先心病重度肺高压术后肺动脉压改变与术时年龄关系

目前 ,左向右分流先天性心脏病 (先心病 )伴重度肺高压的治疗仍是临床难题 ,对产生不可逆性肺血管病变和手术的时间认识不一 ,致使一些病儿殆误手术时机。为此 ,我们于1995年 10月至 2 0 0 0年 3月 ,连续观察了 32例左向右分流先心病伴重度肺高压 [肺动脉 /体动脉收缩压比值 (Pp/Ps)≥0 75 ]病儿术前和术后肺动脉压的变化 ,并分析其与术时年龄的关系 ,以便为此类病儿的适宜治疗年龄提供依据。资料和方法　左向右分流、经心导管检查确诊重度肺高压并经手术治疗的先心病病儿 ,男 17例 ,女 15例。年龄 6月龄～ 12岁 ,平均 (2 92± 2 5 5 )…     

小儿先心病重度肺高压的核素肺灌注显像与肺活检对比研究

目的　对比放射性核素肺灌注显像 (PPI)与肺活检资料 ,探讨在合并重度肺动脉高压 (PH)的左向右分流先天性心脏病病儿中的相关性。方法　 43例心脏畸形矫治术前行PPI检查 5 2例次 ,肺活检 2 6例 ,肺活检标本以显微图像分析仪作定量分析。经统计学处理 ,分析各参数间的相关性。结果　右肺上、下肺野计数比 (RULR)≥ 0 .70组较 <0 70组肺小动脉平均中膜厚度百分比 ( %MT) (P <0 0 1)、肺小动脉平均中膜面积百分比 ( %MS) (P <0 0 5 )显著性增高。RULR与 %MS正相关 ,回归方程为 %MS =3 819RULR +49 82 0 (P <0 .0 5 ) ,相关系数 0 44。双肺核素总计数 (LRC)与每平方厘米肺小动脉计数 (APSC)正相关 ,回归方程APSC =0 0 0 1317LRC - 342 3148(P <0 0 1) ,相关系数 0 5 7。结论　PPI参数与肺小动脉病变程度相关 ,在左向右分流先天性心脏病合并肺动脉高压病儿的手术前评价、手术适应证选择及术后随访都有临床应用价值 ,尤为适用于幼儿病例。     

先天性心脏病重度肺高压术后肺动脉压监测的临床意义

为了认识左向右分流先天性心脏病（先心病）重度肺动脉高压（肺高压）患儿术后肺动脉压的变化规律,提供临床分析处理的依据,我们1995年10月～1999年12月连续观察25例左向右分流先心病重度肺高压病儿和12例中度肺高压病儿术后肺动脉压的变化,现报告如下。     

先心病肺高压病儿血浆内皮素与肺组织I、III型胶原含量的相关研究

目的　探讨先天性心脏病 (先心病 )肺动脉高压病儿血浆内皮素 1(ET 1)和胶原增生的相互关系及其在先心病肺动脉高压形成中的作用。方法　 2 0例左向右分流型先心病病儿分为肺动脉高压组(PH组 )和无肺动脉高压组 (对照组 ) ,用放射免疫学方法测定两组病儿血浆ET 1水平 ,用SDS 间断聚丙烯酰胺凝胶电泳 (SDS PAGE)测定肺组织I、III型胶原含量。结果　与对照组相比 ,PH组血浆ET 1水平和肺组织I、III型胶原含量明显增加 (P <0 0 1)。PH组血浆ET 1水平与肺组织I、III型胶原含量呈显著正相关。结论　先心病肺动脉高压的形成可能与ET 1水平升高 ,诱导肺组织I、III型胶原增生有关。     

先心病肺高压病儿血浆内皮素与肺组织Ⅰ、Ⅲ型胶原含量的相关研究

目的探讨先天性心脏病(先心病)肺动脉高压病儿血浆内皮素-1(ET-1)和胶原增生的相互关系及其在先心病肺动脉高压形成中的作用.方法 20例左向右分流型先心病病儿分为肺动脉高压组(PH组)和无肺动脉高压组(对照组),用放射免疫学方法测定两组病儿血浆ET-1水平,用SDS-间断聚丙烯酰胺凝胶电泳(SDS-PAGE)测定肺组织I、III型胶原含量.结果与对照组相比,PH组血浆ET-1水平和肺组织I、III型胶原含量明显增加(P＜0.01).PH组血浆ET-1水平与肺组织I、III型胶原含量呈显著正相关.结论先心病肺动脉高压的形成可能与ET-1水平升高,诱导肺组织I、III型胶原增生有关.     

