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唐氏综合征的姑息性和矫正性心脏手术(作者译)

[Palliative and corrective cardiac surgery in Down's syndrome (author's transl)].

作者信息

Seybold-Epting W, Hoffmeister H E, Stunkat R

出版信息

Thoraxchir Vask Chir. 1976 Oct;24(5):424-30. doi: 10.1055/s-0028-1095958.

Abstract

Since 1970 46 infants and children with Down's Syndrome were subjected to palliation of congenital heart disease, and 54 mongoloid children underwent correction of their cardiac defects. The most common cardiac malformation was endocardial cushion defect (72%). Palliation consisted in pulmonary artery banding (PAB) with or without division of a patent ductus arteriosus (PDA) in 16 infants, and sole division of a PDA in another 16 infants with large left to right shunts due to common canalis atrioventricularis (avcanal) or ventricular septal defect (VSD). An aorto-pulmonary anastomosis was performed for relief of severe hypoxia due to right ventricular outflow tract obstruction (RVOTO) in 14 patients. Operative mortality was 41% for PAB, 21% for aortopulmonary anastomosis, anastomosis, and 0% for division of a PDA. In the group of corrective cardiac surgery the operative mortality rate was 0% in ostium primum defects and 4% in VSD closure. Correction of complete av-canal and of malformations with RVOTO had a high mortality rate of 20 and 40%, respectively. Similar results were obtained in nonmongoloid children operated upon for the same cardiac defects. The complications and causes of death were due to the complexity of the cardiac malformations and had no relation to the Down's Syndrome itself. Mongoloid children do not pose additional medical problems to management of congenital heart disease. Their results do not differ from those obtained in nonmongoloid children.

摘要

自1970年以来,46例患有唐氏综合征的婴幼儿接受了先天性心脏病的姑息治疗,54例患唐氏综合征儿童接受了心脏缺陷矫正手术。最常见的心脏畸形是心内膜垫缺损(72%)。姑息治疗包括16例婴儿接受肺动脉环扎术(PAB),伴或不伴动脉导管未闭(PDA)切断术,另有16例因共同房室管(房室管)或室间隔缺损(VSD)导致大量左向右分流的婴儿仅接受PDA切断术。14例患者因右心室流出道梗阻(RVOTO)进行了主-肺动脉吻合术以缓解严重缺氧。PAB的手术死亡率为41%,主-肺动脉吻合术的手术死亡率为21%,PDA切断术的手术死亡率为0%。在心脏矫正手术组中,原发孔缺损的手术死亡率为0%,VSD修补术的手术死亡率为4%。完全性房室管矫正术和RVOTO畸形矫正术的死亡率分别高达20%和40%。对患有相同心脏缺陷的非唐氏综合征儿童进行手术也得到了类似结果。并发症和死亡原因是由于心脏畸形的复杂性,与唐氏综合征本身无关。唐氏综合征儿童在先天性心脏病治疗中不会带来额外医疗问题。他们的治疗结果与非唐氏综合征儿童的结果并无差异。

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