先天性心脏病肺动脉高压肺血管内皮功能与结构的改变

先天性心脏病肺动脉高压肺血管内皮功能与结构的改变李筠，周爱卿对８９例先天性心脏病肺动脉高压患儿血浆有关物质的测定显示：肺高压组中八因子相关抗原（ｖＷＦ：Ａｇ）及血栓烷素Ｂ＿２／６一酮一前列环素Ｆｉａ（ＴｘＢ，／６一Ｋ一ｐＧＦｉａ）明显升高，不同程度肺...     

儿童动脉导管未闭治疗进展

动脉导管未闭是儿童常见先天性心脏病,及时有效治疗可防止肺炎、心力衰竭、感染性心内膜炎、肺动脉高压等并发症的发生.治疗方法包括药物治疗、经导管介入治疗和手术治疗.目前绝大部分动脉导管未闭通过介入封堵可获治愈.     

前列腺素E1治疗左向右分流先天性心脏病并肺动脉高压的临床研究

目的观察雾化吸入前列腺素E1（PGE1）对左向右分流型先天性心脏病并肺动脉高压患儿的疗效，探讨治疗肺动脉高压的新途径。方法40例患儿分为PGE1雾化吸入治疗组（n=20）和静脉治疗组（n=20），分别于治疗前及治疗后测量肺动脉平均压、肺动脉收缩压、心指数以及Tei指数的变化。结果应用PGE1治疗后，两组肺动脉平均压、肺动脉收缩压、Tei指数均明显下降，心指数较治疗前增大；两组间治疗前后各指标分别比较，差异无显著性（P均〉0．05）。结论PGE1雾化吸入和静脉给药途径均可使肺动脉压下降，临床症状明显改善，而雾化吸入途径较静脉途径更简便、安全。     

吸入一氧化氮治疗先天性心脏病术后肺动脉高压

目的评价吸入一氧化氮(NO)在左向右分流性先天性心脏病(先心病)术后严重肺动脉高压的治疗效果。方法81例先天性心脏病患儿术后予以吸入NO治疗,治疗指征:肺/体动脉收缩压之比(Pp/Ps)>0.5,氧合指数(PaO2/FiO2)<150mmHg,传统降肺动脉压治疗无效。初始吸入浓度为20×10-6vol/vol,根据血气及血流动力学情况进一步调整,不超过45×10-6vol/vol,同时监测二氧化氮(NO2)浓度及高铁血红蛋白浓度。结果81例吸入NO治疗后肺动脉压力明显下降,从(54.0±15.8)mmHg降至(45.7±12.8)mmHg(P<0.01),而体动脉压力无明显变化,同时PaO2/FiO2明显改善,从100.6±40.1升至135.8±29.7(P<0.01)。监测NO2浓度<1×10-6vol/vol,高铁血红蛋白浓度<2%。未发现明显出血及其他不良反应。结论吸入NO治疗可以安全有效地降低肺动脉压力,提高氧合指数,从而改善先心病合并肺动脉高压的预后。     

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     

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.     

室间隔缺损合并中重度肺动脉高压108例外科治疗

探讨先天性心脏病室间缺损合并中重度肺动脉高压的外科治疗及围术期处理。全组１０８例，年龄４－９６个月，体重４．７－２０ｋｇ。均有明显明显症状。其中３２例为双动脉干下型ＶＳＤ，其中４例伴有主动脉瓣脱垂和中主以上主动脉瓣返流。     

先天性心脏病并肺动脉高压的诊治进展

肺动脉高压是先天性心脏病常见的并发症,如不及时诊断和治疗,会影响到先天性心脏病患儿的根治效果。通过先天性心脏病并肺动脉高压的基础和临床研究,对于及时终止肺动脉高压的进行性加剧,改善先天性心脏病患儿预后,具有重要意义。     

小儿先天性心脏病合并肺动脉高压的手术指征及预后

小儿先天性心脏病合并肺动脉高压的手术指征及预后广东心血管病研究所（５１０１００）王慧深，李渝芬，潘微，石继军先天性心脏病（ＣＨＤ）并发肺动脉高压（ＰＨ）的手术适应证至今仍有争议。本文对１９７８年２月至１９９４年２月经左、右心导管或心血管造影确诊的２０...     

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。     

Pharmacokinetics of magnesium sulfate in the sheep model

The pharmacokinetic parameters of MgSO4 were followed in the pregnant sheep model following intravenous dosages of MgSO4 comparable to those used in the therapy of the preeclamptic woman. Hemodynamic parameters, including maternal arterial pressure, central venous pressure, systemic vascular resistance, pulmonary artery pressure, heart rate, cardiac output, cardiac index, rate pressure product, stroke volume, stroke index, blood gases, fetal arterial pressure, heart rate, and blood gases, all remain stable during the infusion of MgSO4. Biochemical changes accompanying MgSO4 infusion in these doses were evaluated. It was found that the fetal serum levels of MgSO4 were approximately 70% of those in the mother. The MgSO4 was rapidly excreted into the maternal urine and 8.9% of the MgSO4 infused was cleared by 2 hr after the termination of the infusion. MgSO4 was also excreted by the fetus into amniotic fluid. It was found that a minimum dosage of 1 mg/kg/hr of magnesium was required to achieve a magnesium level in maternal serum at the lower limit of the therapeutic range of 4 mEq/L.     

