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Pulmonary fibrosis is a chronic condition typically affecting both lungs; however, cases of unilateral pulmonary fibrosis are exceedingly rare and often result from specific unilateral inflammatory conditions like radiation pneumonitis or infection. An even rarer occurrence is the unilateral proximal interruption of a pulmonary artery (PIPA), a developmental anomaly resulting from the failed connection of the sixth aortic arch to the pulmonary trunk. This condition can manifest alone or alongside other cardiac abnormalities. There are limited reports of pulmonary fibrosis associated with PIPA. In this case, a 33-year-old male with chronic mild asthma presented with bilateral shoulder pain. Initial radiographs showed reticular opacities and volume loss in the right lung with a rightward mediastinal shift, suggesting possible fibrosis. Further investigations with chest Computed Tomography (CT) and CT angiogram confirmed right-sided pulmonary fibrosis and the absence of the right pulmonary artery, with no other significant cardiopulmonary symptoms reported. This case highlights the complexity of diagnosing and managing rare unilateral pulmonary conditions., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Bae et al.)

Background: Patients with acute aortic dissection type A (AADA) often have hypoxemia (partial pressure of oxygen [PaO2]/fraction of inspired oxygen [FiO2]