Unilateral absence of a pulmonary artery is a very rare congenital cardiovascular malformation. In this report we present the case of a 31-year-old woman with unilateral absence of a pulmonary artery who developed chronic thromboembolic pulmonary hypertension after multiple episodes of pulmonary embolism due to thrombophilia. Pulmonary thromboendarterectomy was performed, and the short-term outcome was satisfactory.
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