先心病肺高压小儿体外循环转流及分流修复前后呼吸功能的变化

观察４０例左向右分流先心病小儿（肺高压组２０例，对照组２０例）体外循环转流前后呼吸功能的变化。结果：转流前，肺高压组呼吸阻力（ＲＲ）（吸气阻力ＲＩ２３．８７±７．５３，呼气阻力ＲＥ４２．９８±１２．１）明显高于对照组（ＲＩ１７．９５±７．５３，ＲＥ３４．９５±５．１３），肺胸顺应性（ＣＬＴ）１１．５８±４．８７明显低于对照组１４．３２±１．９９。转流后，肺高压组ＲＲ（ＲＩ３０．９４±１１．９９，Ｒ     

前列腺素E_1在先心病重度肺动脉高压中的应用

对３２例先天性心脏病合并重度肺动脉高压病人术前行前列腺素Ｅ１（ＰＧＥ１）治疗后，根据用药前后其肺动脉平均压、周围动脉血氧分压、血氧饱和度及心率、血压的变化，并结合肺活检指标进行对比研究。结果表明，ＰＧＥ１不但明显改善部分先心病重度肺动脉高压病人的血流动力学指标，而且可使部分病例Ｈｅａｔｈ分级ＩＩ级或ＩＩ级以下的肺血管病变结构趋向好转；ＰＧＥ１不仅有治疗作用，并对手术适应证有重要的参考价值。先天性心脏病合并肺动脉高压，术前应用ＰＧＥ１治疗，可提高手术安全性。     

室间隔缺损伴肺动脉高压患者血浆内皮素、一氧化氮与肺细小动脉形态变化的关系

目的　探讨内皮素 ( ET- 1)、一氧化氮 ( NO)在肺动脉高压 ( PH)发生、发展过程中的作用。　方法　 60例室间隔缺损患者分为两组 ,无 PH组 :2 0例 ,不合并 PH,平均肺动脉压 ( MPAP) <2 0 mm Hg( 1k Pa=7.5 mm Hg) ;PH组 :4 0例 ,合并 PH,MPAP>2 0 mm Hg。测定两组血浆 ET- 1和 NO含量 ,作肺组织活检 ,观察肺血管的病理改变及测定肺细小动脉显微形态指标。　结果　全部肺标本按阮英茆的 级分类进行病理分级 :无 PH组患者肺血管无明显病理改变 ,PH组患者 级 7例、 级 13例、 级 2 0例 ,未见 级病理改变。随着肺血管病理改变分级的增加 ,血浆ET- 1、NO、血流动力学指标及肺细小动脉显微形态指标测定均呈阶梯样变化 ,除 NO、血管腔面积 /血管总面积 ( EA/ TA)逐渐减小 ,其它指标均逐渐增高 ,ET- 1、NO、MPAP、肺血管阻力 ( PVR)、肺细小动脉中膜厚度 ( m MTPA)、血管壁面积 /血管总面积 ( WA/TA)、EA/ TA和肌型肺细小动脉 /微动脉总数的比值 ( TWPV) ,无 PH组与 PH组 级、PH组 级与 级、 级与 级之间差别均有显著性意义 ( P<0 .0 5 ,0 .0 1) ;血浆 ET- 1与 MPAP、PVR、m MTPA、WA/ TA和 TWPV均呈显著正相关 ,血浆 NO均呈显著负相关 ;血浆 ET- 1与 EA/ TA呈显著负相关 ,血浆 NO呈显著正相关 ( P<0 .0     

