先天性心脏病血浆肾上腺髓质素的测定及临床意义

目的 探讨血浆肾上腺髓质素(ADM)的变化对先天性心脏病病理生理的影响.方法 2005年1月至2007年11月筛选在山东大学第二医院儿内科住院的59例先心病患儿,在心导管检查术中测定血流动力学指数及采血备测,依据血流动力学特征分为高肺血流非肺动脉高压组、高肺血流并肺动脉高压组、紫绀组,平均肺动脉压(mPAP)20mmHg(1mmHg=0.133kPa)为肺动脉高压的标准.选6名正常健康儿童作为对照组.采用特异性放射免疫法检测血浆ADM的水平.结果 先心病惠儿股静脉中的血浆ADM水平较股动脉明显增高(P<0.05);与对照组相比,高肺血流并肺动脑高压组及紫绀组中的ADM明显增高,均P<0.01;股静脉血浆ADM与主动脉平均压,主动脉血氧饱和度及混合静脉血氧饱和度均呈显著负相关;而与肺血管阻力有显著正相关.结论 ADM的变化同肺动脉阻力和缺氧有密切关系,推测ADM水平的升高可能有助于减轻肺动脉阻力和改善缺氧.     

先天性心脏病重度肺动脉高压性质的综合评价

目的探讨先天性心脏病（简称先心病）合并重度肺动脉高压患儿器质性肺动脉高压（简称肺高压）的诊断标准。方法３７例经手术治疗后早期肺动脉压力降至正常的患儿作为动力性肺高压组；７例经手术治疗后仍持续性肺动脉高压及６例临床诊断为器质性肺高压而未予手术的共１３例患儿作为器质性肺高压组，对比两组心导管检查血液动力学指标。结果两组肺血管阻力、肺小动脉楔压、肺循环血流量与体循环血流量之比（Ｑｐ／Ｑｓ）及降主动脉血氧饱和度（ＳａＯ２）差异均有显著意义，如按年龄大于２岁、肺动脉阻力＞７２ｋＰａ·ｓ－１·Ｌ－１（９Ｗｏｏｄ单位）、肺小动脉楔压≤１．６ｋＰａ（１２ｍｍＨｇ）、Ｑｐ／Ｑｓ＜２和动脉血氧饱和度＜０．９０作为临床诊断器质性肺高压的指标，则本组动力性肺高压组仅有５．４％的患儿符合上述指标３项或３项以上，而器质性肺高压组所有病例均符合上述指标３项或３项以上。结论先心病合并重度肺动脉高压存在上述５项指标中３项或３项以上，高度提示患儿存在器质性肺动脉高压     

氨基末端脑钠素原在小儿先天性心脏病并肺动脉高压的诊断价值

目的探讨氨基末端脑钠素原(NT-proBNP)在小儿先天性心脏病(先天性心脏病)并肺动脉高压(肺高压)的诊断价值。方法2005年1月至2007年3月山东大学齐鲁儿童医院内科采用竞争性酶免疫法检测20例正常对照组小儿及65例先天性心脏病患儿血清NT-proBNP浓度。65例先天性心脏病患儿经用彩色多普勒超声心动图估测肺动脉压力,分为三组:无肺高压组20例、轻度肺高压组23例、中重度肺高压组22例。结果1、先天性心脏病无肺高压组较正常对照组NT-proBNP水平略有增高,但无明显统计学差异(P>0.05)。2、先天性心脏病并轻度肺高压组NT-proBNP水平明显高于先天性心脏病无肺高压组(P<0.001)。3、中重度肺高压组患儿NT-proBNP水平较轻度肺高压组明显增高(P<0.001)。4、以500 fmol/m l诊断轻度肺高压的敏感度为91%,特异度为90%,且经直线相关分析,显示NT-proBNP与肺高压高低呈正相关,r=0.80。结论血清NT-proBNP检测对先天性心脏病并肺高压具有敏感而特异的诊断价值,且可反映肺动脉压增高程度。     

