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[左肺动脉吊带合并右肺发育不全及室间隔缺损]

[Left pulmonary artery sling associated with right pulmonary hypoplasia and ventricular septal defect].

作者信息

Rossignol A M, Dyon J F, Vailloud G, Andrini P, Jouk P S, Bost M

机构信息

Service de médecine infantile, CHU de Grenoble.

出版信息

Arch Mal Coeur Vaiss. 1989 May;82(5):803-6.

PMID:2500106
Abstract

The pulmonary artery sling is a congenital malformation where the left pulmonary artery forms a vascular sling that bends around the right border of the lower trachea. Its clinical manifestations are signs of tracheal compression in the first months of life. The diagnosis of this rare anomaly is suspected on radiological and echocardiographic grounds and confirmed by oesophagography, tracheoscopy and angiocardiography. Diagnostic problems may be encountered when the condition is associated with tracheobronchial (50% of the cases) or cardiovascular malformations. In the case reported here respiratory symptoms and heart failure were present in a 1-month hypotrophic infant who also had ventricular septal defect and dextrocardia due to right lung hypoplasia. The pulmonary artery sling was diagnosed by angiography. The severity of the clinical signs precluded all attempts at surgical repair of the cardiovascular anomalies. The child died at the age of 2 1/2 months.

摘要

肺动脉吊带是一种先天性畸形,其中左肺动脉形成一个血管吊带,绕过气管下部的右缘。其临床表现为出生后最初几个月出现气管受压的体征。这种罕见异常的诊断基于放射学和超声心动图检查结果怀疑,并通过食管造影、气管镜检查和心血管造影确诊。当该病症与气管支气管畸形(50%的病例)或心血管畸形相关时,可能会遇到诊断问题。在本文报道的病例中,一名1个月大的发育不良婴儿出现呼吸症状和心力衰竭,该婴儿还因右肺发育不全患有室间隔缺损和右位心。通过血管造影诊断出肺动脉吊带。临床体征的严重性排除了对心血管异常进行手术修复的所有尝试。患儿于2个半月时死亡。

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