心血管系统疾病

05 1 62 8　先天性心脏病肺动脉高压手术前后右心功能变化和治疗 /朱卫华…∥临床儿科杂志 .-2 0 0 4,2 2 (3 ) .-1 43～ 1 46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…     

单纯性室间隔缺损术后死亡的高危因素

手术治疗４４３例单纯性室间隔缺损（ＶＳＤ），死亡２２例。引起死亡的危险因素包括１．年龄＜８岁，体重＜１１ｋｇ；２．术前并发心力衰竭，３．心电图示右室肥大伴心肌损害，４．胸片示心胸比率重度增大。５．ＶＳＤ＞１．５ｃｍ。６．肺动脉平均压＞５．０ｋＰａ，肺总动脉阻力＞５００达因·秒·ｃｍ－５；７．左→右分流量占肺循环量６０％以上，肺循环与体循环流量比值＞３：１。     

前列腺素E1在婴儿室间隔缺损并肺高压的应用

目的　探讨术前应用前列腺素E1(PGE1)对室间隔缺损并肺高压婴儿 ,增强心功能 ,降低肺动脉及肺小动脉阻力 ,提高手术耐受性的意义。方法　本组 18例 ,男 13例 ,女 5例。年龄 5个月～ 6岁平均 (2 9.6 7± 2 3.14 )个月。术前经右心导管测量右房、肺动脉、肺小动脉压力及相应部位的血氧含量 ,依Fick’s公式分别计算出体循环、肺循环血流量及分流量 ,肺小动脉阻力、肺总阻力。选取Pp/Ps>0 .4 5者为研究对象。术前给予静滴PGE114d ,观察患儿用药后气急、肺部叮罗音的改变。对比根治手术中所测量肺动脉及肺小动脉压力 ,再次计算出上述各项数据。结果　用药后肺动脉压、肺小动脉压、Pp/Ps、全肺阻力、肺小动脉阻力等均有明显下降 ;在心内分流量及体循环压力基本不变情况下 ,左、右心排量明显增加。全组病例术后均治愈。随访 2年患儿生长发育良好。结论　PGE1是一种强有力的血管扩张剂 ,能直接扩张肺动脉 ,降低肺动脉、肺小动脉压力和肺循环阻力 ,提高左右心输出量 ,改善心功能。术前用药可改善患儿心肺功能 ,增加对体外循环及手术的耐受性 ,渡过术后肺动脉高压危险期及提高治愈率     

常见左向右分流先天性心脏病的治疗及时机选择

先天性心脏病(简称先心病)按血流动力学即血流方向分为三大类:左向右分流型;右向左分流型;无分流型.左向右分流型先心病系指血流由体循环通过异常交通流向肺循环;常见的左向右分流型先心病有动脉导管未闭(PDA)、继发孔房间隔缺损(ASD)、室间隔缺损(VSD),除此以外还有主肺动脉间隔缺损、冠状动脉瘘、左室右房通道等.     

选择性心电图R.S振幅对室间隔缺损患儿肺动脉压力的估计

室间隔缺损(VSD)在小儿先心病中发病率最高,其左至右分流引起的肺动脉高压约占50％,肺高压导致的右室肥大可在心电图上表现出来。本文选择反映右室大小的心电图指标对比心导管测得的右心压力,以探讨这种间接估测肺动脉压力的价值。     

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；     

先心病血浆心钠素,环核苷酸与肺血流动力参数关系及术后变化

目的研究先心病患者外用静脉血心钠素、环核苷酸与肺血流动力参数关系及外科矫治术后的变化。方法经临床确诊的３２例先心病人，用放射免疫法同步测定血中ＡＮＰ及环核苷酸浓度。术前对２１例患儿行右心导管检查并监测其血流动力参数。结果左向右分流型先心病人，血浆ＡＮＰ、ｃＧＭＰ明显高于对照组，二者呈正相关；血浆ＡＮＰ、ｃ－ＧＭＰ与肺动脉压相关显著，而与右房、右室压无相关性。术后７～１８天血中ＡＮＰ、ｃＧＭＰ含量无明显下降。结论左向右分流的先心病人，心房扩张，血浆ＡＮＰ值升高；血浆ｃＧＭＰ浓度随ＡＮＰ的不同而变化，提示ｃＧＭＰ是ＡＮＰ的第二信使；手术前后血中ＡＮＰ、ｃＧＭＰ水平无明显差异，可能与肺动脉压及肺血管阻力尚未降至正常有关。     

