Unilateral Pulmonary Artery Agenesis

Physiology

  • Pulmonary hypertension due to absence of development of unilateral PA

Diagnosis

  • V/Q Scan: unilateral absence of perfusion (with normal ventilation)

Clinical Presentations

  • Asymptomatic: in many cases
  • Bronchiectasis (see [[Bronchiectasis]]): usually mild and focal
    • Hemoptysis: may occur
  • Pulmonary Hypertension (see [[Pulmonary HTN-Etiology]]): variable

References

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