吸入一氧化氮治疗先天性心脏病术后肺动脉高压的疗效观察

目的 探讨吸入一氧化氮(NO)治疗先天性心脏病术后肺动脉高压患儿的疗效.方法 选择32例先天性心脏病术后传统治疗无效的、难治的、反应性肺动脉高压或肺动脉高压危象患儿,吸入NO(10～25)×10-6,定时记录各项血流动力学和呼吸功能指标,定期监测二氧化氮、高铁血红蛋白含量.结果 吸入NO后,平均肺动脉压(mPAP)从(38.0±3.2)mm Hg(1 mm Hg=0.133 kPa)降至(28.0±2.1)mm Hg,肺血管阻力从(62.2±6.7)kPa/(L·S)降至(49.9±5.6)kPa/(L·S),氧合指数从(67.0±30.1)mm Hg升至(92.6±25.0)mm Hg,动脉血氧饱和度从0.78±0.14升至0.84±0.09,差异均有非常显著性(P＜0.01).吸入NO期间,二氧化氮和高铁血红蛋白含量均在安全范围.结论 NO可以明显降低先天性心脏病术后肺动脉压力和肺血管阻力,是一种安全且理想的肺血管扩张剂.     

心血管系统疾病

341 972590先天性心脏病合并肺动脉高压患儿血浆心钠素含量的变化及临床意义/滕月娥二声,},国实用儿科杂志.一19,6、11(5)一299一3阴 研究结果人明.患儿血浆免疫性心房利钠多肤(;rANP),先亡病月!1}‘常明显高于健康对照组。肺高压组明显高J几仆肺高)]组先心病.蕊压组的肺动脉血浆高干外周血浆肺高压组术后ANP水平明显降低肺高压组肺动脉血.浆、外周血浆ANP水平与肺动脉压力、肺血管阻力、QP/QS显著正相关。(董贵章) 972591法乐四联症患儿心肌细胞和红细胞内外钙的变化/李自普…/)’中华)L科杂志一1997,35(3),一142一145 12例测定结果…     

前列地尔对先天性心脏病合并重度肺动脉高压的治疗作用

目的观察前列地尔脂微球载体制剂(LipoPGE1,商品名:凯时)对高肺血流性先天性心脏病(先心病)患儿的肺循环和体循环压力及阻力的影响作用。方法将50例先心病合并重度肺动脉高压的患儿随机设为治疗组(25例),于右心导管检查术中直接静脉推注凯时及对照组(25例),推注立其丁,观察肺动脉(PA)、主动脉(AO)、肺毛细血管(PW)压力,以及肺循环阻力、体循环阻力和心率的改变。结果凯时能有效降低PA压力(P<0.001),对AO压力和心率影响不明显;而立其丁在降低PA压力的同时,也使AO压力下降,并明显加快心率(P均<0.001)。结论对于先心病合并肺动脉高压的患儿,凯时可能是一种比较理想的选择性降低PA压力的制剂。     

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目的 探讨雾化吸入伊洛前列素进行急性血管扩张试验的价值.方法 研究对象为2007年2月至2008年5月在广东省心血管病研究所住院的50例先天性心脏病合并肺动脉高压患儿,对所有患儿进行左右心导管检查,之后肺动脉内注射酚妥拉明或雾化吸入伊洛前列素进行急性血管扩张试验,试验后重复左右心导管检查.根据Fick公式计算血流动力学参数.综合判断肺动脉高压性质,将惠几分成两组:动力组和梗阻组.动力组患儿进行手术治疗,术后定期随访并修正术前诊断.结果 酚妥拉明会显著增高受试者的心率,而伊洛前列素对心率的影响较轻微;酚妥拉明和伊洛前列素都能够降低平均肺动脉压力和肺血管阻力,升高肺循环血流量;酚妥拉明同时会降低平均主动脉压力和体循环阻力,升高体循环血流量,而伊洛前列素对体循环没有明显的影响.在使用伊洛前列素的急性血管扩张试验中,平均肺动脉压力、肺血管阻力/体循环阻力和肺循环血流量/体循环血流量等参数的变化在动力组和梗阻组中差异无统计学意义(P值分别为0.016、0.024和0.030).而使用酚妥拉明的急性血管扩张试验中,平均肺动脉压力和肺血管阻力两项参数的变化在动力组和梗阻组中差异有统计学意义(P值分别为0.017和0.004).结论 在先天性心脏病合并肺动脉高压的患儿中,使用酚妥拉明或伊洛前列素进行急性血管扩张试验都能够有效区分动力性与梗阻性肺动脉高压.酚妥拉明用药前后,肺循环和体循环的压力、阻力和血流量都有明显变化.而伊洛前列素雾化吸入以影响肺循环为主,可以保持相对平稳的血流动力学,在安全性上优于酚妥拉明.     

