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儿童期法洛四联症的血管造影与临床关联(作者译)

[Angiographic-clinical correlation in Fallot's-tetralogy during childhood (author's transl)].

作者信息

Beitzke A

出版信息

Rofo. 1980 Feb;132(2):178-84. doi: 10.1055/s-2008-1056546.

Abstract

Thirty-eight patients with Fallot's tetralogy were investigated in order to correlate their clinical and angiographic findings. In the light of embryological knowledge about the morphogenesis of Fallot's tetralogy there are good explanations for the various modes of clinical presentation. Patients with extreme cyanosis in the newborn period show diffuse hypoplasia of their right ventricular outflow tract and pulmonary arteries because of extreme malseptation of the conus with anteposition of the infundibular septum. Patients who present with cyanosis and blue spells in infancy show infundibular narrowing and dextro- and anteposition of the ascending aorta. Conal malseptation together with malrotation of the conus seem to be the responsible factors in this patient group. Malrotation of the conus without malseptation is probably the mechanism in patients who present initially without cyanosis and signs of left to right shunting. Anteposition and overriding of the aorta are the initial angiographic signs while right ventricular hypertrophy producing infundibular narrowing and subsequent cyanosis appear later.

摘要

为了将法洛四联症患者的临床和血管造影结果相关联,对38例法洛四联症患者进行了研究。根据有关法洛四联症形态发生的胚胎学知识,对于各种临床表现模式有很好的解释。新生儿期出现极度紫绀的患者,由于圆锥隔的极度错位和漏斗隔的前移,其右心室流出道和肺动脉呈现弥漫性发育不全。婴儿期出现紫绀和缺氧发作的患者表现为漏斗部狭窄以及升主动脉右旋和前移。圆锥隔错位以及圆锥旋转不良似乎是该患者组的致病因素。无圆锥隔错位的圆锥旋转不良可能是最初无紫绀和左向右分流体征患者的发病机制。主动脉前移和骑跨是最初的血管造影征象,而右心室肥厚导致漏斗部狭窄并随后出现紫绀则较晚出现。

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