Congenital isolated unilateral pulmonary artery agenesis: a case report and literature review

Unilateral pulmonary artery agenesis is a rare congenital malformation, typically observed in infancy or childhood, but rarely in adulthood. An elderly female patient admitted to our hospital with a chief complaint of acute chest pain is reported here. The patient experienced a sudden onset of chest pain during physical exertion (folding quilts) 4 days before admission, which progressively worsened. Thoracoabdominal computed tomography angiography (CTA) was performed to rule out acute aortic syndromes, revealing agenesis of the right pulmonary artery with systemic collateral circulation supplying the right lung. A further examination of the clinical history and symptoms uncovered a past medical history of chronic pulmonary disease lasting several decades. The patient's clinical manifestations had consistently presented as symptoms of common conditions such as chronic bronchitis, bronchiectasis, pneumonia, and pulmonary tuberculosis, and a definitive diagnosis of Isolated Unilateral Pulmonary Artery Agenesis (IUAPA) had not been established, nor had the association between this disorder and chronic pulmonary lesions been previously considered. Although follow-up examinations confirmed that the present episode of chest pain resulted from an osteoporotic vertebral fracture, further in-depth research is necessary to fully understand the relationship between the absence of the pulmonary artery and the chronic pulmonary lesions. This report, together with the literature review, discusses the key characteristics, misdiagnosis challenges, and strategies for improving the diagnosis of IUAPA.