前列腺素E1对血管内皮生长因子含量的影响及其与肺小动脉病理分级的关系

目的 研究应用前列腺素E1(PGE1)对先天性心脏病合并肺动脉高压(PH)患者血清血管内皮生长因子(VEGF)含量的影响及其与肺小动脉病理分级的关系. 方法 2002年1月至2003年6月共收治先心病合并PH患者53例(MPAP＞20 mmHg),其中有明显紫绀6例(MPAP＞90 mmHg).入院后静脉滴注PGE1 10～15ng/kg*min,每天用药12小时,连续15天.分别于用药0、5、10和15天晨抽取静脉血,采用酶联免疫吸附法(ELISA)测定血清VEGF含量.做肺组织活检,按Heath和Edwards分类进行病理分级,观察肺小动脉的病理改变. 结果病理分级:Ⅰ级9例,Ⅱ级14例,Ⅲ级19例,Ⅳ级5例,≥Ⅴ级 (紫绀者)6例.应用PGE1前,肺小动脉病理分级Ⅲ级时血清VEGF达到峰值,Ⅳ级、特别是≥Ⅴ级后有明显下降的趋势;应用PGE1后,Ⅰ级血清VEGF与用药前比较差别无显著性意义(P>0.05),Ⅱ、Ⅲ级者血清VEGF明显降低(P<0.01),Ⅳ级者有轻度的降低(P<0.05),而≥Ⅴ级患者无明显变化(P>0.05). 结论 PGE1可降低血清VEGF含量, 其降低幅度与肺小动脉病理改变程度有较密切的关系,可作为术前判断肺小动脉病理分级的一个参考指标.     

分泌型蛋白质GREM1在体-肺分流性肺动脉高压中的作用

目的探讨骨形态发生蛋白(BMP)拮抗因子GREM1作为先天性心脏病相关肺动脉高压(CHD/PAH)患者BMP信号通路活性下调机制的可能。方法外科手术建立大鼠体-肺分流性PAH模型。术后12周,右心导管及心脏超声测量右心系统血流动力学指标及形态学指标,心、肺、血标本取材。计算右心肥厚指数,评估肺血管重构情况,检测肺组织BMP信号通路相关蛋白及GREM1的表达变化及血浆GREM1浓度。体外培养肺动脉内皮细胞(PAECs),研究GREM1对PAECs增殖及凋亡的影响。结果体-肺分流大鼠模型成功再现了CHD/PAH的PAH状态。在分流性肺组织中,缺氧诱导因子-1α(HIF-1α)表达水平无改变(P>0.05),GREM1表达增加,但BMP信号通路组份的表达下降(P<0.05),而矫正体-肺分流可逆转这一趋势(P<0.05)。免疫组织化学染色显示重构的肺小动脉中GREM1表达增加(P<0.05)。体外细胞实验显示,增殖的PAECs中BMP信号通路相关蛋白下调而GREM1表达和分泌显著升高(P<0.05),且GREM1通过拮抗BMP信号通路显著促进PAECs的增殖并抑制其凋亡(P<0.05)。此外,体-肺分流大鼠的血浆GREM1显著升高且与肺血流动力学参数密切相关(P<0.05)。结论体-肺分流引起肺组织GREM1表达升高,而升高的GREM1通过下调BMP信号通路活性诱导肺血管重构,促进肺动脉高压的形成。此研究结果为CHD/PAH肺组织BMP信号通路活性下调机制提供了一种新的解释。     

Usefulness of nitric oxide treatment for pulmonary hypertensive infants during cardiac anesthesia

BACKGROUND: The beneficial effect of inhaled nitric oxide (NO) on pulmonary hypertension is well known. However, the indications for NO inhalation therapy for pulmonary hypertension associated with congenital heart lesions are still unclear. The aim of the current study was to seek a measure that would predict the effectiveness of inhaled NO in infants undergoing cardiac surgery. METHODS: Forty-six infants with pulmonary hypertension were studied. Pulmonary vascular resistance (PVR) measured at the time of cardiac catheterization was used as an indicator and compared with pulmonary arterial pressure/systemic blood pressure ratio (Pp/Ps) at the time of weaning from cardiopulmonary bypass. The effect of 40 ppm of inhaled NO for 15 min was evaluated in patients whose Pp exceeded systemic values. RESULTS: Preoperative PVR correlated positively with Pp/Ps at the time of weaning from cardiopulmonary bypass (r2 = 0.86; P < 0.05; n = 46). A Pp/Ps greater than or equal to 1 was not observed in any cases in which the preoperative PVR values were less than 7 Wood units m2; Pp/Ps ratio greater than or equal to 1 occurred in four patients. Each of these had PVR values greater than 7 Wood units m2. Three of these patients who had PVR values in the 7-12 Wood units m2 range were responsive to inhaled NO. The fourth patient, whose PVR value was greater than 15 Wood units m2, was unresponsive. Lung biopsy specimens were obtained in two patients whose preoperative PVR values were greater than 10 Wood units m2. CONCLUSION: Preoperative PVR correlates reasonably well with postbypass Pp/Ps.     