Is oxygen supply the only regulator of erythropoietin levels? Serum immunoreactive erythropoietin during the first 4 months of life in term infants with different levels of arterial oxygenation

Serum immunoreactive erythropoietin (siEp) levels were measured in 35 full-term infants aged 0-13 weeks, 31 of whom had congenital heart disease. The infants displayed a wide range in arterial oxygen tension (PaO2) and oxygen saturation (SaO2). During the first days of life siEp varied widely with a range from less than 3 to more than 10,000 mIU/ml. The wide variation is consistent with findings in cord blood at term. The siEp levels did not correlate significantly with haemoglobin, haematocrit, PaO2, SaO2, or arterial oxygen content in the total sample, nor when the cohort was split up into different age groups. Cyanotic infants aged 2-13 weeks had significantly higher siEp concentrations than normal adults (p less than 0.001) and than children with cyanotic congenital heart disease, aged 4 months-10 years (p less than 0.001). The raised siEp levels in cyanotic children aged 2-13 weeks found in this study and the normal levels found in their older counterparts (4 months-10 years) (reported elsewhere) are consistent with the pattern observed in man and animals exposed to prolonged hypobaric hypoxia, in which after an initial rise in erythropoietin concentrations the levels fall to normal while increased erythropoiesis is sustained.     

Is Oxygen Supply the Only Regulator of Erythropoietin Levels?Serum Immunoreactive Erythropoietin during the First 4 Months of Life in Term Infants with Different Levels of Arterial Oxygenation

ABSTRACT. Serum immunoreactive erythropoietin (siEp) levels were measured in 35 full-term infants aged 0–13 weeks, 31 of whom had congenital heart disease. The infants displayed a wide range in arterial oxygen tension (Pa_o2) and oxygen saturation (Sa_o2). During the first days of life siEp varied widely with a range from less than 3 to more than 10000 mIU/ml. The wide variation is consistent with findings in cord blood at term. The siEp levels did not correlate significantly with haemoglobin, haematocrit, Pa_o2, Sa_o2, or arterial oxygen content in the total sample, nor when the cohort was split up into different age groups. Cyanotic infants aged 2–13 weeks had significantly higher siEp concentrations than normal adults ( p <0.001) and than children with cyanotic congenital heart disease, aged 4 months-10 years ( p <0.001). The raised siEp levels in cyanotic children aged 2–13 weeks found in this study and the normal levels found in their older counterparts (4 months-10 years) (reported elsewhere) are consistent with the pattern observed in man and animals exposed to prolonged hypobaric hypoxia, in which after an initial rise in erythropoietin concentrations the levels fall to normal while increased erythropoiesis is sustained.     

Arterial oxygen tension and response to oxygen breathing in differential diagnosis of congenital heart disease in infancy

Arterial oxygen tension was measured from radial artery samples in 276 infants referred for cardiological investigation. Values obtained during air breathing in infants with congenital heart disease showed considerable overlap between 'cyanotic' and 'acyanotic' groups, and are of limited diagnostic use. By contrast, values obtained while breathing oxygen in concentrations of over 80%, measured in 182 infants, allowed clear differentiation between these groups. All infants with acyanotic, but only 2 of 109 with cyanotic lesions, achieved an arterial oxygen tension of more than 150 mmHg. In the cyanotic group the response to oxygen breathing was significantly greater in common mixing situations and in the hypoplastic left heart syndrome than with either pulmonary outflow tract obstruction or transposition of the great arteries. Infants with transposition had a significantly lower mean arterial oxygen tension in air than infants with other forms of cyanotic congenital heart disease. Of 23 infants whose final diagnosis was primary lung disease but in whom cyanotic congenital heart disease had been suspected, 7 achieved arterial oxygen tensions of more than 150 mmHg during oxygen breathing, and on this basis cardiac catheterization was not performed. We therefore conclude that measurement of the arterial oxygen tension while breathing high concentrations of oxygen should be routinely performed in the initial assessment of sick infants with suspected congenital heart disease.     

先天性心脏病婴幼儿体外循环时动脉血氧分压与脑损伤的关系

脑损伤是先天性心脏病婴幼儿围术期的常见并发症之一,病因较成人心脏病复杂,存在于整个围术期过程中,并有其独特的临床特点.其发病机制至今尚未完全明了,有学者认为进行心脏纠正手术体外循环时的再氧合损伤起重要作用.现将近年来关于先天性心脏病婴幼儿体外循环时动脉血氧分压与脑损伤的关系进行综述.     

