左向右分流先天性心脏病患儿血浆内皮素-1水平变化及意义

目的 :探讨影响先天性心脏病 ( CHD)患儿血浆内皮素 - 1( ET- 1)水平的因素及临床意义。方法 :选择69例左向右分流的 CHD患儿 ,其中伴肺动脉高压 ( PH) 5 0例 ,轻度 PH17例 ,中度 PH16例 ,重度 PH17例 ;不伴PH 19例。分别采股静脉血 ,伴有中、重度 PH 3 3例患儿随机给予前列腺素 E1 ( PGE1 ,16例 ) 2 0 ng/ ( kg· min)静脉滴注或卡托普利 ( 17例 ) 1mg/ ( kg· d)口服治疗 15 d后再采血 ,用放射免疫法测定样品中 ET- 1含量。结果 :1肺血流量增加 ,血浆 ET- 1水平亦增加 ,达正常对照 2倍多 ( P <0 .0 0 1) ;2 CHD并 PH者血浆 ET- 1水平较无PH者明显增高 ( P<0 .0 0 1) ,ET- 1含量随肺动脉压力的升高而增加 ;3 PGE1 可显著降低 PH患儿的平均肺动脉压 ( P <0 .0 5 )及血浆 ET- 1水平 ( P <0 .0 5 ) ;4卡托普利可使患儿的平均肺动脉压及血浆 ET- 1水平下降 ,但与治疗前比较差异无显著性意义 ( P >0 .0 5 )。结论 :PGE1 和卡托普利可能延缓 PH的发展。     

Fontan术后血浆内皮素-1和肾上腺髓质素的改变研究

目的 探讨血浆内皮素-1(ET-1)和肾上腺髓质素(AM)在Fontan手术前后的变化情况及相互关系.方法 随机选择2012年1～6月武汉亚洲心脏病医院行Fontan手术的患儿8例,为Fontan组;另随机选择8例先天性心脏病患儿行双心室修复,为对照组.两组患者术前经超声心动图或心导管检查都排除了继发性肺动脉高压.于体外循环(CPB)前即刻采5ml静脉血测定血浆ET-1和AM水平.术后即刻,术后6h、24h采静脉血测定血浆ET-1和AM的水平.结果 两组血浆ET-1水平于CPB后均升高,在CPB后6h达到峰值,CPB后24h减少.在CPB后6h及24 h,Fontan组与对照组相比,血浆ET-1水平呈显著高值[(8.41±2.28)pg/ml比(5.30±1.17)pg/ml,P=0.004;(8.40±2.28)pg/ml比(2.45±0.70)pg/ml,P=0.031],差异有统计学意义.两组血浆AM水平在CPB后均立即增加,在CPB 6 h后对照组达到峰值.CBP后6h,Fontan组血浆AM水平升高的程度显著低于对照组[(81.50±37.93)pg/ml比(221.00±59.71)pg/ml,P=0.045],24h迅速恢复到CPB前基线水平,与对照组相比表现为显著低值[(72.62±25.07)pg/ml比(174.75±73.33)pg/ml,P=0.000],差异有统计学意义.结论 Fontan手术体外循环后患者ET-1表现出显性效应,可能在Fontan手术后的血管收缩机制中起重要作用.AM参与了Fontan手术后保护机制.     

内皮素-1在肺动脉高压发病机制中的研究进展

内皮素-1是一种作用强、持续时间长的血管收缩物质.内皮素-1不仅可作用于肺血管的内皮细胞、平滑肌细胞、成纤维细胞,参与肺动脉高压的发生,还可通过参与炎性反应与免疫、线粒体代谢异常等过程,在肺动脉高压的发病机制中发挥重要作用.     

大内皮素-1评价特发性肺动脉高压严重程度和预后的研究

目的研究特发性肺动脉高压（IPAH）患者血浆大内皮素-1水平与肺动脉高压严重程度和预后的关系。方法回顾性分析2005年6月到2007年6月在阜外心血管病医院住院并诊断为IPAH的82名患者,并进行随访,统计采用SPSS13．0软件包。结果82名IPAH患者平均随访（17．51±7．66）个月,随访到71例（86．6％）,11例失访（13．4％）,随访期间有21人（25．6％）死于右心衰竭,男性IPAH患者血浆大内皮素-1水平（1．77±1．76fmol／ml）较女性（1．52±1．79fmol／ml）没有显著性差异（P=0．666）。以性别和年龄作为控制因素,偏相关分析结果显示IPAH患者血浆大内皮素-1水平与平均肺动脉压呈正相关（R=0．354,P=0．037）,与动脉血氧分压不相关（R=0．058,P=0．694）。单因素COX分析显示动脉血氧分压（P=0．015,RR=0．950）、血浆大内皮素-1（P=0．022,RR=1．281）和平均肺动脉压（P=0．004,RR=1．054）是影响IPAH患者生存的危险因素。多因素COX分析显示仅血浆大内皮素-1（P=0．037,RR=1．450）能预测IPAH患者的预后。结论男性和女性IPAH患者血浆大内皮素-1水平没有显著差异。血浆大内皮素-1与平均肺动脉压呈正相关,与动脉血氧分压不相关。血浆大内皮素-1是特发性肺动脉高压患者预后的独立预测因子,浓度越高,预后越差。     

