Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N
Department of Thoracic Medicine, Medical School, University of Crete, Greece.
Chest. 1995 Sep;108(3):670-6. doi: 10.1378/chest.108.3.670.
Unilateral pulmonary artery agenesis (UPAA), a rare congenital anomaly that is frequently associated with other cardiovascular abnormalities, is usually diagnosed in childhood. Most patients who have no associated cardiac anomalies have only minor or absent symptoms and survive into adulthood. The conditions of such patients are frequently misdiagnosed in adulthood. In this report, we describe six patients with UPAA in whom the diagnosis was first established in adulthood. The varied clinical presentation of these patients is reviewed and the relative effectiveness of a variety of diagnostic tests is compared.
During the period January 1987 through December 1990, six male patients, aged 17 to 20 years, were found to have UPAA at the time of their medical screening for enrollment into the armed forces. The diagnosis was based on history, clinical and imaging examinations, including chest radiography, ventilation-perfusion lung scan, digital subtraction angiography (DSA), computed tomography (CT), and magnetic resonance imaging (MRI).
In four of the patients, the UPAA was on the left side and in two it was on the right. A right aortic arch was present in three patients and other cardiovascular anomalies were found in three. Pulmonary function studies showed a mild restrictive pattern in four. In contrast to previous reports, the ventilation scan showed a diminished "wash in" and "equilibrium" phase without a delayed "wash out" phase on the affected side in all patients. Selective bronchography through the fiberoptic bronchoscope revealed ipsilateral mixed-type bronchiectasis in two of four patients studied, a finding of clinical significance that has not been described previously. In all cases, the diagnosis was made by DSA. CT of the thorax (n = 6) and MRI (n = 4) were diagnostic in all cases in which they were performed, but added no significant information.
UPAA is frequently misdiagnosed in adulthood and is often not considered in the differential diagnosis of the unilateral hyperlucent lung. Clinicians and radiologists should be aware of the possibility of undiagnosed cases in adults, with many atypical characteristics.
单侧肺动脉缺如（UPAA）是一种罕见的先天性异常，常与其他心血管异常相关，通常在儿童期被诊断出来。大多数无相关心脏异常的患者仅有轻微症状或无症状，并能存活至成年。这类患者的病情在成年期常被误诊。在本报告中，我们描述了6例成年期首次确诊为UPAA的患者。回顾了这些患者多样的临床表现，并比较了各种诊断检查的相对有效性。
在1987年1月至1990年12月期间，6名年龄在17至20岁的男性患者在入伍体检时被发现患有UPAA。诊断基于病史、临床和影像学检查，包括胸部X线摄影、通气-灌注肺扫描、数字减影血管造影（DSA）、计算机断层扫描（CT）和磁共振成像（MRI）。
4例患者的UPAA位于左侧，2例位于右侧。3例患者存在右位主动脉弓，3例发现其他心血管异常。肺功能研究显示4例呈轻度限制性模式。与既往报道不同的是，所有患者患侧的通气扫描均显示“吸入”和“平衡”期减弱，而无延迟的“呼出”期。通过纤维支气管镜进行的选择性支气管造影显示，在接受研究的4例患者中有2例出现同侧混合型支气管扩张，这一具有临床意义的发现此前尚未见报道。在所有病例中，诊断均通过DSA做出。胸部CT（n = 6）和MRI（n = 4）在所有进行检查的病例中均具有诊断价值，但未提供重要的额外信息。
UPAA在成年期常被误诊，在单侧肺透亮度增加的鉴别诊断中常未被考虑。临床医生和放射科医生应意识到成人中存在未被诊断病例的可能性，这些病例具有许多非典型特征。
