Diagnostic Strategy for Suspected Unilateral Absence of the Pulmonary Artery

Background: Unilateral absence of the pulmonary artery (UAPA) is a very rare congenital anomaly. Objective: To analyze the diagnostic strategy applied to seven patients with UAPA who were examined and subsequently treated at the National Lung Hospital, Hanoi, Vietnam. Methods: All seven patients, including three pediatric cases (1, 2, and 14 years old) and four adult cases (21, 26, 44, and 53 years old), had a history of recurrent pneumonia, and the clinical symptoms on admission included cough, progressive dyspnea, chest pain, and fatigue. The patients were initially examined clinically, followed by hematological testing, blood biochemistry testing, and chest X-ray radiology. The results suggested UAPA, so echocardiography and contrast-enhanced chest computed tomography (CT) were performed as soon as practical. Results: The echocardiographic and CT imaging findings confirmed the suspected diagnosis of UAPA in all seven patients, which was accompanied by congenital heart disease in three patients. Three of the seven patients had mild and medium pulmonary hypertension. All seven patients were treated with drugs, which led to improvement in symptoms. Conclusion: Frontal chest X-ray provided the initial signs suggesting a diagnosis of UAPA. Subsequent echocardiography and contrast-enhanced chest CT were effective diagnostic tools for fast and accurate confirmation of UAPA.