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大动脉完全转位和主动脉缩窄

Complete transposition of the great arteries and coarctation of the aorta.

作者信息

Vogel M, Freedom R M, Smallhorn J F, Williams W G, Trusler G A, Rowe R D

出版信息

Am J Cardiol. 1984 Jun 1;53(11):1627-32. doi: 10.1016/0002-9149(84)90591-5.

DOI:10.1016/0002-9149(84)90591-5
PMID:6731307
Abstract

Thirty-two patients with complete transposition of the great arteries (TGA) and coarctation of the aorta (C of A) were seen at The Hospital for Sick Children, Toronto, Canada, between 1963 and 1983. Three patients had only mild C of A and have not required coarctectomy (Group I); 29 had a severe form of C of A (Group II). Two patients in Group I and 21 in Group II had a ventricular septal defect. Subaortic obstruction was present in 5 patients in Group II. The mechanisms included anterior deviation of the infundibular ventricular septum, anomalous right ventricular muscle bundles, and abnormal ventriculoinfundibular fold. Five patients in Group II had a hypoplastic right ventricle. Coarctectomy was performed in 25 patients, and 5 died (20% mortality rate). Sixteen patients had repair for TGA (13 Mustard, 2 Jatene , 1 Rastelli), and 2 died (12% mortality rate). Life-table analysis shows that only 68% of the patients with TGA and C of A survived the first month of life. The 5-year survival in this group was 57%. In the same period, 94% of patients with uncomplicated TGA survived the first month of life and the 5-year survival rate was 89%.

摘要

1963年至1983年间,加拿大多伦多病童医院收治了32例患有大动脉完全转位(TGA)和主动脉缩窄(CoA)的患者。3例患者仅患有轻度主动脉缩窄,无需进行主动脉缩窄切除术(第一组);29例患有严重形式的主动脉缩窄(第二组)。第一组中有2例患者,第二组中有21例患者患有室间隔缺损。第二组中有5例患者存在主动脉下梗阻。其机制包括漏斗部室间隔向前移位、异常的右心室肌束以及异常的室漏斗皱襞。第二组中有5例患者右心室发育不全。25例患者接受了主动脉缩窄切除术,5例死亡(死亡率为20%)。16例患者接受了大动脉转位修复术(13例Mustard手术、2例Jatene手术、1例Rastelli手术),2例死亡(死亡率为12%)。生命表分析显示,患有大动脉转位和主动脉缩窄的患者中,只有68%存活至出生后第一个月。该组患者的5年生存率为57%。在同一时期,患有非复杂性大动脉转位的患者中,94%存活至出生后第一个月,5年生存率为89%。

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