Hoang Van Luong, Lam Viet Anh, Pham Thanh Nguyen
Department of Radiology, Hanoi National Lung Hospital, Hanoi, Vietnam.
College of Health Sciences, Vin University, Hanoi, Vietnam.
Curr Med Imaging. 2024 Mar 19;20:e15734056266404. doi: 10.2174/0115734056266404231207071244.
Unilateral absence of the pulmonary artery (UAPA) is a very rare congenital anomaly.
To analyze the diagnostic strategy applied to seven patients with UAPA who were examined and subsequently treated at the National Lung Hospital, Hanoi, Vietnam.
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.
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.
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.
单侧肺动脉缺如（UAPA）是一种非常罕见的先天性异常。
分析越南河内国家肺科医院对7例接受检查并随后接受治疗的UAPA患者所应用的诊断策略。
所有7例患者，包括3例儿科病例（1岁、2岁和14岁）和4例成人病例（21岁、26岁、44岁和53岁），均有反复肺炎病史，入院时的临床症状包括咳嗽、进行性呼吸困难、胸痛和乏力。患者最初接受临床检查，随后进行血液学检测、血液生化检测和胸部X线检查。结果提示UAPA，因此尽快进行了超声心动图和胸部增强计算机断层扫描（CT）检查。
超声心动图和CT影像学检查结果证实了所有7例患者疑似UAPA的诊断，其中3例患者伴有先天性心脏病。7例患者中有3例患有轻度和中度肺动脉高压。所有7例患者均接受了药物治疗，症状得到改善。
胸部正位X线提供了提示UAPA诊断的初步迹象。随后的超声心动图和胸部增强CT是快速、准确确诊UAPA的有效诊断工具。
