Ghazarian Armen, King Matthew, Premyodhin Ned, Gradus-Pizlo Irmina
Department of Medicine, School of Medicine, University of California, Irvine, Orange, CI, USA.
Division of Cardiology, School of Medicine, University of California, Irvine, Orange, CI, USA.
SAGE Open Med Case Rep. 2022 Sep 29;10:2050313X221127667. doi: 10.1177/2050313X221127667. eCollection 2022.
Unilateral absence of pulmonary artery is a rare congenital abnormality that occurs due to malformation of the sixth aortic arch during embryonic development. The clinical presentation of unilateral absence of pulmonary artery can vary based on age of diagnosis; however, in the adult population, it can present with a variety of manifestations including hemoptysis, recurrent pneumonia, and pulmonary hypertension or as an incidental finding. Diagnosis and management of unilateral absence of pulmonary artery remain a challenge. Here, we describe a case of a 37-year-old female with no known past medical history who presented with progressively worsening dyspnea and fatigue. She was incidentally found to have unilateral absence of pulmonary artery on computerized tomography angiography of the chest. Her imaging and physical exam demonstrated signs of volume overload and severe pulmonary hypertension. She received diuretics with good response and was discharged with referral to pulmonary hypertension clinic and eventual follow-up with right heart catheterization. In summary, we describe a rare congenital condition and highlight its diagnostic and therapeutic challenges.
单侧肺动脉缺如是一种罕见的先天性异常，它是由于胚胎发育过程中第六对主动脉弓畸形所致。单侧肺动脉缺如的临床表现会因诊断年龄而异；然而，在成年人群中，它可表现为多种症状，包括咯血、反复肺炎、肺动脉高压，或作为偶然发现。单侧肺动脉缺如的诊断和治疗仍然是一项挑战。在此，我们描述一例37岁女性病例，该患者既往无已知病史，出现进行性加重的呼吸困难和疲劳。在胸部计算机断层血管造影检查中，她被偶然发现单侧肺动脉缺如。她的影像学检查和体格检查显示有容量超负荷和严重肺动脉高压的迹象。她接受了利尿剂治疗，反应良好，出院时被转诊至肺动脉高压门诊，并最终接受右心导管检查随访。总之，我们描述了一种罕见的先天性疾病，并强调了其诊断和治疗挑战。