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.     

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2006年,我国在儿童心血管疾病的临床和基础研究方面均取得了长足的进展,现综述如下。1心血管疾病发病中气体信号因子的病理生理学意义北京大学第一医院在国家杰出青年基金的资助下,率先提出了硫化氢(H2S)是继一氧化氮(NO)和一氧化碳(CO)之后的心血管功能调节的新型气体信号因子,对其在各种心血管疾病发病中的病理生理学意义展开了深入而广泛的研究。研究发现,先天性心脏病患儿血浆NO水平明显升高。先天性心脏病并中重度肺动脉高压的患儿其血浆H2S水平明显降低,肺动脉收缩压与血浆H2S水平呈负相关[1];在高肺血流性肺动脉高压动物模型上…     

新生儿持续肺动脉高压与肺血管内皮细胞功能

新生儿持续肺动脉高压 (ｐｅｒｓｉｔｅｎｔｐｕｌｍｏｎａｒｙｈｙｐｅｒｔｅｎｓｉｏｎｏｆｎｅｗｂｏｒｎ ,ＰＰＨＮ)是指新生儿期肺血管阻力增高及肺血流速度增加而发生的临床综合征。其发生率为活产儿的 1‰ ,是新生儿多种疾病引起死亡的最终病理途径。本症多继发于先天性心脏病、慢性缺氧或急性呼吸衰竭 ,其中主要为肺实质疾病 ,特点为严重肺动脉高压 ,导致肺外右向左血液分流及低氧血症[1] 。1　ＰＰＨＮ病理生理改变1 1　肺血流速度增加　伴有肺内或心内分流的新生儿 ,随着肺血管阻力降低 ,肺血流速度 (Ｑｐ)常增加 ,使肺血管床扩张…     

Two molecular forms of adrenomedullin in congenital heart disease

To investigate the pathophysiological role of two forms of adrenomedullin (AM), a mature AM (AM-m) and a glycine-extended AM (AM-Gly), in congenital heart disease, we measured plasma levels of AM in patients with cyanotic heart disease, high pulmonary blood flow without pulmonary hypertension (PH), high pulmonary blood flow with PH, Fontan procedure, intracardiac repair without complication, and intracardiac repair with PH and control subjects. Plasma AM-m and AM-Gly were increased only for cyanotic heart disease (2. 5 ± 1.3 pmol/L, p < 0.001; 13.1 ± 6.2 pmol/L, p < 0.05) and intracardiac repair with PH (2.3 ± 1.5 pmol/L, p < 0.01; 13.0 ± 7.0 pmol/L, p < 0.05) compared with control (1.0 ± 1.4 and 8.6 ± 1.3 pmol/L, respectively). They were similarly correlated with mean systemic arterial pressure (r = –0.40 and –0.37 respectively; p < 0.001), mixed venous oxygen saturation (r = –0.60 and –0.50; p < 0.0001), systemic arterial oxygen saturation (SA_sat) (r = –0.56 and –0.46; p < 0.0001), and pulmonary arterial resistance (Rp) (r = 0.41 and 0.38; p < 0.005). Multiple regression analysis revealed that SA_sat and Rp were independently correlated with AM. Interestingly, the venous AM-m level was significantly higher than the arterial AM-m, suggesting that the mature form is extracted in pulmonary circulation, whereas there were no venoarterial differences in AM-Gly. These results suggest that plasma AM-m and AM-Gly are similarly regulated and the main clearance site of AM-m is the lung in patients with congenital heart disease.     

Plasma Levels of Nitrate in Congenital Heart Disease: Comparison with Healthy Children

Nitric oxide (NO) is an endothelium- derived relaxing factor, and plasma nitrate is the stable end product of NO production. The aim of this study was to investigate the change in levels of plasma nitrate according to age and to elucidate the effect of pulmonary hypertension (PH) associated with congenital heart disease on NO production. We measured plasma levels of nitrate in 48 healthy children aged 5 days to 12 years to establish the normal range. Forty-six preoperative patients aged 4 months to 12 years with congenital heart disease were studied by cardiac catheterization. Plasma nitrate in healthy children decreased with age, from 1 month to 1 year, and then remained almost constant until the age of 12 years. Plasma nitrate was significantly increased in 22 preoperative patients with PH (mean pulmonary arterial pressure >?25 mmHg) compared with age-matched normal controls: (mean 56.9 vs 33.5 μmol/L, p<0.05) and was significantly correlated with pulmonary to systemic pressure ratio (r= 0.83, p < 0.0001). There was no significant difference between plasma nitrate levels in 24 preoperative patients without PH and those in the age-matched normal control (mean 25.6 vs 24.9 μmol/L). In 10 patients with preoperative PH who were examined before and after surgery, plasma nitrate levels remained high in the cases with residual PH but decreased to the normal range in the cases without residual PH. Plasma nitrate level is useful for evaluating PH both before and after operation in patients more than 4 months of age, and it is important to note differences in normal plasma nitrate levels according to age.     

