磁共振技术对新生儿青紫型先天性心脏病的诊断意义

近年来,随着心脏外科的发展,儿童先天性心脏病(简称先心病)的手术年龄越来越小,对于某些先心病如完全性大动脉转位等,在新生儿期手术治疗的效果远比新生儿期后手术治疗效果要好。新生儿先心病外科手术要求术前精确地显示心脏畸形的形态,这就对新生儿心脏影像诊断提出了很高的要求。超声心动图是新生儿心脏影像检查的首选方法,能很好地显示心内结构异常,但对于心外大血管异常和复杂性先心病有时还需要其他影像检查方法作为补充。心血管造影、多层螺旋CT、电子束CT和磁共振检查都能补充超声心动图的不足,但前3种检查方法患儿均需接受较大量…     

Stent implantation into the patent ductus arteriosus in cyanotic congenital heart disease with duct-dependent or diminished pulmonary circulation

We assessed the efficacy and safety of stent implantation into the ductus arteriosus in infants with cyanotic heart disease. Ten patients with duct-dependent or diminished pulmonary circulation underwent cardiac catheterization with the aim of stent implantation. Coronary stents of 3.5-4 mm in diameter were used and successfully implanted in eight. All patients in whom stent implantation was successful had adequate relief of their cyanosis and were discharged home. During a mean follow-up of 9.3 months (median 10 months), one patient had evidence of restenosis of the stent at five months. Re-dilatation of the stenosed stent was attempted but was unsuccessful and a modified left Blalock-Taussig shunt was placed surgically. Stent implantation of ductus arteriosus in neonates and infants with a duct-dependent pulmonary circulation is an effective and safe palliative procedure. It may be an alternative to surgical systemic-pulmonary artery shunt in a select group of patients.     

Cardiac magnetic resonance imaging in children with congenital heart disease

Electrocardiogram-gated magnetic resonance imaging (MRI) was used to evaluate 36 children, ages 2 to 17 years, with congenital heart disease. With the use of multiple imaging planes, including transverse, sagittal, coronal, and 60-degree left anterior oblique views, high contrast images with excellent spatial resolution were produced. In 34 of the 36 patients the anatomic detail provided by MRI was sufficient to make the cardiac diagnosis. Electrocardiogram-gated MRI is an important new imaging technique for use in children with cardiovascular disease.     

Systemic to pulmonary arterial shunts for patients with cyanotic congenital heart disease: Current status

The various central vascular shunting operations used for palliation of patients with congenital heart disease plus decreased pulmonary blood flow are discussed. The status of each in current practice is outlined in order to provide an up-to-data reference for the pediatric radiologist. In general, the Waterston shunt is preferred for infants under six months and the Blalock-Taussig anastomosis for patients older than this.Supported by: Grant 260, Children's Bureau, Maternal and Child Health Service, Department of Health, Education and Welfare, Washington, D. C.; and grants from the National Heart and Lung Institute of the National Institutes of Health, the John A. Hartford Foundation, Inc., and the Max Kade Foundation, Inc.     

Fibrinogen half-life in children with cyanotic congenital heart disease

Fibrinogen half-life was determined in 3 healthy individuals and in 17 children with cyanotic congenital heart disease. Fibrinogen half-life was normal in the healthy individuals and shortened in 9 of the 17 patients. The simultaneously performed coagulation tests were often pathological but did not lead to a clear diagnosis of the haemostatic defect and did not correlate with the fibrinogen half-life. Significant correlations were found between fibrinogen half-life and red cell count (P<0.001), packed cell volume (P<0.001), mean corpuscular volume of the red cell (P<0.001), platelet count (P<0.01), aortic oxygen saturation (P<0.001), base excess (P<0.01), and maximal amplitude of the thrombelastography (P<0.001). The results indicate a definite relationship between the hypoxaemia and the frequently observed disturbance of haemostasis in cyanotic heart disease. The chronic hypoxaemia causes a partly compensated intravascular coagulation with increased fibrinogen metabolism.Supported by Land Nordrhein-Westfalen, Ministerium für Wissenschaft und Forschung     

Retinopathy of prematurity in infants with cyanotic congenital heart disease

We undertook a study of premature infants with cyanotic congenital heart disease to determine whether these infants develop retinopathy of prematurity despite a persistent hypoxemic state. Using the computerized registry of the neonatal intensive care unit of Vanderbilt University Medical Center, Nashville, Tenn, we identified six premature infants (less than 37 weeks' gestational age, with birth weights of 1100 to 2050 g) with cyanotic congenital heart disease who survived the neonatal period and underwent ophthalmologic evaluation. Review of their charts revealed that three of six infants developed retinopathy of prematurity (two had grade 1 and one had grade 3 disease), but none required treatment. Our data support the findings of other investigators that elevated arterial oxygen tension is not the sole factor leading to the development of retinopathy of prematurity. Premature infants with cyanotic congenital heart disease can develop retinopathy of prematurity despite persistent hypoxemia. Cyanotic premature infants should be screened for retinopathy of prematurity with the same thoroughness as other premature infants.     

