Selman E, Sosa H, Arango Casado J E, Cárdenas Gonzáles F, Abella Anton R, de la Torre García T, Nuñéz Castagnon P
Servicio de Cardiocirurgía Pediátrica, Hospedal William Soler, La Habana, Cuba.
G Ital Cardiol. 1994 May;24(5):517-9.
We report the clinical and surgical data of 11 patients with atrioventricular canal and vascular ring. Down syndrome was present in 8 cases (72.7%); 8 patients were female (72.7%). Seven patients underwent, at the same time, correction of vascular ring and banding of the pulmonary artery with 4 death. Two children underwent correction of vascular ring and subsequent correction of atrioventricular canal without mortality. Two patients underwent correction of atrioventricular canal and subsequent correction of the vascular ring with one death. The mortality of the 6 patients operated after the age of 6 months was in measure of 16.6%. This association of malformations may be due to anomalies of neural crest and, although rare, should be particularly kept in mind referring to female patients with Down syndrome.
我们报告了11例房室管畸形合并血管环患者的临床和手术数据。8例（72.7%）患者患有唐氏综合征；8例（72.7%）为女性。7例患者同时接受了血管环矫治和肺动脉环扎术，4例死亡。2例儿童先接受了血管环矫治，随后进行了房室管矫治，无死亡病例。2例患者先接受了房室管矫治，随后进行了血管环矫治，1例死亡。6例6个月龄后接受手术的患者死亡率为16.6%。这种畸形关联可能归因于神经嵴异常，尽管罕见，但对于患有唐氏综合征的女性患者应特别予以关注。
