A 77-year-old female, with no respiratory history of interest, she consults for persistent respiratory infection symptoms of one month of evolution despite optimized medical treatment. In chest X-ray we did not observe pathological findings, so computed chest tomography (CT) with intravenous contrast (IV) was indicated and showed unilateral agenesis of the left pulmonary artery and ipsilateral pulmonary hypoplasia (Fig. 1).
(A) The absence of the left pulmonary artery and ipsilateral pulmonary hypoplasia in a coronal section of the pulmonary window. (B) Axial view of the mediastinal window showing contrast uptake only in the right pulmonary artery. (C) Coronal section in mediastinal window, showing hypertrophy of bronchial arteries with compensation for the agenesis of the left pulmonary artery.
Unilateral pulmonary artery agenesis is a rare and low prevalence case, with an estimated prevalence of one per 200,000–300,000 individuals.1 It is mainly secondary to an anomaly in the rotation and migration of the sixth primitive aortic arch in the fourth or fifth week of embryonic development. The right pulmonary artery agenesis is more frequent and has fewer complications since it is less associated with other malformations than the left one. It is usually diagnosed in childhood after repeated respiratory infections and incidentally in adults by imaging test.2
Currently, the most accepted treatment is conservative management in asymptomatic patients, and surgery in symptomatic patients.2
Pulmonary artery agenesis, although a rare disorder, should be considered as a differential diagnosis in patients with persistent infectious respiratory symptoms.
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Conflicts of interestThe authors declare that they have no conflict of interest.