肾上腺髓质素在先天性心脏病中的测定及临床意义

目的：肾上腺髓质素(ADM)对心血管疾病有多方面的影响,但其与先天性心脏病关系的研究较少。该实验通过检测不同类型先天性心脏病患儿体内ADM的变化,分析ADM在先天性心脏病病理生理中的作用。方法：筛选住院的48例先天性心脏病患儿,经超声心动图及心导管检查术证实,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)>20 mmHg为肺动脉高压的标准。选6例川崎病痊愈患儿作为对照组。采用特异性放射免疫法检测血浆ADM的水平。结果：先天性心脏病患儿股静脉中的血浆ADM水平较股动脉明显增高(P<0.05);与对照组相比,高肺血流并肺高压组及紫绀组中的ADM明显增高,均P<0.01;ADM与主动脉平均压(mSAP)、混合静脉血氧饱和度(MVsat)、主动脉血氧饱和度(AOsat)及肺血管阻力(Rp)之间有密切的关系。结论：高肺血流并肺动脉高压和紫绀型先天性心脏病患儿的ADM水平增高,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。     

Arterial oxygen tension and response to oxygen breathing in differential diagnosis of congenital heart disease in infancy.

Arterial oxygen tension was measured from radial artery samples in 276 infants referred for cardiological investigation. Values obtained during air breathing in infants with congenital heart disease showed considerable overlap between ''cyanotic'' and ''acyanotic'' groups, and are of limited diagnostic use. By contrast, values obtained while breathing oxygen in concentrations of over 80%, measured in 182 infants, allowed clear differentiation between these groups. All infants with acyanotic, but only 2 of 109 with cyanotic lesions, achieved an arterial oxygen tension of more than 150 mmHg. In the cyanotic group the response to oxygen breathing was significantly greater in common mixing situations and in the hypoplastic left heart syndrome than with either pulmonary outflow tract obstruction or transposition of the great arteries. Infants with transposition had a significantly lower mean arterial oxygen tension in air than infants with other forms of cyanotic congenital heart disease. Of 23 infants whose final diagnosis was primary lung disease but in whom cyanotic congenital heart disease had been suspected, 7 achieved arterial oxygen tensions of more than 150 mmHg during oxygen breathing, and on this basis cardiac catheterization was not performed. We therefore conclude that measurement of the arterial oxygen tension while breathing high concentrations of oxygen should be routinely performed in the initial assessment of sick infants with suspected congenital heart disease.     

Acute effects of two different doses of magnesium sulphate in infants with birth asphyxia.

The effects of two different doses of magnesium sulphate (MgSO4) were evaluated in a group of 15 full term infants with Apgar scores of < 6 at 10 minutes, studied within 12 hours of delivery. Seven infants received 400 mg/kg MgSO4 and eight received 250 mg/kg. After the larger dose, mean arterial pressure (MAP) fell by a mean of 6 mm Hg (13%) at one hour but was not significantly reduced thereafter. Respiratory depression lasted three to six hours. EEG readings and heart rate were not significantly different. Mean serum Mg2+ increased from 0.79 to 3.6 mmol/l at one hour. After 250 mg/kg MgSO4, MAP, EEG, tone and heart rate were unchanged. One infant developed transient respiratory depression. Mean serum Mg2+ rose from 0.71 to 2.42 mmol/l at one hour. MgSO4 (400 mg/kg) has an unacceptable risk of hypotension; 250 mg/kg MgSO4 was not associated with hypotension although respiratory depression can occur.     

Effects of oxygen and nitric oxide in oxygen on pulmonary arterial pressures of children with congenital cardiac defects

Inhaled nitric oxide is a specific pulmonary vasodilator. This study was undertaken to assess the effect on pulmonary arterial pressure of administering 100% oxygen compared with nitric oxide in oxygen. Thirteen mechanically ventilated children undergoing routine cardiac catheterization for the investigation of congenital heart disease were studied. Pulmonary arterial pressures were measured during inhalation of 30% oxygen (baseline), 100% oxygen, and nitric oxide (40 parts per million) in oxygen. In addition, in six children the pulmonary/systemic blood flow ratio and pulmonary vascular resistance were calculated using oxygen content, an assumed value for oxygen uptake, and the Fick principle. Results were compared using analysis of variance and the Wilcoxon signed-rank test. Pulmonary arterial pressure decreased from a mean value of 29.5 mmHg (SD 15.1) to 25.6 mmHg (SD 9.3), p= 0.048, after increasing the inspired oxygen fraction from 0.3 to 1.0. The addition of nitric oxide caused a further reduction to 22.9 mmHg (SD 7.9), p= 0.0001. There was no change in systemic arterial pressure or heart rate during the study period, but a small increase occurred in the mean methemoglobin level (1.1% to 1.3%) p= 0.039. Changes in the pulmonary/systemic blood flow ratio and pulmonary vascular resistance (n= 6) were not significant. Nitric oxide in oxygen appears to be a more potent pulmonary vasodilator than oxygen alone in pediatric patients with congenital cardiac defects.