Arginine vasopressin is an ideal drug after cardiac surgery for the management of low systemic vascular resistant hypotension concomitant with pulmonary hypertension

Low systemic vascular resistance (SVR) hypotension concomitant with pulmonary hypertension (PH) is difficult to manage postoperatively because they are often catecholamine-resistant. So, we applied arginine vasopressin (AVP), which is a potent vasoconstrictor in a specific condition, for post-cardiotomy refractory low SVR hypotension concomitant with PH. We treated nine cases of postoperative refractory vasodilatory hypotension concomitant with PH even after conventional treatment that included nitric oxide inhalation and/or intraaortic balloon pump. AVP was administrated with 0.05 approximately 0.1 U/min intravenously. After AVP administration, the mean systemic arterial pressure increased from 47.3+/-9.5 to 76.5+/-12.2 mmHg (P<0.01) and SVR increased from 488.1+/-92.7 to 1188+/-87 dynes x s x cm(-5) (P<0.01). Fortunately, even though the cardiac index decreased, it remained in a normal range. Alteration in the PVR was not significant, but the Pp/Ps became somewhat lower (0.66+/-0.2 to 0.47+/-0.16, P<0.01). AVP increased the urine output and improved oxygenation. AVP improved systemic circulation (increased systemic blood pressure with maintaining cardiac output) without deterioration of pulmonary hypertension. AVP is an ideal drug for treating refractory low SVR hypotension concomitant with PH. But its indication must be limited.     

8kg以下婴儿室间隔缺损伴肺动脉高压手术治疗

为提高小婴儿室缺伴肺动脉高压的手术疗效，总结１６例８ｋｇ以下室间隔缺损（室缺）伴肺动脉高压婴儿行急诊手术修补室缺的经验。其中１４例在深低温停循环下进行，手术效果满意；术后死亡１例（６％）。作者认为，婴儿巨型室缺应尽早手术治疗为宜，深低温停循环方法尤为适合；术后强调持续保持病婴镇静及呼吸道通畅，防止肺高压危象的发生。     

右心旁路手术对肺血管eNOS分泌功能的影响

目的 研究长期肺循环无搏动血流对微血管床结构和功能的影响.方法 建立犬右肺单侧无搏动血流灌注的动物模型,6个月后对比研究两侧肺组织毛细血管床微小动脉结构以及血管内皮细胞一氧化氮合酶(endothelial nitric oxide synthase,eNOS)的变化.结果 无搏动血流灌注的右肺微小动脉eNOS表达显著低于左肺(t=2.24,P＜0.05),细胞凋亡fas表达显著高于左肺(t=2.16,P＜0.05),两者显著负相关(r=-0.82,t=0.151,P＜0.05).图像分析显示,微小动脉管壁面积与血管总面积比值右肺显著低于左肺(t=2.14,P＜0.05).结论 长期无搏动性血流灌注会引起肺血管内皮细胞对eNOS合成减少,导致微动脉血管壁平滑肌细胞凋亡增加,动脉静脉化.     

Left pneumonectomy for lung cancer after correction of contralateral partial anomalous pulmonary venous return

We report the successful treatment of a 48-year-old man with left lung cancer and contralateral partial anomalous pulmonary venous return (PAPVR). He was found to have an abnormal shadow on a regular checkup. Sputum cytology revealed squamous cell carcinoma. Chest computed tomography showed not only a left hilar mass but also showed that his right superior pulmonary vein was draining into the high portion of the superior vena cava. In the presence of the right partial anomalous pulmonary venous return, it was believed that left pneumonectomy would cause serious postoperative heart failure due to an increase in the left-to-right shunt. Therefore his partial anomalous pulmonary venous return was corrected first under cardiopulmonary bypass, and 3 weeks later he underwent successful radical left pneumonectomy.     

