成人单侧肺动脉缺如——一项诊断与治疗挑战。
Unilateral absent pulmonary artery in an adult - A diagnostic and therapeutic challenge.
作者信息
Seedat Faheem, Kalla Ismail S, Feldman Charles
机构信息
Department of Internal Medicine, Faculty of Health Sciences, University of Witwatersrand, Johannesburg, South Africa.
Division of Pulmonology, Department of Internal Medicine, Charlotte Maxeke Johannesburg Academic Hospital and Faculty of Health Sciences, University of Witwatersrand, Johannesburg, South Africa.
出版信息
Respir Med Case Rep. 2017 Sep 14;22:238-242. doi: 10.1016/j.rmcr.2017.09.004. eCollection 2017.
Unilateral absent pulmonary artery (UAPA) is a congenital abnormality rarely diagnosed in adults. UAPA has a myriad of clinical presentations and pulmonary hypertension is present in a quarter of all cases. Isolated UAPA commonly affects the right pulmonary artery and occurs as a result of abnormal development of the sixth aortic arch segment. Due to its rarity, it remains a diagnostic and therapeutic challenge. We describe a case of UAPA in an adult presenting with severe pulmonary hypertension. We describe the appropriate diagnostic approach to a patient with pulmonary hypertension and illustrate the importance of a detailed evaluation to determine the underlying aetiology, particularly in rare causes. Furthermore, we review the clinical presentation, diagnosis and management challenges of UAPA in adults.
单侧肺动脉缺如（UAPA）是一种先天性异常，在成人中很少被诊断出来。UAPA有多种临床表现，四分之一的病例存在肺动脉高压。孤立性UAPA通常影响右肺动脉，是第六主动脉弓节段异常发育的结果。由于其罕见性，它仍然是一个诊断和治疗挑战。我们描述了一例患有严重肺动脉高压的成年UAPA病例。我们描述了对肺动脉高压患者的适当诊断方法，并说明了详细评估以确定潜在病因的重要性，特别是在罕见病因中。此外，我们回顾了成人UAPA的临床表现、诊断和管理挑战。
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