Unilateral Pulmonary Fibrosis with Pulmonary Artery Agenesis (PAA): A Case Report

Unilateral pulmonary artery agenesis (AUAP) is a rare clinical condition that can be seen either in isolation or in combination with other congenital vascular disorders. When isolated, the patient may be almost asymptomatic and only become aware of this anomaly through medical examinations performed for other reasons. Here we discuss the case of a female patient with chest CT evidence of unilateral fibrous thickening, with pulmonary hypoexpansion; investigations revealed the coexistence of AUAP. This clinical association is described by very rare case-reports in the literature.