肺动脉高压幼犬肺动脉压力与肺动脉结构的关系

目的 探讨肺动脉高压（ＰＨ）时肺动脉压力（ＰＡＰ）与结构的关系。方法 实验幼犬１５条,分２组：分流组（ｎ＝１２）,正常对照组（ｎ＝３）。分流术后９０ｄ两组行血流动力学检测后,放血处死,肺组织切片行病理学检测。结果 （１）分流组左下肺动脉压（ＰＬＬＰＡ）由１．３６ｋＰａ（１ｋＰａ＝０．７５ｍｍＨｇ）升至１１．１３ｋＰａ,左下肺血管阻力（ＬＬＰＶＲ）由５．６５Ｗｏｏｄ’ｓ单位升至１４．３１Ｗｏｏｄ’ｓ     

Total anomalous pulmonary venous connection in adult: report of a case

Total anomalous pulmonary venous connection (TAPVC) is a cardiac disorder that seldom permits survival into adulthood without surgical correction in infancy. We reported a successful total correction in a 47-year-old man with TAPVC. Cardiovascular angiography demonstrated the features of Darling I a type TAPVC. Cardiac catheterization showed Qp/Qs: 5.8, Pp/Ps: 0.31, Rp/Rs: 0.06 and mild pulmonary hypertension. On operation, the posterior wall of left atrium anastomosed to the common pulmonary vein over 4 cm length, ASD (5.0x3.8 cm) was closed with patch, and the communicating vein was ligated. The patient made uneventful postoperative recovery and is well 3 months following surgery.     

A successful arterial switch operation for a patient with pulmonary venous obstruction and pulmonary hypertension five years after the Mustard procedure

A five year and six month old boy, who had undergone Mustard operation for complete transposition of the great arteries with intact ventricular septum at two months of age, was reoperated on for pulmonary venous obstruction and pulmonary hypertension (PLV/RV = 0.96). The other complications which he had were moderate tricuspid regurgitation and various supra-ventricular arrhythmias. After the atrial baffle of the Mustard procedure was taken down, atrial septum was reconstructed and arterial switch operation was performed at the same time. One month after the operation, Pp/Ps was 0.41 and no tricuspid regurgitation and arrhythmias were found. He was discharged and his quality of life was ameliorated surprisingly.     

Anomalous dual drainage of the right pulmonary veins in a patient with cor triatriatum: report of a case without scimitar sign

The majority of patients with partial anomalous drainage of pulmonary veins are asymptomatic during infancy and childhood. Patients with significant left-to-right shunt develop symptoms and benefit from early corrective surgery. Anomalous pulmonary veins draining into inferior vena cava is very rare and frequently encountered in association with scimitar syndrome. The purpose of this case report is to describe a non-scimitar patient with cor triatriatum who had anomalous dual drainage of right pulmonary veins into inferior vena cava/left atrium and anomalous connection of persistent left superior vena cava with a common pulmonary venous chamber. The patient underwent an operation with redirection of anomalous pulmonary venous drainage into left atrium and ligation of persistent left superior vena cava.     

Anesthetic management of a patient with pulmonary hypertension due to a large anterior mediastinal tumor anticipated by preoperative computed tomography

A 74-year-old man underwent an operation for an extremely large mediastinal tumor. Despite the lack of respiratory difficulty or distention of the jugular veins, preoperative computed tomography showed suppression of the left atrium by the huge tumor. We suspected pulmonary hypertension and monitored continuously with a pulmonary catheter. The Pp/Ps ratio decreased gradually from 0.8 to 0.7 by continuous administration of alprostadil or olprinone. After tumor resection, the Pp/Ps ratio was reduced and maintained at 0.2 even after alprostadil and olprinone administration was discontinued. The tumor was considered to have caused the severe pulmonary hypertension, as anticipated from the preoperative CT.