Physiology

• Pulmonary hypertension due to absence of development of unilateral PA

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Diagnosis

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

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Clinical Presentations

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

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References

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