Abstract
BackgroundUnilateral absence of pulmonary artery (UAPA) is a rare congenital disease of pulmonary circulation, which is often accompanied by other cardiovascular anomalies. Infrequently, it may remain undiagnosed until adulthood. More rarely, it is to be found with lung cancer in the ipsilateral or contralateral lung simultaneously.Case presentationA 56-year-old man with complaints of intermittent left chest pain for 2 months was referred to our hospital. Chest computed tomography(CT) revealed a cavitary lesion measuring 5.5 cm × 5.7 cm in the superior segment of the left lower lobe. Absence of left pulmonary artery and right-sided aortic arch were found on chest computed tomography angiography (CTA). The tumor was successfully removed via left pneumonectomy, and postoperative histopathology showed that the tumor was a squamous cell carcinoma (T2bN1). At a postoperative 24-month follow-up, the patient was free of disease and no evidence of recurrence or metastasis. Based on literature review, this is the ninth case of lung cancer in UAPA patients.ConclusionsLung cancer and UAPA occurred ipsilaterally in 66.7% of these cases (6/9), including the present case. For those patients who occurred contralaterally, surgical treatment may be more challenging. CT and CTA could provide an accurate diagnosis for this disease entity. Identification and recognition of this rare and special disease entity may facilitate timely diagnosis and appropriate treatment.
Highlights
Unilateral absence of pulmonary artery (UAPA) is a rare congenital disease of pulmonary circulation, which is often accompanied by other cardiovascular anomalies
The sole right-sided aortic arch in our patient is not a hazard anomaly, which is probably responsible for delayed diagnosis of UAPA
The right-sided aortic arch is generally related to the agenesis of the left pulmonary artery instead of the right pulmonary artery
Summary
Unilateral absence of pulmonary artery (UAPA) is a rare congenital disease of pulmonary circulation, which is often accompanied by other cardiovascular anomalies. The estimated prevalence of isolated UAPA without other cardiac anomalies is around 1 in 200,000 individuals [1–3], which was calculated by cases per year in a certain medical center. It has no sex predilection and is often accompanied by other cardiovascular anomalies, such as Tetralogy of Fallot in pediatric patients. 13 to 15% of patients with unilateral pulmonary artery agenesis remain
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