THE VARIED MANIFESTATION OF PULMONARY-ARTERY AGENESIS IN ADULTHOOD
THE VARIED MANIFESTATION OF PULMONARY-ARTERY AGENESIS IN ADULTHOOD
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DOI：
10.1378/chest.108.3.670
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发表时间：
1995-09-01
期刊：
影响因子：
9.6
通讯作者：
SIAFAKAS, N
中科院分区：
文献类型：
--
作者：
BOUROS, D;PARE, P;SIAFAKAS, N
目的：单侧肺动脉发育不全(UPAA)是一种少见的先天性畸形，常合并其他心血管异常，多见于儿童时期。大多数没有相关心脏异常的患者只有轻微或没有症状，并存活到成年。这类患者的病情在成年后经常被误诊。在这份报告中，我们描述了六名UPAA患者，他们的诊断是在成年时首次确定的。对象和方法：1987年1月至1990年12月，6例男性患者，年龄17~20岁，在入伍时被发现患有UPAA，诊断依据是病史、临床和影像检查，包括胸部X线片、呼吸-灌注肺扫描、数字减影血管造影(DSA)、计算机体层摄影(CT)和磁共振成像(MRI)。UPAA位于左侧2例，位于右侧2例。右主动脉弓3例，其他心血管异常3例。肺功能检查显示4例患者表现为轻度限制性模式。与以前的报道不同，通风扫描显示所有患者患侧的“洗入”和“平衡”阶段减少，没有延迟的“洗出”阶段。经纤维支气管镜选择性支气管镜检查发现，在所研究的四名患者中，有两名患者患有同侧混合型支气管扩张症，这一发现具有以前没有描述过的临床意义。所有病例均经数字减影血管造影确诊。胸部CT(n=6)和MRI(n=4)对所有病例均有诊断价值，但均未增加有意义的信息。结论：UPAA在成人常被误诊，在单侧高透明肺的鉴别诊断中往往不被考虑。临床医生和放射科医生应该意识到成人中可能有未诊断的病例，具有许多非典型特征。
Objective: 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.Subjects and methods: 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).Results: 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.Conclusion: 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.