一氧化氮对急性缺氧性肺动脉高压及内皮素-1分泌的影响

一氧化氮（NO）可通过激活平滑肌细胞内的可溶性鸟着酸环化酶而使肺血管舒张。我们观察了NO吸入对犬急性缺氧性肺动脉高压和血浆内皮素-1（ET1）的影响。此外尚观察了NO合成酶抑制剂NG-硝基-L精氨酸甲酯（L-NAME）对人脐静脉内皮细胞分泌ET-1的影响,以研究NO是否具有抑制ET-1分泌的作用。材料与方法整体动物试验分成2组：单纯缺氧组,犬10只,体重（12．8±26）kg和NO吸入组,犬8只,体重（13．8±2．4）kg。动物麻醉后行气管插管,接呼吸机,右颈外静脉置人漂浮导管达肺动脉,右股动脉、静脉分别留置导管。先记录空气通气时的动…     

霉酚酸对内皮素-1诱导的肺动脉平滑肌细胞增殖迁移收缩的影响

目的 研究霉酚酸对内皮素-1诱导的大鼠肺动脉平滑肌细胞(PASMCs)的增殖、收缩和迁移的影响,探讨霉酚酸酯对肺动脉高压的作用机制.方法 采用四甲基偶氮唑蓝(MTT)、划痕实验、Millicell培养小室及测微尺计量的方法观察霉酚酸对内皮素-1诱导PASMCs增殖、迁移和收缩的影响,并了解鸟嘌呤对霉酚酸抑制PASMCs增殖的逆转作用.采用配对t检验进行统计分析.结果 与内皮素-1组相比,低浓度霉酚酸组平滑肌细胞增殖降低[吸光度(A)值:0.348±0.036与0.447±0.013,t=6.357,P=0.000];高浓度MPA组进一步降低.与低浓度霉酚酸组相比,霉酚酸加鸟嘌呤组平滑肌细胞增殖有所增加(A值:0.390±0.018与0.348±0.036,t=2.573,P=0.028).霉酚酸组细胞平均迁移距离较内皮素-1组缩短,霉酚酸组平均迁移细胞数较内皮素-1组减少,霉酚酸组平均细胞长度长于内皮素-1组.结论 霉酚酸具有抑制内皮素-1诱导的PASMCs增殖、迁移和收缩的作用,外源性鸟嘌呤可部分逆转霉酚酸抑制PASMCs增殖的作用.     

缺氧性肺动脉高压大鼠血浆降钙素基因相关肽和内皮素-1的测定及其相互关系

为了探讨血浆降钙素基因相关肽和内皮素—1含量及其相互关系在缺氧性肺动脉高压调节机制中的作用,将Wistar大鼠20只分为:一周缺氧组(5只,一周对照组(5只),二周缺氧组(5只)及二周对照组(5只),低氧处理采用常压低氧舱,舱内氧浓度10±0.5%,用放射免疫方法测定对照组和不同缺氧时间组大鼠血浆降钙素基因相关肽和内皮素—1含量.并通过P_(50)压力传感器法测定其肺动脉平均压。结果表明在缺氧性肺动脉高压大鼠中,一周缺氧组血浆降钙素基因相关肽含量与一周对照组比降低,血浆内支素—1含量与一周对照组比增高,但均无显著差异(t=1.58,2.24,P>0.05)。二周缺氧组血奖降钙素基因相关肽含量与二周对照组比明显降低,血浆内皮素含量与对照组比明显增高(t=2.61,2.31。P<0.05),肺动脉压力与血浆降钙素基因相关肽水平呈明显负相关,与内皮素—1水平呈明显正相关(负相关系数R=0.910,P<0.01)。这一研究结果说明血浆降钙素基因相关肽和内皮素—1共同参与缺氧性肺动脉高压调节。     

