracic echocardiography at age 8 demonstrated no cardiac anomaly
  • patient's mother mentions mediastinal shift to right on chest x-ray taken at age 4 months

    Differential diagnosis of a small lung on plain film includes:

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