Unilateral Absence of Pulmonary Artery (UAPA) is a rare congenital malformation that is usually associated with cardiac anomalies. When there is no congenital cardiac abnormality it is rarer still and is termed isolated unilateral absence of pulmonary artery (IUAPA) (4). IUAPA may remain undetected until adulthood and frequently found incidentally on imaging for other indications.
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