大型室间隔缺损合并肺动脉高压术后远期疗效的随访

先天性心脏病 (简称先心病 )左向右分流合并重度肺动脉高压术后可复性问题一直是临床关注的焦点。手术矫正心内畸形后 ,如肺动脉高压不能恢复 ,甚至加重则失去了外科治疗的意义。有关手术的疗效和手术适应证的选择 ,国内外均有些报道 ,但缺乏肺动脉高压血流动力学长期随访的资料。我们通过对 2 4例大型室间隔缺损合并重度肺动脉高压(全肺阻力增高 )患儿进行术前及术后长期血流动力学的随访 ,旨在了解手术对这些患儿肺动脉高压的远期疗效。对象本组患儿 2 4例 ,男 11例 ,女 13例 ;年龄 6个月～ 14岁 ,平均 (5 8± 4 7)岁。≤ 2岁者 11例 ,>2…     

左向右分流致肺动脉高压对大鼠肺动脉胶原代谢的影响

目的建立左向右分流所致肺动脉高压大鼠模型,了解高肺血流量对肺血管胶原代谢的影响。方法对大鼠行腹主动脉下腔静脉分流术。术后11周以右心导管法测定肺动脉平均压(PAMP),采用免疫组织化学法检测大鼠肺动脉Ⅰ、Ⅲ型胶原蛋白的表达。结果分流11周后大鼠肺循环与体循环血流量之比分别为3.3∶1.0,为大量左向右分流。PAMP较对照组明显升高(P<0.01)。分流组大鼠肺中、小型肺动脉中Ⅰ、Ⅲ型胶原蛋白表达与对照组比较明显增加。结论高肺血流量可导致肺动脉高压,并促进肺动脉高压大鼠细胞外基质/胶原的堆积。     

新生儿持续肺动脉高压24例诊断和治疗

新生儿持续肺动脉高压是由多种病因致生后肺循环阻力不下降,肺动脉压力升高,而出现动脉导管及卵圆孔的右向左分流,是导致新生儿死亡的重要原因之一.     

Bidirectional shunt flow across a ventricular septal defect: Pulsed doppler echocardiographic analysis

Pulsed Doppler echocardiographic and hemodynamic examinations were performed in 31 patients (mean age 17.8 years) with isolated ventricular septal defect (VSD). Three groups were studied: group I (n=6) patients had severe pulmonary vascular obstructive disease (PVOD); group II (n=12) patients had pulmonary hypertension (PH) without severe PVOD; group III (n=13) patients had no PH. Bidirectional shunting was detected in 9 VSD patients (6 in group I and 3 in group II). Patients with low to moderately elevated right ventricular pressures demonstrated left-to-right shunting across the defect throughout the cardiac cycle. When systolic pressure in the right ventricle reached approximately 60% of the left ventricular pressure, right-to-left shunting occurred across the defect during early and mid diastole. However, in patients with Eisenmenger syndrome (group I) the right-to-left shunting occurred during late systole with continuation during the early and mid diastolic period. The earlier occurrence of right-to-left shunting (index <0.5 second) signifies the presence of severe PVOD.     

Right ventricular performance and pulmonary haemodynamics in adolescent and adult patients with cystic fibrosis

A combined haemodynamic and radionuclide approach was used to evaluate right ventricular performance in 16 adolescent and adult patients with cystic fibrosis (CF). There were nine patients with mild arterial hypoxaemia (PaO₂>80% of predicted) and normal resting pulmonary artery pressure and seven patients with severe arterial hypoxaemia (PaO₂<70% of predicted) and resting pulmonary arterial hypertension (PH). The right ventricular ejection fraction (RVEF) by equilibrium angiocardiography using krypton 81^m as a tracer and stroke volume index (SVI) by thermodilution techniques were measured simultaneously and right ventricular end-diastolic and end-systolic volumes were derived. RVEF was normal in CF patients without PH (58.9±7.2%) but was reduced in those with PH (45.4±2.6%). There was a statistically significant inverse linear correlation between RVEF and afterload as assessed by mean pulmonary artery pressure and pulmonary vascular resistance (PVR: r=–0.78), indicating that RVEF ist afterload-dependent. Right ventricular function, however, as assessed by right ventricular end-systolic pressure-volume relations was even higher in CF patients with PH, indicating preserved or even increased right ventricular function in the face of an increased afterload stress.Abbreviations CF cystic fibrosis - PaO₂ arterial oxygen tension - PH pulmonary arterial hypertension - RVEF right ventricular ejection fraction - SVI stroke volume index - Pap pulmonary artery pressure - PVR pulmonary vascular resistance - COPD chronic obstructive pulmonary disease - VC vital capacity - FEV₁ forced expiratory volume in 1 s - RV resisdual volume - TLC total lung capicity - RVEDP right ventricular end-diastolic pressure - Pap_s systolic pulmonary artery pressure - Pap_d diastolic pulmonary artery pressure - PCWP pulmonary capillary wedge pressure - CO cardiac output - ED end-diastolic background-corrected counts - ES end-systolic background-corrected counts - CI cardiac index - RVEDVI right ventricular end-diastolic volume index - RVESVI right ventricular end-systolic volume index - P/V relation right ventricular end-systolic pressure/right ventricular end-systolic volume index - S-K score Shwachmann-Kulczycki score     