Oxygen desaturation during sleep in infants and young children with congenital heart disease.

Oxygen saturation (SaO2) during sleep and pulmonary functions were evaluated in 19 infants with congenital heart disease, aged 6 +/- 4 months, and in 11 normal infants, aged 8 +/- 5 months, to determine whether infants with congenital heart disease have more frequent oxygen desaturation during sleep and, if so, its relationship to underlying pulmonary function. Infants with congenital heart disease were classified as acyanotic (n = 11) or cyanotic (n = 8) on the basis of their aortic SaO2 at the time of cardiac catheterization (greater or less than 90% SaO2). Pulmonary function tests included respiratory rate, functional residual capacity, total respiratory system compliance, and maximal flows at functional residual capacity. Significant differences were found in the values for the lowest SaO2 of each 5-minute epoch (SaO2L) averaged during the entire sleep time (normal 94% +/- 2%, acyanotic 90% +/- 3%, and cyanotic 74% +/- 4%; p less than 0.01). The three groups also differed significantly in frequency distributions of percentage of total sleep time with SaO2L less than 90% (SaO2%T) (normal 10% +/- 17%, acyanotic 36% +/- 34%, and cyanotic 97% +/- 4%; p less than 0.05). Compared with the control group, the acyanotic group had a higher respiratory rate (66 +/- 19 breaths/min vs 35 +/- 6 breaths/min; p less than 0.01), a lower tidal volume (65% +/- 29% predicted vs 105% +/- 18% predicted; p less than 0.01), and a lower total respiratory compliance (59% +/- 18% predicted vs 106% +/- 30% predicted; p less than 0.01). A negative correlation existed between SaO2%T and aortic SaO2 (R2 = 0.64; p less than 0.01). We conclude that oxygen desaturation occurs during sleep in infants with congenital heart disease; the presence of desaturation appears to be related to the initial degree of hypoxemia and the presence of abnormal pulmonary function.     

Is adrenomedullin involved in the pathophysiology of persistent pulmonary hypertension of the newborn?

Although adrenomedullin (ADM) is a potent vasodilating peptide reported to play a possible role in the mechanisms of fetal lung differentiation and maturation, the ADM blood level in fetuses and in neonates with persistent pulmonary hypertension (PPHN) and pulmonary hypoplasia is not known. Therefore, we examined 15 patients with PPHN: 10 with congenital diaphragmatic hernia, four with congenital cystic adenomatoid malformation of the lung, and one with misalignment of pulmonary vessels with alveolar capillary dysplasia. Eight surgical patients with neonatal conditions such as intestinal atresia served as controls. Blood samples were drawn from the umbilical artery and vein at birth, and arterial blood was drawn from patients with PPHN on the 3rd and 6th days after birth. Plasma levels of ADM were measured by radiometric assay. Plasma levels of ADM in the umbilical artery and vein were elevated in patients with PPHN compared with controls, and in all groups the levels in the umbilical vein were higher than those in the umbilical artery. The arterial levels in patients with poor prognoses were elevated on the 3rd and 6th days after birth compared with those in survivors. These results indicate that ADM may be involved in the pathophysiology of PPHN and in the mechanisms of lung differentiation and/or maturation.     

