Congenital unilateral pulmonary artery agenesis and aspergilloma

Isolated unilateral pulmonary artery agenesis is a rare condition, which in most patients is asymptomatic. Occasionally patients present with symptoms that are nonspecific and not necessarily attributable to disease of the respiratory system. In these individuals the clue to the diagnosis is found in a plain chest roentgenogram, often revealing a hyperlucent contracted hemithorax. We present an unusual case of isolated unilateral pulmonary artery agenesis associated with the opportunistic organism Mycobacterium kansasii and Aspergillus fumigatus in which the diagnosis was made 10 years after initial presentation. Clinicians should be aware of this condition and include it in their differential diagnosis of a hyperlucent lung field on the chest roentgenogram.