Pneumothorax in a Patient with Unilateral Pulmonary Artery Agenesis.

A 17-year-old Japanese woman presented with right chest pain and exertional dyspnea. The patient had a history of isolated unilateral absence of the right pulmonary artery (UAPA) and pulmonary hypertension from early childhood. Chest radiography revealed right-sided pneumothorax and a small lung volume with mediastinal and tracheal shift to the right as well as right diaphragm elevation (Picture 1). Computed tomography revealed pneumothorax and multiple thin-walled bullae only in the right lung, and absence of the right main pulmonary artery (Picture 2). The pneumothorax improved without any invasive procedures, such as needle aspiration or chest tube drainage (Picture 3). In comparison to the previous chest radiography findings, the right-sided reduction in lung volume, elevated right diaphragm and bullous lung disease showed slow progression (Picture 4). Isolated UAPA is a rare congenital anomaly, and only a few cases of bullous lung disease in UAPA patients have been reported (1,2). This is the first report of a patient with UAPA and pneumothorax on the side of the affected lung.

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The authors state that they have no Conflict of Interest (COI).