室间隔缺损合并肺动脉高压肺血管床功能状诚评估指标的研究

探讨室间隔缺损合并肺动脉高压肺血和功能状态的评估指标。方法 对室间隔缺损合并重度肺动脉高压的患儿，于心导管术中应用酚妥拉明，将轻工全肺循环阻力增加的２７例与重度全肺循环阻力增加的１２例患儿的试验结果进行比较。结果 两组患儿的单一肺动脉压降幅分别为２．３ｋＰａ和２．２ｋｐＡ，Ｐ〉０．０５差异无显著性意义；     

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     

小儿扩张型心肌病肺动脉压的多普勒超声估测及其临床意义

目的估测扩张型心肌病肺动脉压并探讨其临床意义。方法用多普勒超声检查２６例扩张型心肌病患儿及２６名配对健康儿。肺动脉压用测三尖瓣最大返流速度及肺动脉血流加速时间（ＡＴ）两种方法估测。结果２６例患儿中２１例有三尖瓣返流，肺动脉收缩压（ＰＡＳＰ）３．２～１０．４ｋＰａ（６．０±１．９ｋＰａ）（２４～７５ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）。其中１８例（８６％）＞４ｋＰａ；对照组１４例有可测三尖瓣返流，ＰＡＳＰ均＜４ｋＰａ。患儿组ＡＴ缩短，ＡＴ与右室射血时间比值降低，也提示肺动脉压增高。ＰＡＳＰ与患儿左室短轴缩短分数、心功能低下持续时间及有无心内超声自发显影有关。随诊显示心功能好转者的ＰＡＳＰ较死亡或病情无改善者低，且后者肺动脉压继续增高。结论扩张型心肌病患儿多存在肺动脉高压，用多普勒超声估测的肺动脉压与患儿的病情及临床预后可能有一定的关系。     

一氧化氮合酶mRNA在缺氧性肺动脉高压大鼠肺动脉的表达

目的探讨一氧化氮体系在缺氧性肺动脉高压形成机制中的作用。方法采用地高辛精标记的一氧化氮合酶（ＮＯＳ）ｃＲＮＡ探针对缺氧组大鼠（６只）及对照组大鼠（７只）进行原位杂交。结果缺氧２周后的大鼠肺动脉收缩压（３．８±０．７ｋＰａ）（２８±５ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）、肺动脉平均压（２．８±０．６ｋＰａ）及肺动脉舒张压（１．４±０．４ｋＰａ）与对照组（２．９±０．５ｋＰａ，１．９±０．５ｋＰａ及０．９±０．５ｋＰａ）相比均显著升高。缺氧组大鼠肺动脉内皮细胞中ＮＯＳｍＲＮＡ表达信号为弱阳性（３只）及阴性（３只），平滑肌细胞中表达信号均为阴性；对照组大鼠肺动脉内皮细胞中ＮＯＳｍＲＮＡ表达信号为阳性（７只），平滑肌细胞中表达信号均为阴性。ＮＯＳｍＲＮＡ的表达强度与大鼠肺动脉收缩压、肺动脉平均压及肺动脉舒张压分别呈负相关（ｒｓ＝－０．６７３、－０．５９６及－０．６２１，Ｐ均＜０．０５）。结论缺氧时肺动脉内皮细胞ＮＯＳｍＲＮＡ表达的改变可能参与慢性缺氧性肺动脉高压的形成。     

胎粪吸入综合征及并发持续肺动脉高压的肺机制研究

目的 探讨胎粪吸入综合征（ＭＡＳ）及并发持续肺动脉高压（ＰＰＨＮ）患儿的肺功能变化。方法 应用美国Ｂｉｃｏｒｅ公司的ＣＰ－１００新生儿肺功能监护仪检测２０例ＭＡＳ患儿的肺功能,结果 ＭＡＳ患儿呼吸频率（ＲＲ）为（８３±１５）次／ｍｉｎ,气道阻力（ＡＲ）为（１０８±４６）ｃｍＨ２Ｏ（Ｌ·ｓ）。比对照组的（４５±６．０）次／ｍｉｎ（３８±１７）ｃｍＨ２Ｏ／（Ｌ·ｓ）明显增高（ｔ＝２．３０,２．５１,Ｐ     