Nitric oxide-related compounds in patients with congenital heart defects and pulmonary hypertension

BACKGROUND: The relationship between blood levels of nitric oxide (NO)-related compounds in patients with congenital heart defects (CHD) and degree of pulmonary hypertension (PH) has not yet been described. METHODS: Thirty-six patients (aged 6 months to 19 years) with CHD were divided into three groups on the basis of their hemodynamic characteristics: group 1 (control, n = 5), left-to-right shunt (-) without PH (pulmonary to systemic artery peak pressure ratio, Pp/Ps < 0.4); group 2 (n = 14), left-to-right shunt (+) without PH; group 3 (n = 17), left-to-right shunt (+) with PH (Pp/Ps > 0.4). Blood samples were obtained from the right atrium, pulmonary artery, left atrium or pulmonary capillary wedge and aorta during cardiac catheterization. All NO-related compounds in whole blood were measured by chemiluminescent assay using Sievers NO analyzer. RESULTS: The sampling site had no significant impact on NO-related compound levels. However, the patients with PH (group 3) had significantly higher (P < 0.01) blood levels of NO-related compounds (117.3 +/- 14.7 mumol/L) than the patients without PH (groups 1 and 2, 23.9 +/- 3.2 and 38.4 +/- 4.8 mumol/L, respectively). In addition, pulmonary artery resistance (Rp) values of less than 6 Wood U/m2 were directly correlated with levels of NO-related compounds, whereas Rp values greater than 6 Wood U/m2 were inversely correlated with blood levels of NO-related compounds. CONCLUSION: The present results suggest that the hemodynamic status of the pulmonary circulation in CHD affects, at least partly, blood levels of NO-related compounds.     

先天性心脏病并心力衰竭患儿血浆肾上腺髓质素和内皮素水平变化的意义

目的探讨左向右分流先天性心脏病(CHD)患儿并充血性心力衰竭(CHF)血浆肾上腺髓质素(ADM)和内皮素(ET-1)变化的意义。方法左向右分流CHD并CHF患儿18例。正常对照组20例,检测CHD患儿心力衰竭期和症状、心衰体征消失的恢复期及对照组儿童血浆ADM和ET-1。结果CHF急性期ADM、ET-1明显高于恢复期和正常对照,恢复期ADM或ET-1仍高于正常对照组(P<0.01或0.02)。结论ADM和ET-1均参与了左向右分流CHD并CHF的病理生理过程,观察其血浆水平变化,可能对判断心衰患者病情有一定意义。     

肾上腺髓质素缓解低氧性肺动脉高压大鼠肺动脉中胶原堆积的研究

目的：观察长期应用肾上腺髓质素（adrenomedullin, ADM）对慢性低氧性肺动脉高压大鼠肺血管胶原代谢的影响,以探讨ADM对慢性低氧性肺血管结构重建的作用及其可能机制。方法：19只雄性Wistar大鼠随机分为对照组（n=6）、低氧组（n=7）、低氧+ADM组（n=6）。对于低氧+ADM组大鼠,通过微量渗透泵皮下持续给予ADM（300 ng/h）2周。低氧2周后,以右心导管法测定肺动脉平均压,检测右心室与左心室加室间隔比值,观测肺血管显微变化。用免疫组化法检测肺动脉中胶原I、胶原III和转化生长因子（TGF）-β的表达。结果：低氧2周后,大鼠肺动脉平均压明显升高(P<0.01),右心室与左心室加室间隔的比值明显增加(P<0.01),肺小血管肌化程度和肺动脉相对中膜厚度较对照组均明显增高（P<0.01）,同时肺动脉胶原I、胶原III和TGF-β表达增强。与低氧组大鼠相比,低氧+ADM组大鼠肺动脉平均压明显降低（P<0.01）,右心室与左心室加室间隔的比值明显下降（P<0.01）,肺小血管肌化程度和肺动脉相对中膜厚度较低氧组均明显降低（均P<0.01）,同时肺动脉胶原I、胶原III和TGF-β表达减弱。结论：外源性补充ADM可能通过抑制肺动脉TGF-β表达,减少肺动脉壁胶原异常堆积,对低氧性肺动脉高压和肺血管结构重建发挥调节作用。     