Changes in Pulmonary Venous Flow Patterns in Patients with Ventricular Septal Defect

We studied pulmonary venous (PV) flow patterns using Doppler echocardiography in 26 patients with ventricular septal defect less than 3 years of age. Fifteen patients had moderate or severe symptoms, and the remaining 11 had no significant symptoms. Peak velocity of PV diastolic flow and flow velocity integral of PV diastolic flow in the symptomatic patients were significantly larger than those in either asymptomatic patients or the normal controls. The ratio of PV diastolic flow velocity to PV systolic flow velocity and the ratio of flow velocity integral of PV diastolic flow to that of PV systolic flow in the symptomatic patients were significantly larger than those in either asymptomatic patients or the normal controls. The ratio of PV diastolic flow velocity to PV systolic flow velocity as well as the ratio of flow velocity integral of PV diastolic flow to that of PV systolic flow correlated with V wave in left atrial or pulmonary capillary wedge pressure and indexes of left ventricular mass and left atrial volume. We conclude that the abnormal pulmonary venous flow patterns in ventricular septal defect might be associated with large left-to-right shunting and left atrial pressure V wave.     

Ventricle works as a converting organ of atrial blood flow: Physiological significance of mean ventricular pressure

The roles and characteristics of the ventricle were examined using mean ventricular pressure (MVP) in ventricular-vascular assocation. One hundred and two patients with congenital heart diseases who had undergone cardiac catheterization were studied. They were divided into five groups: Group 1, atrial septal defect without pulmonary hypertension (PH); Group 2, ventricular septal defect (VSD) without PH; Group 3, VSD with PH; Group 4, pulmonary valvular stenosis; and Group 5 as a control group. Then, we examined the relationships between mean pulmonary artery pressure (MPAP) and mean right ventricular pressure (MRVP), and also between mean systemic arterial pressure (MSAP) and mean left ventricular pressure (MLVP) among the five groups. Furthermore, we defined new indicators to express the ease of blood flow through each ventricle. They were referred to as a conductance of the right ventricle (CD_R) and a conductance of the left ventricle (CD_L), respectively. Then they were compared among the five groups. The values of MPAP/MRVP and MSAP/MLVP were kept constant to be about 1.3 and 1.7, respectively. Furthermore, CD_R was different betweeen each group according to the property of the pulmonary vascular bed, whereas CD_L took almost the same value among the five groups. The ventricle works as a converter of atrial blood flow so that it can achieve efficient blood transport.     

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目的探讨组织多普勒(TDI)、二维斑点追踪技术(STI)和血清脑利钠肽(BNP)评估室间隔缺损(VSD)合并不同程度肺动脉高压(PH)患儿右心室功能的价值。方法 2008年6月至2010年6月在苏州大学附属儿童医院住院的VSD合并PH患儿68例,根据三尖瓣反流压差测得肺动脉压力程度分为轻度组(23例)、中度组(21例)、重度组(24例)。同时选择24名健康体检者作为正常对照组。采用GEVIVID7仪器测量右室长轴整体收缩期应变(GLS)和应变率(GLSR),三尖瓣环脉冲组织多普勒图测量三尖瓣环收缩期峰值速度(Sa)、等容收缩加速度(IVA)、Tei指数,用M型超声测量三尖瓣环收缩期位移(TAPSE)。采用ELIASA法测血清脑钠钛(BNP)浓度。结果 (1)IVA和TAPSE随着PAH升高而降低,而Tei指数随着PAH增加而增大,3个指标除正常对照组和轻度组间差异无统计学意义外(P>0.05),其他各组间差异均有统计学意义(P<0.05)。Sa在中、重度组间差异无统计学意义(P>0.05),在正常对照、轻度组间差异无统计学意义(P>0.05),其余组间有统计学意义(P<0.05)。(2)右室GLS、GLSR随着PAH升高而减小,GLS在4组间两两比较差异有统计学意义(P<0.05);GLSR中度和重度组与正常对照组和轻度组差异有统计学意义(P<0.05),中重度间及正常对照组和轻度间差异无统计学意义(P>0.05)。(3)血清BNP浓度在VSD组中随着PAH程度的加重而升高,在4组间两两比较差异有统计学意义(P<0.05)。(4)BNP与Tei指数及心功能分级相关性最好。结论 TDI和STI各个参数均能反映VSD合并PH患儿右心室功能,其中BNP和Tei指数与心功能相关性最好,而BNP和GLS可以反映轻度PAH时右室功能受损。     

