ABSTRACT

Unilateral pulmonary artery agenesis (UPA) is an uncommon congenital anomaly that results from the absence of ipsilateral sixth aortic arch development. Genetic and mechanical factors have been blamed so far; however, no significant etiological factor has been found up to now. UPA is mostly asymptomatic in adulthood, on the other hand, because of being associated with other congenital cardiac malformations, it is usually symptomatic in childhood. Therefore, the majority of adult patients are identified incidentally with PA chest radiographs performed for different reasons. Typical chest radiographic findings are ipsilateral volume loss, absence of hilar shadow, hemidiaphragm elevation, mediastinal displacement and hyperinflation of thecontrolateral lung. In symptomatic cases, the most frequent symptoms are recurrent pulmonary infections, dyspnea and exercise intolerance. In this article, because of its clinical importance, we report the case of a 16-yearold male who was diagnosed with right pulmonary artery agenesis with typical imaging findings.