Isolated Unilateral Absence of a Pulmonary Artery A Case Report

Unilateral absence of a pulmonary artery (UAPA) is a rare pulmonary vascular anomaly that is usually associated with other cardiac anomalies. We report a 9-year-old female diagnosed with isolated unilateral absence of the left pulmonary artery and a right aortic arch but without other cardiac anomalies. The only symptoms the patient had were recurrent respiratory tract infections. The chest x-rays showed asymmetric lung fields, prompting subsequent studies. A series of imaging studies including chest x-rays, esophagography, echocardiography, chest computed tomography, lung ventilation-perfusion scintigraphy, angiocardiography, and 3-dimensional magnetic resonance angiography (3D-MRA) were done. The diagnosis was confirmed by the cardiac catheterization and pulmonary angiography, which disclosed absence of the left pulmonary artery. 3D-MRA conclusively demonstrated total absence of the left pulmonary artery without an occult left pulmonary artery or systemic-to-pulmonary collateral arteries. Therefore, surgical re-establishment of pulmonary continuity was impossible.