一氧化氮吸入治疗小儿急性呼吸衰竭

为观察一氧化氮（ＮＯ）治疗小儿急性呼吸衰竭的疗效，应用我院自行研制的ＮＯ吸入装置，对１５例急性呼吸窘迫综合征（ＡＲＤＳ）和急性呼吸衰竭（简称急性呼衰）患儿进行ＮＯ吸入治疗。结果：７例有效，ＮＯ吸入前后比较氧分压与吸入氧浓度比值上升４．１±２．３ｋＰａ（３０．５±１７ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）（ｔ＝４．５２，Ｐ＜０．０５），氧合指数降低９±３（ｔ＝４．６３，Ｐ＜０．０５）。对２例肺动脉导管压力监测显示，肺动脉压和肺血管阻力明显下降，体动脉压和心率无显著性变化。结论：ＮＯ吸入疗法对部分急性呼衰患儿有效，宜在急性低氧性呼衰、心功能未受严重损害时应用。     

吸入一氧化氮逆转幼猪急性缺氧性肺动脉高压

目的 观察吸入不同浓度一氧化氮（ＮＯ）是否可逆转幼猪急性缺氧性肺动脉高压。方法 选用１０头上海种白猪。利用低氧建立急性缺氧性肺动脉高压模型。吸入不同浓度ＮＯ（０,１０,２０,４０,８０,１２０ｐｐｍ）。在各时相分别进行血液动力学指标、二氧化氮和高铁血红蛋白浓度监测。结果 吸入不同浓度ＮＯ可明显降低肺动脉平均压（ＭＰＡＰ）、肺循环阻力指数（ＰＶＲＩ）、跨肺压（ＴＰＧ）、体肺动脉压之比（Ｐｐ／Ｐｓ）、     

内皮素在左向右分流型先天性心脏病肺动脉高压中作用的研究

为探讨内皮素在左向右分流型先天性心脏病（简称先心病）肺动脉高压（简称肺高压）中的作用，采用放射免疫法测定５２例肺高压和１１例非肺高压先心病患儿腔静脉和肺动脉血浆内皮素（ｉｒＥＴ－１）水平，并对其中１０例肺高压患儿进行肺活检免疫细胞化学染色。结果，肺高压患儿肺动脉和腔静脉血浆ｉｒＥＴ－１水平均随肺动脉压力升高而增加，并高于非肺高压患儿（Ｆ＝２．９５；３．３９，Ｐ＜０．０５）。肺高压患儿血浆ｉｒＥＴ－１与肺动脉压力及阻力成正相关（ｒ＝０．６５；０．５８，Ｐ＜０．０１）。肺高压患儿肌性和弹性肺小动脉ｉｒＥＴ－１染色明显加深。研究提示，左向右分流型先心病患儿内皮素产生的增加可导致肺高压患儿肺血管的中膜肥厚和内膜增生，促进了肺高压的发展。     

超声测量肺动脉和主动脉血流收缩时间间期比值评价肺动脉和主动脉收缩压比值

为探讨超声测量肺动脉和主动脉收缩压比值（Ｐｐ／Ｐｓ）的方法，本研究运用脉冲多普勒（ＰＷＤ）技术测量肺动脉和主动脉血流收缩时间间期比值（ＲＳＴＩ／ＬＳＴＩ），与心导管测量的Ｐｐ／Ｐｓ相对照，现将结果报告如下。对象：３３例研究对象均系我院１９９１年１０月...     

小儿肺动脉高压的研究进展

小儿肺动脉高压的研究进展杜军保李树政当肺动脉收缩压（ｐｕｌｍｏｎａｒｙａｒｔｅｒｉａｌｓｙｓｔｏｌｉｃｐｒｅｓ－ｓｕｒｅ，ＰＡＳＰ）超过４．００ｋＰａ（３０ｍｍＨｇ，１ｋＰａ＝７．５ｍｍＨｇ）或肺动脉平均压（ｐｕｌｍｏｎａｒｙａｒｔｅｒｉａｌｍｅａｎ...     