内皮素-1对家犬肺动脉的作用机制

目的探讨内皮素-1(ET-1)对家犬肺动脉的作用机制。方法利用家犬离体肺动脉标本,观察ET-1对其张力的影响。结果ET-1(1.0～30.0nmol/L)引起正常肺动脉(27例中的19例)的一过性舒张(低浓度)后持续性收缩(高浓度)的双向反应,去掉内皮可使舒张反应消失且反转为收缩反应,而其收缩反应明显增强(P<0.01)。一氧化氮合酶(NOS)抑制剂左旋硝基精氨酸预处理可使其舒张反应消失,且反转为明显增强的收缩反应(P<0.01)。ETB受体阻断剂BQ788预处理可使其舒张反应消失且反转为收缩反应(P<0.01),而收缩反应明显增强(P<0.01)。在部分正常肺动脉(27例中的8例)ET-1仅引起收缩反应,对该8例用ETA受体阻断剂BQ123预处理后亦引起舒张(低浓度)和收缩(高浓度)双向反应,并使其收缩反应明显减弱(P<0.01);前列腺素内过氧化物合成酶抑制剂吲哚美辛(IM)预处理对其舒张和收缩反应并无影响。结论在家犬肺动脉上存在ETA受体和ETB受体。ET-1作用于平滑肌上的ETA受体引起肺动脉的收缩反应;作用于内皮上的ETB受体后通过释放内皮源性一氧化氮引起肺动脉的舒张反应。     

内皮素与肺动脉高压

肺动脉高压是心血管疾病发病中的异常病理状态，其确切的发病机制尚不清楚。已知内皮素是一种强烈的血管收缩和促平滑肌细胞增殖因子，并对肺血管有较强的作用，因而可能在肺动脉高压的发病中有重要意义。     

Pulmonary Regurgitation after Tetralogy of Fallot Repair: Clinical Features,Sequelae, and Timing of Pulmonary Valve Replacement

Pulmonary regurgitation following repair of tetralogy of Fallot is a common postoperative sequela associated with progressive right ventricular enlargement, dysfunction, and is an important determinant of late morbidity and mortality. Although pulmonary regurgitation may be well tolerated for many years following surgery, it can be associated with progressive exercise intolerance, heart failure, tachyarrhythmia, and late sudden death. It also often necessitates re‐intervention. Identifying the appropriate timing of such intervention could be very challenging given the risk of prosthetic valve degeneration and the increased risk of reoperation. Comprehensive informed and regular assessment of the postoperative patient with tetralogy of Fallot, including evaluation of pulmonary regurgitation, right heart structure and function, is crucial to the optimal care of these patients. Pulmonary valve replacement performed in an experienced tertiary referral center is associated with low operative morbidity and mortality and very good long‐term results. Early results of percutaneous pulmonary valve replacement are also promising.     

Fetal MRI Correlates with Postnatal CT Angiogram Assessment of Pulmonary Anatomy in Tetralogy of Fallot with Absent Pulmonary Valve

In tetralogy of Fallot with absent pulmonary valve, pulmonary stenosis and regurgitation results in significant pulmonary artery dilatation. Branch pulmonary artery dilatation often compresses the tracheobronchial tree, causing fluid trapping in fetal life and air trapping and/or atelectasis after birth. Prenatal diagnosis predicts poor prognosis, which depends on the degree of respiratory insufficiency from airway compromise and lung parenchymal disease after birth. Fetal magnetic resonance imaging (MRI) has been useful in evaluating the effects of congenital lung lesions on lung development and indicating severity of pulmonary hypoplasia. This report is the first demonstrating the utility of fetal MRI in tetralogy of Fallot/absent pulmonary valve patients, which predicted postnatal pulmonary artery size and visualized airway compression and lung parenchymal lesions. The distribution of lobar fluid trapping on fetal MRI correlated with air trapping on postnatal computed tomography angiogram.     

Variability in Surgical Referral Patterns for Pulmonary Valve Replacement in Adults with Repaired Tetralogy of Fallot