新生儿青紫型先天性心脏病围术期心力衰竭的紧急治疗

对于青紫型先天性心脏病（简称先心病）的新生儿来说，围术期是极特殊的应激阶段。除了心脏畸形对血流动力学的影响外，患儿还受到心导管、心脏手术、麻醉和体外循环等的干扰影响。心脏术后重建血流动力学、心脏功能重新调整、体液酸碱和电解质失衡、内源性和外源性儿茶酚胺升高使病情更复杂，需在细致的治疗下度过危险期。新生儿先心病围术期心力衰竭的急救重点是积极生命支持和保持动脉导管开放。其他抢救措施包括保持通气、纠正代谢性酸中毒、补充血容量、应用正性肌力药物等增加心输出量和改善组织灌注，同时注意纠正低血糖和监控感染。     

Renal involvement in patients with congenital cyanotic heart disease.

Patients with congenital cyanotic heart disease may develop a glomerulopathy with proteinuria and impaired renal function. In order to investigate this problem we conducted a study on 27 patients with uncorrected cyanotic heart disease who were between 1 day and 25 years old. As a consequence of hypoxaemia haematocrit was elevated to 57%. Proteinuria was above 150 mg/day/1.73 m2 body surface in 12 patients. Only one of 9 children under 10 years of age had pathological proteinuria presenting as isolated albuminuria. Seven out of 10 patients between 11 and 20 years had an elevated proteinuria with a glomerular pattern. Creatinine clearance was normal in these patients. All four patients above 20 years of age had a considerable glomerular proteinuria with a mean excretion of 5.7 g/24 h/1.73 m2 body surface. These patients suffered additionally from chronic cardiac failure and creatinine clearance was below the normal range. There was a clear relationship between pathological proteinuria and age of the patients and thus duration of hypoxaemia. Patients with pathological proteinuria had a significant higher erythrocyte count (7.3 +/- 1.3 vs 5.6 +/- 1.4 10(12)/l p less than 0.01) and a lower mean corpuscular haemoglobin. In summary, children with persistent congenital cyanotic heart disease have substantial risk of developing a glomerulopathy if the cyanosis remains unchanged for more than ten years.     

Renal impairment in patients with long-standing cyanotic congenital heart disease

Nephropathy is known to occur in patients with long-standing cyanotic congenital heart disease (CCHD). In order to assess the incidence, nature and degree of the problem among such patients, discriminating urine analyses were performed in 26 patients with CCHD, with a mean age of 22 (10-42) y. Ten patients showed reduced glomerular function, six of whom also had advanced glomerulopathy. Glomerular filtration rates were below normal in half of the patients and occurred with glomerular-type proteinuria in five, with tubular-type proteinuria in one and without pathological proteinuria in four. An elevated haematocrit and duration of cyanosis were identified as the main risks factors for the development of glomerulopathy. The risk of developing glomerular lesions rose sharply during the second decade of life. Nephropathy in CCHD is common and the dominant feature is glomerular damage, which is related to the duration of cyanosis and the extent to which the haematocrit is elevated.     

磁共振成像及电影磁共振成像诊断小儿先天性心脏病

为评价磁共振成像（ＭＲＩ）及电影ＭＲＩ（Ｃｉｎｅ－ＭＲＩ）在小儿先天性心脏病（简称先心病）中的诊断价值，对３４例先心病患儿进行心脏ＭＲＩ及Ｃｉｎｅ－ＭＲＩ成像，并与二维超声心动图（２ＤＥ）、心血管造影（ＣＡＧ）及手术结果进行对照分析。结果显示，ＭＲＩ测量６例左向右分流型先心病患儿缺损口或内径值与手术结果完全吻合（Ｐ＝０．９２４）。Ｃｉｎｅ－ＭＲＩ诊断２８例复杂型先心病，其中２７例与ＣＡＧ结果一致。提示，ＭＲＩ及Ｃｉｎｅ－ＭＲＩ在小儿先心病诊断上有较大的临床应用价值。     

紫绀型先天性心脏病患儿脑组织磁共振波谱研究

目的 应用氢质子磁共振波谱(1H-MRS)技术评估紫绀型先天性心脏病患儿脑代谢的变化.方法紫绀型先天性心脏病患儿29例,按不同的血氧饱和度(SaO2)分为紫绀组14例(SaO2为76% ～ 85%)、严重紫绀组15例(SaO2为65% ～ 75%);另选血氧饱和度正常、年龄性别相匹配的对照组儿童30例.应用场强为1.5T超导型磁共振扫描仪分别对这些儿童的右侧基底核区(感兴趣区20 mm × 20 mm × 20 mm 范围)进行氢质子磁共振波谱图像采集.检测指标为N-乙酰天冬氨酸(NAA)、乳酸(Lac)和肌酸(Cr),计算并比较紫绀型先天性心脏病患儿与对照组儿童、严重紫绀组与紫绀组患儿之间的NAA/Cr和Lac/Cr比值的差异.采用SPSS11.0统计软件进行统计分析.结果 紫绀型先天性心脏病患儿的NAA/Cr比值显著低于对照组(P ＜ 0.05),而Lac/Cr比值则明显高于对照组(P ＜ 0.001),差异均有统计学意义.严重紫绀组患儿的NAA/Cr比值也较紫绀组低(P ＜ 0.001),而Lac/Cr比值则较紫绀组高(P ＜ 0.001),差异均有统计学意义.结论 紫绀型先天性心脏病患儿脑组织存在NAA及Lac代谢异常,其改变程度与缺氧严重程度相关,提示慢性缺氧影响紫绀型先天性心脏病患儿的脑代谢.这将有助于阐明紫绀型先天性心脏病患儿脑功能障碍的发生机制,为早期诊断、及时干预,进而提高这些患儿的远期生活质量提供理论依据.