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先天性重度主动脉瓣狭窄合并儿童晚期及青少年期充血性心力衰竭:球囊瓣膜成形术后对左心室功能的影响

Congenital severe aortic stenosis with congestive heart failure in late childhood and adolescence: effect on left ventricular function after balloon valvuloplasty.

作者信息

Jindal R C, Saxena A, Kothari S S, Juneja R, Shrivastava S

机构信息

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Catheter Cardiovasc Interv. 2000 Oct;51(2):168-72. doi: 10.1002/1522-726x(200010)51:2<168::aid-ccd7>3.0.co;2-c.

DOI:10.1002/1522-726x(200010)51:2<168::aid-ccd7>3.0.co;2-c
PMID:11025569
Abstract

Left ventricular (LV) dysfunction with congestive heart failure (CHF) resulting from severe congenital aortic stenosis (AS) is a well-described condition in infancy, but it is rarely found in older children and adolescents. Aortic valve surgery in such cases may be associated with higher rates of morbidity and mortality. Aortic valve balloon dilatation (AVBD) is a viable alternative, but its effect on LV function has not been evaluated. We describe follow-up results of AVBD in 10 cases of severe congenital AS in older children and adolescents with CHF and LV dysfunction. The ages of these patients ranged from 5 to 18 yr (mean +/- SD: 10.8 +/- 4 yr), and nine were males. The follow-up period after AVBD ranged from 3 mo to 7 yr (mean +/- SD: 2.93 +/- 2.1 yr). Success was achieved in all cases, with no immediate complications. After valvuloplasty, the peak-to-peak systolic gradient declined from 74.7 +/- 30.8 to 33.9 +/- 18.2 mm Hg (P < 0.0001). The cardiac index increased slightly but significantly, from 1.9 +/- 0.27 to 2.2 +/- 0.5 L/min/m(2) (P < 0.015). Hemodynamic improvement was also confirmed by a significant decrease in mean pulmonary artery and pulmonary artery wedge pressures from 41.9 +/- 9 to 32.6 +/- 6.6 and from 25.5 +/- 2.9 to 19.3 +/- 3.4 mm Hg, respectively. The echocardiographically derived left ventricular ejection fraction (LVEF) improved from 21.6 +/- 5. 37% to 31 +/- 6.5% within 24 hr after AVBD, and it further improved in all cases on follow-up. Mean LVEF at last follow-up was 59.4 +/- 11.4%. The Doppler instantaneous peak systolic gradient (IPSG) increased from 37.3 +/- 18.8 to 64.8 +/- 30.7 mm Hg at late follow-up. Significant aortic regurgitation (AR) developed in 20% of patients. The Doppler IPSG across the aortic valve was > 60 mm Hg in five cases on follow-up. Two of these patients underwent another AVBD successfully 4 and 16 mo later, respectively. Aortic valve replacement was done in two patients, one for severe restenosis with mild AR 12 mo after AVBD and another for severe re-restenosis with moderate AR 21 mo after a second AVBD. Severe congenital AS can be associated with LV dysfunction and CHF in late childhood and adolescence. AVBD results in good palliation with improvement in LV function on follow-up.

摘要

严重先天性主动脉瓣狭窄(AS)导致的左心室(LV)功能障碍合并充血性心力衰竭(CHF)在婴儿期是一种已被充分描述的病症,但在大龄儿童和青少年中很少见。在此类病例中进行主动脉瓣手术可能会有更高的发病率和死亡率。主动脉瓣球囊扩张术(AVBD)是一种可行的替代方法,但其对左心室功能的影响尚未得到评估。我们描述了10例大龄儿童和青少年严重先天性AS合并CHF及左心室功能障碍患者接受AVBD后的随访结果。这些患者年龄在5至18岁之间(平均±标准差:10.8±4岁),9例为男性。AVBD后的随访期为3个月至7年(平均±标准差:2.93±2.1年)。所有病例均成功,无即刻并发症。瓣膜成形术后,收缩期峰-峰压差从74.7±30.8降至33.9±18.2 mmHg(P<0.0001)。心脏指数略有但显著增加,从1.9±0.27升至2.2±0.5 L/min/m²(P<0.015)。平均肺动脉压和肺动脉楔压分别从41.9±9降至32.6±6.6 mmHg以及从25.5±2.9降至19.3±3.4 mmHg,也证实了血流动力学的改善。超声心动图测得的左心室射血分数(LVEF)在AVBD后24小时内从21.6±5.37%提高到31±6.5%,且在随访中所有病例均进一步改善。末次随访时平均LVEF为59.4±11.4%。随访后期,多普勒瞬时收缩期峰值压差(IPSG)从37.3±18.8升至64.8±30.7 mmHg。20%的患者出现了明显的主动脉瓣反流(AR)。随访中有5例患者主动脉瓣处的多普勒IPSG>60 mmHg。其中2例患者分别在4个月和16个月后成功接受了再次AVBD。2例患者接受了主动脉瓣置换术,1例在AVBD后12个月因严重再狭窄合并轻度AR进行置换,另1例在第二次AVBD后21个月因严重再再狭窄合并中度AR进行置换。严重先天性AS在儿童晚期和青少年期可能与左心室功能障碍及CHF相关。AVBD能实现良好的姑息治疗效果,随访中左心室功能得到改善。

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