Agénésie de l’artère pulmonaire gauche

Unilateral pulmonary artery agenesis is a rare malformation accounting for 1% of congenital heart defects. It can lead to several complications. We report the case of a 16-year-old female patient with no particular past medical history, presenting with recurrent low abundance hemoptysis within a context of preservation of patient’s general condition. Clinical examination showed no abnormalities and frontal (A) and lateral (B) chest x-ray revealed small left lung-field associated with decreased vascular pattern and mediastinal shift to the left. Given this radio-clinical presentation, diagnosis of suspected left pulmonary hypoplasia, sequelae of a previous pulmonary infection and endobronchial foreign body were made. Thoracic CT scan objectifies the absence of left pulmonary artery (C) and multiple systemic collateral circulation Left pulmonary parenchyma was hypoplastic, well ventilated and without bronchial abnormality (D). The descending aorta was located on the right. The electrocardiogram showed right bundle-branch block and transthoracic ultrasound did not reveal cardiac abnormality including sign of pulmonary arterial hypertension. Spirometry showed normal respiratory function as well as laboratory tests. Hemoptysis treatment was symptomatic and the patient was kept under medical monitoring.