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Unilateral absence of pulmonary artery is a rare malformation that can present as an isolated lesion or may be associated with other congenital heart defects. Clinical presentation is subtle when the lesion occurs alone, and may include hemoptysis, which results from rupture of abundant bronchial submucosal vessels perfused by enlarged systemic collaterals that supply the affected lung. Pneumonectomy is recommended as definitive treatment in such an adult patient.
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