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??Abstract??Objective??To investigate the level of serum catecholamine ??CA????including epinephrine??E???? norepinephrine??NE???? dopamine??DA???? and aldosterone??ALD?? in children with left to right shunt congenital heart disease??CHD????and analyze the correlation between the serum CA?? ALD and cardiac function??ventricular volume load?? pulmonary artery pressure and myocardial remodeling. Method??Totally 53 cases of left to right shunt CHD and 20 cases of control group were induded in the study. We measured the content of serum CA and ALD in the control group and CHD group. We applied real-time three- dimensional echocardiogram to measure the left atrium diameter ??LAD???? left ventricular end diastolic inner diameter ??LVEDD???? and cardiac function. Result????1??The level of serum NE and ALD in CHD group was higher than the control group ??P < 0.01??. ??2??The level of serum CA and ALD was higher in heart failure group?? large shunt group and pulmonary artery hypertension group than in non-heart failure group?? small shunt group and non-pulmonary artery hypertension group??P < 0.01??.??3??The left ventricular end diastolic inner diameter index??LVEDDI???? left ventricular end diastolic volume index??LVEDVI?? and left ventricular mass index of heart failure group were larger than those of non-heart failure group??P < 0.01??.??4??There was positive correlation between the serum level of CA and left atrial diameter index ??P < 0.01?? and LVEDDI ??P < 0.01???? LVEDVI ??P < 0.01??. Conclusion??The serum CA and ALD may be regarded as an objective parameter in the early diagnosis of heart failure and pulmonary artery hypertension. There is a positive correlation between the serum CA and myocardial remodeling.     

Pulmonary artery pressure evaluated by pulsed Doppler echocardiography in children with a left-to right intracardiac shunt

Summary In a study of 25 children with left-to-right intracardiac shunt we found a good correlation (r>0.92;p<0.001) between right ventricular pre-ejection period/acceleration time, derived from pulsed Doppler echocardiography, and pulmonary artery systolic, diastolic, and mean pressures, measured at cardiac catheterization. This may enhance the noninvasive estimation of pulmonary artery pressure.     

Dependence of canine right coronary arterial flow upon heart rate and right ventricular pressure

There is a paucity of knowledge regarding right coronary pulsatile hemodynamics when the right ventricle is under hemodynamic overload as is often the case in pediatric patients with congenital cardiac anomalies. To elucidate the exact mechanisms for the right coronary artery (RCA) to cope with the overload, we studied nine open-chest adult Beagles and analyzed the flow signals of the RCA in relation to independently varied heart rate (pacing) and right ventricular pressure (pulmonary artery banding). Both increased heart rate and right ventricular pressure increased the total volume flow of the RCA. The diastolic over total flow ratio (D/T), however, enlarged on increasing right ventricular pressures while it declined on increasing heart rates. Our data confirmed, as well, that increased flow of RCA on rising heart rate was provided mainly by an increase in systolic phase, while the increase on augmented right ventricular pressure was provided by the increase in diastolic phase. The RCA manages to deliver blood to the right ventricular musculature in two different ways in response to increasing heart rate and right ventricular pressure.     

Regression of Pulmonary Vascular Disease After Therapy of Abernethy Malformation in Visceral Heterotaxy

A 1-year-old boy who had left isomerism and corrected transposition of the great arteries (c-TGA) with moderate-sized ventricular septal defect, severe pulmonary artery hypertension (PAH), and pulmonary vascular disease with significant right-to-left shunting received a diagnosis of type 2 Abernethy malformation, which was partly responsible for disproportionate PAH in the child. The malformation was treated by plugging of the portosystemic shunt. Follow-up cardiac catheterization on sildenafil demonstrated significant left-to-right shunting (2.16:1) and a fall in pulmonary vascular resistance, making surgical correction possible. This case highlights the importance of searching for additional rare causes of PAH in patients with congenital heart diseases when the degree of pulmonary hypertension is disproportional to the defect size.     

高海拔地区重症肺炎婴幼儿心脏功能检测的意义

目的探讨高海拔地区重症肺炎婴幼儿左右心室收缩及舒张功能变化的特点。方法应用彩色多普勒超声诊断仪测量(海拔2260 m)2000年1月~2005年1月本院住院30例(男16例,女14例)西宁地区婴幼儿重症肺炎和32例(男17例,女15例)健康婴幼儿左右心室收缩及舒张功能参数,并进行比较分析。结果病例组右室射血前期/射血加速时间(RPEP/AT)明显延长,右心室射血分数(RVEF)、肺动脉最大血流速度(Vpa)降低,右心室(RV)、右心房(RA)扩大。二、三尖瓣A峰、A/E增高,左室等容舒张时间(LIRT)、右室等容舒张时间(RIRT)明显延长(Pa<0.01)。结论高海拔地区婴幼儿重症肺炎存在肺动脉高压,及明显右心室收缩功能及左右心室舒张功能异常,左心室收缩功能无明显异常。