复杂性先天性心脏病的介入治疗

目的探讨先天性心脏病(简称先心病)介入治疗在复杂性先心病中的应用.方法应用Rashkind球囊导管对59例复杂性先心病患儿行球囊房隔造口术(BAS);对2例右室发育不良综合征患儿进行静态球囊房隔扩张造口术;对1例在大动脉转位术后出现肺动脉吻合口狭窄的完全性大动脉转位(TGA)的患儿,采用球囊扩张导管进行扩张;对15例法洛四联症(TOF)患儿进行经皮球囊肺动脉瓣成形术(PBPV)、球囊血管成形术;对18例合并主动脉至肺动脉侧支血管的患儿以及5例B-T分流术的患儿在外科根治术前采用弹簧圈进行侧支血管和分流管道的堵塞术;对1例Fontan手术后的先心病患儿采用Amplatzer房间隔缺损(简称房缺)堵塞装置进行窗孔堵塞术.结果46例TGA患儿BAS后动脉血氧饱和度(SaO2)由0.57±0.17上升至0.76±0.13(t=14.58, P<0.01),左右心房压差﹤2 mmHg(1 mmHg=0.133 kPa),术后房缺5～20 mm;10例肺动脉闭锁伴室间隔完整的患儿BAS后SaO2变化不大(t=1.57, P﹥0.05),左右心房压差﹤2 mmHg;TOF患儿经PBPV及球囊血管成形术后SaO2平均上升15%,肺动脉瓣狭窄解除;18例主动脉至肺动脉侧支血管堵塞术,14例效果良好被完全堵塞;5例B-T分流管道堵塞术,全部封堵成功;1例Fontan手术后窗孔堵塞术,获完全封堵.结论复杂性先心病应用介入治疗和外科手术相配合,可获得更好的疗效.     

Chronic hypoxemia leads to reduced serum IGF-I levels in cyanotic congenital heart disease

Cyanotic congenital heart disease in children commonly causes more pronounced growth retardation in comparison with acyanotic congenital heart disease. Chronic hypoxemia has been suggested as the cause of poor growth in these patients, but the relationship between serum IGF-I levels and chronic hypoxemia is unclear. Serum IGF-I concentrations, oxygen saturation and nutritional status were evaluated in 29 patients with cyanotic congenital heart disease, and serum IGF-I levels were compared with a group of 20 well-nourished, age-matched control children to assess the relationship between IGF-I levels and chronic hypoxemia. The nutritional status of each patient was determined by using anthropometric parameters and calorie and protein intake ratios. The patients were divided into malnourished and well-nourished groups (21 and 8 patients, respectively) according to their nutritional status. Serum IGF-I concentrations were measured in the two patient groups and the controls. The malnourished group had the lowest IGF-I levels (48.14 +/- 21.8 ng/ml, p<0.05). However, the well-nourished group's IGF-I levels were significantly lower than the control subjects' despite improved nutritional status (85.5 +/- 30.2 and 107 +/- 19.7 ng/ml, respectively, p<0.05). In addition, we found a positive correlation between serum IGF-I levels and oxygen saturation of the patients (r=0.402, p<0.05). These findings indicate that chronic hypoxemia has a direct or indirect effect to reduce serum IGF-I concentrations and this may be a cause of the increased growth failure in patients with cyanotic congenital heart disease.     

Abnormal vascular tone in infants and children with lung hypoplasia: Findings from cardiac catheterization and the response to chronic therapy.

OBJECTIVE: We describe four cases of chronic pulmonary hypertension in infants and children with chronic lung disease and pulmonary hypoplasia due to severe congenital diaphragmatic hernia (CDH) or congenital cystic adenomatoid malformation (CCAM). We report data from cardiac catheterization under various conditions: baseline respiratory support and room air, hyperoxic and inhaled nitric oxide challenge. We further report cardiac catheterization measures after chronic pulmonary vasodilator therapy with sildenafil alone or a combination of sildenafil and inhaled nitric oxide (three patients). DESIGN: Case series. SETTING: Tertiary academic center. PATIENTS: Infants and children ages 0-11 yrs with CDH (n = 3) or CCAM (n = 1) with evidence of chronic pulmonary hypertension by echocardiogram and cor pulmonale (n = 3). INTERVENTIONS: Catheterization and pulmonary vasodilator therapy. MEASUREMENTS AND MAIN RESULTS: Pulmonary vascular resistance, pulmonary arterial pressure, and changes in these measures were assessed. A 20% change in pulmonary vascular resistance was considered a clinically significant response. Ten catheterizations were performed in four patients. All patients had elevated pulmonary vascular resistance and pulmonary arterial pressures at initial catheterizations and significant vasodilation during inhaled nitric oxide. CONCLUSIONS: Chronic lung disease following pulmonary hypoplasia from CDH and CCAM is associated with abnormal pulmonary vascular tone in infants and children with evidence of chronic pulmonary hypertension. Chronic pulmonary vasodilator therapy may improve pulmonary vascular function and enhance lung growth in infants and children who are treated during their period of potential for rapid lung growth.