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主动脉缩窄96例临床分析
作者姓名:Liu F  Huang GY  Liang XC  Sheng F  Lu Y  Wu L  Xu SM  Ning SB
作者单位:200032,上海,复旦大学附属儿科医院心血管中心内科
摘    要:目的观察主动脉缩窄(CoA)的发病情况、分型和伴发畸形,并探讨其诊断及治疗。方法回顾性分析复旦大学附属儿科医院近15年收治的CoA96例,包括发病情况、病理分型、合并畸形、诊断、治疗和预后等。结果96例患者中男女比例为1.7:1,6个月以下婴儿占60%,占同期住院先心病患者数的百分比逐年上升,自1990年0.5%(5/924)上升至2005年4.3%(15/350)。其中93例为左锁骨下动脉远端正对动脉导管处或动脉导管附近缩窄,2例缩窄位于左颈总与左锁骨下动脉间,1例位于左锁骨下动脉开口处。单纯CoA仅占14%,合并室间隔缺损(室缺)或动脉导管未闭各占49%,其中23%同时合并室缺及动脉导管未闭,其他合并畸形包括主动脉瓣畸形或狭窄、完全性房室隔缺损等。超声心动图诊断89例,漏诊7例(7.3%)。55例施行手术治疗,死亡2例(死亡率3.8%),其中7例分期手术,为合并室缺及重度肺动脉高压年长儿或一般情况差的小婴儿,无死亡病例。术后近期复查超声心动图均较满意。结论我国CoA发病率与西方国家无明显差别,缩窄主要发生在左锁骨下动脉远端动脉导管附近,常伴发室间隔缺损和/或动脉导管未闭;超声心动图为首选检查方法,但必要时需进行心导管检查;婴幼儿可采用一期根治术,但对于合并室缺肺高压、一般情况较差患儿,可实施分期手术。

关 键 词:主动脉瓣狭窄  诊断  治疗
收稿时间:2005-11-07
修稿时间:2005-11-07

Clinical features of coarctation of aorta: analysis of 96 cases
Liu F,Huang GY,Liang XC,Sheng F,Lu Y,Wu L,Xu SM,Ning SB.Clinical features of coarctation of aorta: analysis of 96 cases[J].National Medical Journal of China,2006,86(26):1854-1856.
Authors:Liu Fang  Huang Guo-ying  Liang Xue-cun  Sheng Feng  Lu Ying  Wu Lin  Xu Su-mei  Ning Shou-bao
Institution:Cardiovascular Center, Children's Hospital of Fudan University, Shanghai 200032, China.
Abstract:Objective To investigate the clinical features of coarctation of aorta (CoA). Methods The clinical data of 96 pediatric patients with CoA, 60 male and 36 female, aged 3.7 months (7-12 years), were analyzed. Results The male to female ratio was 1.7:1. Infants aged less than 6 months accounted for 60% (57/96). The proportion of CoA in all hospitalized patients with congenital heart diseases admitted in this hospital was 0. 5% (5/924) in 1996, increased every year, and reached 4.3% (15/330) in 2005. The coarctation was situated at the distal end of the left subclavian artery opposite to or near the ductus arteriosus in 93 cases, between the left common carotid artery and left subclavian artery in 2 cases, and at the opening of the left subclavian artery in 1 case. Thirteen patients (4% ) suffered only from CoA, 47 patients were complicated with patent ductus arteriosus (PDA, 47/96, 49% ) and/or ventricular septum defect (VSD, 47/96, 49% ) , 22 of the 96 patients (23% ) complicated with both PDA and VSD. Eighty-nine cases were diagnosed by echocardiography, however, echocardiography failed to diagnose CoA in 7 cases (7.3%). Fifty-five patients underwent surgical repair and 2 of them died with a mortality of 3. 8%. Seven patients with large VSD and severe pulmonary hypertension underwent two-stage repair. Immediate post-operative echocardiography showed satisfactory outcome. Conclusion The morbidity of CoA among Chinese is similar to that among the Western population. Most of the coarctation is situated at the distal end of the left subclavian artery opposite to or near the ductus arteriosus, and most of the cases are complicated by PDA and/or VSD. Echocardiography is the first choice in the diagnosis of CoA; however, angiography is still necessary in some cases. Primary radical operation is indicated for infants with CoA, but older patients, especially those complicated with VSD and severe pulmonary hypertension, should undergo two-stage procedure.
Keywords:Aortic valve stenosis  Diagnosis  Therapy
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