ISOLATED UNILATERAL ABSENCE OF THE PULMONARY ARTERY PRESENTING IN ADULTHOOD: A CASE REPORT

Abstract

TOPIC: Lung Pathology TYPE: Fellow Case Reports INTRODUCTION: Isolated unilateral absence of the pulmonary artery (IUAPA) may rarely go undiagnosed into adulthood (1). CASE PRESENTATION: We report the case of a 44-year-old frequently homeless male with known history of chronic obstructive pulmonary disease (COPD) and pulmonary arterial hypertension (PAH), whose findings on computed tomography (CT) of the chest were consistent with IUAPA. DISCUSSION: This patient was referred to our advanced lung disease center following an episode of acute-onset hemoptysis, concurrent with long-standing history of progressive dyspnea. Infant and early childhood history was significant for pyloric stenosis, cryptorchidism, and scoliosis, but otherwise the patient reported being 'relatively healthy' through adolescence to early adulthood in exception of 5-6 hospitalizations for 'bronchitis treatments'. The patient was diagnosed with COPD in 2010 after complaints of increased dyspnea on exertion. Treatment with supplementary oxygen, tiotropium, and budesonide/formoterol was initiated with successful disease mitigation. In late 2019, the patient was hospitalized for the episode of hemoptysis, where he revealed non-adherence to oxygen and medical therapy, albeit infrequently, attributed to challenge in continued medication use. The patient was otherwise a poor historian, but reported frequent homelessness over the course of the last 9 years. At our center, CT of the chest revealed moderate cardiomegaly (Figure A, blue), bilateral subsegmental atelectasis, hypoplasia of the right lung with emphysematous changes and increased bronchial collateral circulation (Figure A-B, green), and absence of the right main pulmonary artery (Figure B, red). The patient is undergoing evaluation for lung transplantation at this time. CONCLUSIONS: This anomaly should be suspected if imaging reveals asymmetric lung fields, lung hypoplasia, or hyperinflation of the contralateral lung. Early recognition and management of IUAPA is important for preventing more serious complications such as PAH. REFERENCE #1: Jariwala P, Maturu VN, Christopher J, Jadhav KP. Congenital isolated unilateral agenesis of pulmonary arteries in adults: case series and review. Indian J Thorac Cardiovasc Surg. 2021 Jan;37(Suppl 1):144-154. doi: 10.1007/s12055-020-01032-w. Epub 2020 Sep 23. PMID: 33584030; PMCID: PMC7859122. DISCLOSURES: No relevant relationships by Ashwini Arjuna, source=Web Response No relevant relationships by Michael Olson, source=Web Response No relevant relationships by Tejus Walia, source=Web Response