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。     

Inhalation of nebulized nitroglycerin in dogs with experimental pulmonary hypertension induced by U46619

BACKGROUND: Pulmonary hypertension (PH) causes mortality in some congenital and acquired heart and lung diseases. However, inhalation of NO gas requires complicated and expensive instruments and elaborate preparations to avoid toxic gas administration. We tested the effectiveness and safety of inhaled nebulized nitroglycerin (Neb-NTG) in dogs with experimental PH. METHODS: Experimental PH was induced by continuous infusion of a thromboxane analog (U46619). The U46619 infusion rate was adjusted to maintain a systolic pulmonary artery pressure (PAP) at 40 mmHg in 10 anesthetized and mechanically ventilated dogs. Then, 20 micrograms/kg of NTG liquid nebulized by compressed air was inhaled. RESULTS: After infusion of U46619, the systolic, diastolic and mean PAP increased by 119%, 228% and 169%, respectively, and the systolic, diastolic and mean systemic arterial pressures (SAP) increased by 19%, 29% and 23%, respectively. The systolic pulmonary to systemic pressure ratio (Pp/Ps) and mean Pp/Ps increased by 83% and 113%, respectively, and the pulmonary vascular resistance (PVR), systemic vascular resistance (SVR) and pulmonary to systemic resistance ratio (Rp/Rs) increased by 341%, 100% and 145%, respectively. After inhalation of Neb-NTG in dogs with experimental PH, systolic, diastolic and mean PAP and PVR decreased by 25 +/- 4, 26 +/- 11, 25 +/- 9 and 31 +/- 21%, respectively. There were no significant changes in systolic, diastolic and mean SAP, SVR, cardiac output and plasma methemoglobin concentrations. The systolic and mean Pp/Ps decreased by 18 +/- 7 and 20 +/- 7%, respectively. The Rp/Rs decreased by 25 +/- 13%. CONCLUSIONS: The results of this study demonstrate that Neb-NTG is an effective and selective pulmonary vasodilator and may offer a new therapeutic option for PH.     

经右室穿刺肺动脉瓣球囊扩张治疗室间隔完整型肺动脉闭锁

目的 评价经右心室穿刺肺动脉瓣球囊扩张杂交手术治疗室间隔完整型肺动脉闭锁的早期治疗结果.方法 6例室间隔完整型肺动脉闭锁患儿接受右心室穿刺肺动脉瓣球囊扩张杂交手术.术前平均经皮氧饱和度(71±10)%.结果 全组无死亡及围术期并发症发生.术后经皮氧饱和度为(89±5)%;右室-肺动脉压差为(19±11)mm Hg.随访(4±3.6)个月,跨肺动脉瓣压差(45±42)mm Hg,1例术后再次行经导管肺动脉瓣球囊扩张.结论 对于右心室发育良好的室间隔完整型肺动脉闭锁,本术式早期疗效满意,安全性较高,术后肺动脉瓣反流少,可以促进右心室的发育.

Abstract：

Objective To evaluate the early outcomes of balloon valvuloplasty via right ventricle pulmonary valve for the treatment of pulmonary atresia with intact ventricular septum in children.Methods Methods Between January 2010 and December 2010, 6 patients were diagnosed with pulmonary atresia with intact ventricular septum, and underwent balloon valvuloplasty via right ventricle pulmonary valve at this center. Of the 6 patients, the mean body weight was 3. 8 ± 1.6 kg, the mean transcutaneous oxygen saturation was 71 ± 10%, and the mean age was 56 ± 45 days. All of the 6 patients had tripartite right ventricles. Their tricuspid valve z-scores ranged from -2 to 2. 2 (mean zscore: 0. 4 ± 1. 5). Results During operation, the mean size of the balloons used was 7. 3 ± 1. 2 mm,the ratio of the balloon size to pulmonary valve ring was 1.0 ± 0. 1, the pressure gradient between right ventricle and main pulmonary artery was 19 ± 11 mmHg, the ratio of forward flow from right ventricle to that from ductus arteriosus was 0. 35 ± 0. 06. After surgery, the mean ventilation time was 3. 2 ± 1.0 days, and mean ICU stay was 16 ± 5 days. The post-operative transcutaneous oxygen saturation was 89 ± 5%. The patients were followed up for 4 ± 3. 6 months. No death and post-operative complications were noted. One patient underwent interventional balloon valvuloplasty 2 weeks after the first surgery. The transcutaneous oxygen saturation on last follow-up was 85 ± 6%, which was significantly improved after surgery (t = -2. 66, p = 0. 045). The postoperative pressure gradient between right ventricle and main pulmonary artery was 45 ± 42 mmHg. Four of the 6 patients had pulmonary regurgitation, 3 of whom were mild and 1 was moderate. Tricuspid valve z-scores (mean, 0. 5± 1) also improved compared with that before procedure (t = -3. 36, p = 0. 02). Conclusions Balloon valvuloplasty via right ventricle pulmonary valve is safe and effective to treat the pulmonary atresia with intact ventricle septum in children.     

