Lemoine G, Thibert M, Leca F, Rolland J C, Allegraud P, Temkine J
Arch Mal Coeur Vaiss. 1979 May;72(5):552-8.
This is a rare congenital abnormality with only 17 other reported cases in the world literature. The most frequent sign is cyanosis with peripheral arterial desaturation. Angiography with selective injection in the main pulmonary shows early opacification of the left atrium, so confirming the diagnosis. Occasionally, a catheter can be passed through the fistula. Without surgical treatment there is a danger of systemic arterial embolism and two fatal cases were found in our review of the literature. The fistulous connection is often aneurysmal. It arises either from the right main pulmonary artery or from its branch to the inferior lobe. Associated vascular and pulmonary malformations are not rate. Surgical closure of the fistual is curative with immediate regression of the cyanosis and suppression of possible embolic complications.
这是一种罕见的先天性异常，世界文献中仅另有17例报告病例。最常见的体征是发绀伴外周动脉血氧饱和度降低。在主肺动脉进行选择性注射的血管造影显示左心房早期显影，从而确诊。偶尔，导管可通过瘘管。未经手术治疗存在发生体循环动脉栓塞的风险，我们查阅文献时发现了两例致命病例。瘘管连接通常呈瘤样。它要么起源于右主肺动脉，要么起源于其下叶分支。相关的血管和肺部畸形并不少见。手术闭合瘘管可治愈，发绀会立即消退，可能的栓塞并发症也会得到抑制。
