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Unilateral Absence of Pulmonary Artery(UAPA) is a rare disease. It is characterized by no specfiic cardiopulmonary symptoms in general. We experienced a case of UAPA in a 9 year-old female patient with the chief complaint of abnormal chest Xray finding. Lung perfusion scan, cardiac catheterization with pulmonary and aortic angiography confirmed the diagnosis of right side UAPA.
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