We report on a rare case of combined unilateral atresia of the proximal right pulmonary artery (PA) and left patent ductus arteriosus (PDA). A 46 year-old female patient with known PDA and associated advanced pulmonary arterial hypertension presented with progressive hemoptysis and increasing exertional dyspnea. Computed tomography of the chest proved the presence of the known PDA but surprisingly failed to demonstrate an extrapulmonary proximal right PA. We show the imaging features and discuss the embryological background of this rare congenital cardiovascular malformation.
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