Results of surgical treatment of congenital heart defects in children with Down's syndrome
Results of surgical treatment of congenital heart defects in children with Down's syndrome
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DOI：
10.1007/s002469900483
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发表时间：
1999-09-01
影响因子：
1.6
通讯作者：
Zdebska, E
中科院分区：
文献类型：
--
作者：
Malec, E;Mroczek, T;Zdebska, E
我们分析了1990年至1997年间连续100例先天性心脏病（CHD）和唐氏综合征（DS）患儿的早期和晚期手术治疗结果。其中房室共同管（CAVC）50例，室间隔缺损24例，原发口房间隔缺损8例，法洛四联症（TOF）8例，动脉导管未闭3例，继发口房间隔缺损3例，CAVC合并TOF 4例。在93例患者中进行了完全矫正。总死亡率为6%。CAVC组的死亡率为8%，但在过去3年中下降至2.7%。随访儿童（7个月至6岁，平均39个月）NYHA I级或II级。术后并发肺部感染占38%，全身感染占10%，死亡占8%，无再手术病例。结果表明，冠状动脉介入治疗可使冠状动脉狭窄患儿的死亡率和严重二尖瓣房室瓣反流的发生率降低。严重感染的高发生率可能会影响最终结果。在婴儿期对CHD进行修复有助于消除与充血性心力衰竭和肺动脉高压有关的问题。
We analyzed early and late results of surgical treatment of 100 consecutive children with Down's syndrome (DS) and congenital heart defect (CHD) who were operated on between 1990 and 1997. Fifty had common atrioventricular canal (CAVC), 24 ventricular septal defect, 8 the ostium primum atrial septal defect, 8 tetralogy of Fallot (TOF), 3 patent ductus arteriosus, 3 the ostium secundum atrial septal defect, and 4 CAVC coexisting with TOF. In 93 patients total correction was performed. The total death rate was 6%. Death in the CAVC group was 8%, but it decreased to 2.7% during the past 3 years. The children who were followed up (from 7 months to 6 years; mean, 39 months) are in NYHA class I or II. There were no reoperations, The postoperative course was complicated by pulmonary infections in 38% of patients, which converted to generalized infection in 10% and was the cause of death in 8% of patients, These results indicate that CHD in DS children can be repaired with a low death rate and low incidence of severe mitral atrioventricular valve regurgitation in the CAVC group. A high incidence of severe infections can influence the final results. Repair of CHD in infancy helps to eliminate problems connected with congestive heart failure and pulmonary hypertension.