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.     

Paediatric pulmonary vascular disease

Pulmonary vascular disease comprises any congenital or acquired pathology of the intrinsic pulmonary vessels with the unique feature of pulmonary arteries carrying unsaturated blood and pulmonary veins carrying oxygenated blood. Pulmonary hypertension (PH) ensues when pulmonary vascular disease affects at least 50% of the capillary resistance vessels, i.e. pulmonary pre-acinar and intra-acinar arteries (so-called pre-capillary PH), or when pressure in the pulmonary venous system distal to the capillaries rises above a mean of 15 mmHg (so-called post-capillary PH). PH is defined by a mean pulmonary arterial pressure above 25 mmHg at rest. Vasoconstriction, remodelling and thrombosis of small pulmonary arteries lead to an increase in pulmonary vascular resistance. The consequence is failure of the afterload-intolerant right ventricle. In this review, pulmonary vascular disease in children will be addressed according to the 2003 World Health Organisation (Venice) classification of PH.     

儿童先天性心脏病血清Apelin水平与肺动脉压关系的初步探讨

目的 初步探讨先天性心脏病患儿血清Apelin 水平与肺动脉压的关系.方法 手术治疗的先心病患儿126 例,检测患儿术前及术后第7 天的血清Apelin 水平.建立体外循环前检测并计算肺动脉收缩压/体循环收缩压(Pp/Ps)的比值,依据Pp/Ps 分组:无肺动脉高组压(PAH)组、轻度PAH 组、中度PAH 组和重度PAH 组;术后第7 天超声心动图估测肺动脉平均压(PAMP).结果 无PAH,以及轻、中、重度PAH 各组术前及术后的血清Apelin 水平依次降低,差异有统计学意义(PPr=-0.51,-0.54,P结论 先心病患儿并发肺动脉高压及其发展与血清Apelin 水平降低有关系,血清Apelin 对诊断先心病患儿是否并发肺动脉高压及其程度的意义值得深入研究.     

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。     

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.     

肺动脉环缩术在儿童复杂先天性心脏病中的应用

目的 探讨肺动脉环缩术在儿童复杂先天性心脏病中的应用经验,并对手术效果进行评价.方法 回顾性分析2002年1月～2007年3月进行的32例肺动脉环缩术.男22例,女10例,手术年龄平均9.2个月,体重平均6.1 kg.肺动脉环缩后肺动脉周径平均(28.0±4.1)mm,术后估测环缩近远端压差(43.7±9.0)mmHg.结果 围术期死亡1例,2例患儿再次调整环缩.4例患儿出现围术期并发症.31例患儿随访1个月～5年,22例患儿进行Ⅱ期手术.结论 肺动脉环缩术可限制肺血过多,锻炼左心室,为Ⅱ期施行双心室矫治及双向腔肺分流术赢得时机.     

先天性心脏病肺动脉高压手术前后右心功能变化和治疗

目的 了解先天性心脏病左向右分流导致的重度肺动脉高压对右心功能的影响以及手术矫治后右心功能的恢复状态。方法 对室间隔缺损(室缺)合并重度肺动脉高压(全肺循环阻力增加)的40例患儿，用心导管的方法进行术前、术后5～7年右心功能、肺循环的血流动力学随访测定。结果 术前右心心搏指数、作功指数、心排指数显著高于术后；术前右房压、右室收缩压及舒张压、肺动脉压力和阻力均增高，缺损修补后右室舒张压恢复正常，收缩压的降低与肺动脉压力下降有关；大型室缺左向右分流重度肺动脉高压右心功能不全系继发性右室高排出量心力衰竭，且伴有舒张功能障碍。结论 治疗心力衰竭不宜首选正性心肌收缩药物；降低肺动脉压力、减少左向右分流、根治心内畸形是合理的选择；术后右室收缩压持续不能恢复至正常水平，提示继发性肺血管梗阻性病变存在。