Background. Individuals with repaired tetralogy of Fallot (TOF) comprise a substantial proportion of the current adult congenital heart disease population. Pulmonary regurgitation (PR) is one of the most prevalent postoperative sequelae, but timing of pulmonary valve replacement (PVR) in the asymptomatic TOF patient remains controversial. Objective. We sought to explore thresholds for PVR referral among adult congenital physicians. Methods. Physicians attending an international adult congenital cardiac disease conference were given a survey focusing on PVR referral patterns for the asymptomatic individual with repaired TOF. Survey questions related to an asymptomatic adult with repaired TOF, at least moderate PR, and varying degrees of right ventricular (RV) dilation and RV dysfunction. Results. A total of 128 surveys were completed. Nine percent did not feel that PVR was indicated in the asymptomatic patient. Of those practitioners who felt that PVR was indicated, many [(69%, [74/107]) relied on RV end‐diastolic volumes (RVEDV) to guide decision making. Fewer relied on RVEDV for surgical referral as RV ejection fraction (EF) decreased. RVEDV thresholds for PVR referral varied depending on the RV function: with normal RVEF, 180 cc/m² was the most commonly used cutoff; if RV dysfunction was significant, 150 cc/m² was the threshold most often cited. Physicians who utilized RV volumes to guide decision making tended to work in a tertiary care setting (P= 0.008). Conclusions. PVR referral patterns for an asymptomatic TOF patient with significant PR and important RV dilation are variable among adult congenital cardiologists. Uncertainty regarding thresholds for PVR referral underscores the need for further study of this important issue.     

Successful Percutaneous Recanalization of Thrombosed Major Aortopulmonary Collateral Artery in Palliated Tetralogy of Fallot with Pulmonary Atresia

We report the case of a 25‐year‐old male with palliated tetralogy of Fallot and pulmonary atresia presenting with thrombotic occlusion of a major aortopulmonary collateral artery to the right lung. Percutaneous intervention was successful in recanalizing this vessel, resulting in symptomatic improvement.     

法乐四联症合并冠状动脉畸形的外科治疗

目的：先天性冠状动脉畸形少见，但在法乐四联症中则相对多发，给法乐四联症根治术带来了很大困难，并明显地增加其死亡率和并发症发病率。为提高手术成功率和引起外科医生的重视，我们报告了６例此类病人的治疗经验。方法：术前可用超声心动图进行初步筛选，必要时可行心血管造影，以明确冠状动脉走行。手术时根据冠状动脉走行位置选择不同切口，修补室间隔缺损和疏通右心室流出道。结果：除１例无法手术外，５例均成功地实施了根治术。结论：术前诊断直接影响到手术能否成功，术中右心室切口要避免损伤异常冠状动脉     

法乐四联症合并肺动脉瓣缺如的矫治

目的 :探讨法乐四联症合并肺动脉瓣缺如 (TOF/ APV)的外科治疗方法。　　方法 :TOF/ APV患者 5例 ,临床上无明显呼吸道压迫症状 ,均在体外循环下矫治。用同种肺动脉 (4例用带单瓣主肺动脉片 ,1例用带瓣主肺动脉管道 )结合自体心包片重建右心室流出道。　　结果 :无手术和住院死亡。平均随访 2 3± 13个月。超声心动图检查肺动脉瓣功能良好 ,无明显反流。右心室至肺动脉平均流速及压差均较术前明显下降 ,P均 <0 .0 1。　　结论 :TOF/ APV的外科矫治应强调对扩张肺动脉的处理 ,彻底解除气道压迫。应用同种肺动脉重建右心室流出道方法简单 ,效果良好。     

Repair and Follow‐up of Tetralogy of Fallot with Pulmonary Stenosis

Tetralogy of Fallot is the most common cyanotic congenital heart defect. Advances in surgical technique and postoperative care have improved survival which is now very good. Patients now face long‐term morbidities such as reduced exercise tolerance and arrthymias. Cardiologists caring for these patients are confronted with decisions regarding best care practices. This article will review the evidence available on repair and postoperative follow‐up for patients with Tetralogy of Fallot with pulmonary stenosis.     

Anterior and Posterior Pulmonary Cusp Augmentation in the Repair of Tetralogy of Fallot

BackgroundIn this retrospective study, we compared the results from 2 pulmonary valve augmentation techniques in patients undergoing surgical repair of tetralogy of Fallot.MethodsBetween 2015 and 2018, 18 patients had anterior pulmonary valve repair at our institution, and 26 patients had both anterior and posterior pulmonary valve repair.ResultsPatients ranged from 6 months to 30 years of age. The median follow-up period was 8 months in the anterior augmentation group and 5 months in the anterior and posterior augmentation group. Postoperative echocardiograms indicated that only 2 patients (11%) in the anterior augmentation group had moderate or severe pulmonary insufficiency, compared with no patients in the anterior and posterior augmentation group. At follow-up, pulmonary insufficiency was seen in 3 patients (17%) in the anterior augmentation group and no patients in the anterior and posterior augmentation group.ConclusionReconstruction of the native pulmonary valve accompanied by pulmonary cusp augmentation can decrease or even circumvent postoperative pulmonary insufficiency. Both anterior augmentation and anterior and posterior augmentation techniques are easily applied; however, we believe that the anterior and posterior augmentation technique is superior in terms of early postoperative and follow-up pulmonary insufficiency outcomes.