先天性心脏病肺高压肺超微结构临床研究

以左向右分流先心病患儿的肺组织为材料 ,进行肺超微结构与临床的对比研究 ,旨在探讨先心病肺组织超微结构的改变及其与临床的联系 ,为临床应用奠定基础。资料和方法搜集本院近 4年来手术中测压和肺活检病理电镜资料完整者 18例先心病患儿 ,其中男 10例 ,女 8例 ,年龄 0 .5～ 10 .0岁 ,平均 (4.70± 3.40 )岁。室间隔缺损 (ＶＳＤ) 8例 ,房间隔缺损 (ＡＳＤ) 3例 ,动脉导管未闭 (ＰＤＡ) 2例 ,ＶＳＤ ＰＤＡ 3例 ,ＶＳＤ ＡＳＤ 2例。术中测量肺动脉压。肺组织标本均取自右肺中叶内段末梢。常规制作电镜样品 ,在Ｈ 5 0 0型电镜下观察并摄…     

血管形成因子- 1与肺动脉高压形成关系的研究

目的了解血管形成因子-1(Angpt-1)与肺动脉高压(PH)形成的关系。方法收集11例存在PH的先天性心脏病(先心病)患儿的肺组织,应用荧光定量PCR法分析Angpt-1mRNA的表达率,免疫组化技术分析Angpt-1蛋白的表达;并将11例先心病伴肺高血流患儿和6例正常儿童肺组织进行比较。结果PH患儿和肺高血流组患儿肺组织中Angpt-1mRNA的表达率和Angpt-1蛋白的表达均高于正常组(P<0.01)。结论Angpt-1参与和促进先心病肺动脉高压的发生和发展。     

肺出血幼兔右心室压力及肺毛细血管内皮细胞超微结构的改变

目的　研究肺出血幼兔肺动脉压力及肺血管内皮细胞超微结构的变化 ,为预防新生儿肺出血提供理论依据。方法　 15只幼兔耳缘静脉注射高分子右旋糖苷 (T 5 0 0 ) 1ml/kg,连续 5d (7只 )和 8d (8只 )。对照组 (8只 )注射相同剂量的生理盐水 ,连续 5d。经右心室穿刺 ,通过多道生理记录仪记录右心室压力 ,并以此反映肺动脉压力。透射电镜观察肺血管内皮细胞超微结构变化。结果　实验组幼兔右心收缩压第 5天为 (16 6± 1 2 )mmHg (1mmHg =0 .133kPa) ,第 8天为 (19 2±1 1)mmHg ,明显高于对照组的 (12 2± 1.3)mmHg(P <0 .0 1)。超微结构表现为肺动脉内皮细胞水肿 ,肺泡毛细血管内皮细胞破坏 ,II型上皮细胞内层状小体空泡变性。结论　高粘滞血症引起幼兔肺出血 ,伴有肺动脉压力显著升高及肺毛细血管内皮细胞和肺泡上皮细胞超微结构异常。这些改变是高粘滞血症导致幼兔发生肺出血的病理基础。     

小儿先天性心脏病并肺动脉高压血清基质金属蛋白酶与其组织抑制物的变化

目的探讨先天性心脏病(先心病)患儿血清中的基质金属蛋白酶(MMP-2、MMP-9)、金属蛋白酶组织抑制物(TIMP-1)表达水平及其变化。方法2005年6月至2006年5月广西医科大学第一附属医院儿科收治左向右分流型先天性心脏病患儿81例,按治疗前肺动脉收缩压(SPAP)分为4组:非肺动脉高压(PH)组16例(SPAP<30mmHg),轻PH组21例(30mmHg≤SPAP<40mmHg),中PH组21例(40mmHg≤SPAP<70mmHg)和重PH组23例(SPAP≥70mmHg)。其中19例接受前列腺素E1(PGE1)治疗,24例接受手术治疗。对照组为21例为同龄正常体检儿。用酶联免疫吸附法(ELISA)检测血清MMP-2、MMP-9和TIMP-1含量,计算MMP-2/TIMP-1、MMP-9/TIMP-1。结果与对照组相比较,先心病各组MMP-2均显著增高;在各PH组MMP-9、TIMP-1均显著增高;在非PH、轻PH、中PH组MMP-2/TIMP-1均显著增高;在轻PH、中PH组MMP-9/TIMP-1显著增高。19例经PEG1治疗14d后,MMP-2、MMP-9和TIMP-1均显著下降。24例在手术治疗1周后,MMP-2、MMP-9、TIMP-1和MMP-2/TIMP-1均显著降低。结论(1)左向右分流型先心病血清存在MMP-2、MMP-9的显著增高,MMP-2、MMP-9可能协同参与肺血管重构,MMP-2对触发肺血管重构可能起着更积极的作用;(2)PGE1可有效降低先心病血清MMP-2、MMP-9和TIMP-1,这可能是PGE1逆转肺血管重构的机制之一;(3)介入封堵术和外科手术均可有效降低轻、中PH患儿血清MMP-2、MMP-9、TIMP-1和MMP-2/TIMP-1,其机制在于这两手术都阻断了先心病异常的血流动力学这一肺血管重构的始动因子。     

先天性心脏病合并肺动脉高压时肺组织病理与免疫组化研究

为探究先天性心脏病（先心病）伴肺动脉高压（肺高压，PH）患儿肺组织一氧化氮合酶（eNOS)的表达有否改变，随机选择法洛四联症（tetralogy of Fallot,TOF)、单纯室间隔缺损(ventricular septal defect,VSD)/房间隔缺损（atrial septal defect,ASD)、先心病合并PH患儿48例，分成3组，取少许右肺中叶组织，利用免疫组织化学法对eNOS进行半定量分析。结果显示先心病伴PH组患儿肺组织内皮细胞内的eNOS染色明显弱于TOF组和单纯VSD／ASD组患儿（P＜0．01）。提示先心病PH组肺组织的eNOS含量水平低下，造成内源性NO生成减少，为临床吸入NO治疗PH提供了理论依据。     

先天性心脏病肺动脉高压患儿肺组织内皮素-1与胶原合成关系的研究

目的研究左向右分流先心病肺动脉高压(肺高压)患儿肺组织内皮素-1(ET-1)与肺组织Ⅰ、Ⅲ型胶原合成的关系,探讨左向右分流先心病患儿肺高压形成的机理。方法选择左向右分流先心病患儿18例,根据肺动脉收缩压分为肺高压组(n=10),非肺高压组(n=8)。所有患儿在外科手术体外循环开始前,取右肺中叶组织(1cm×1cm×1cm),放射免疫法测定患儿血浆及肺组织ET-1含量;RT-PCR法检测肺组织Ⅰ、Ⅲ型胶原mRNA表达。结果先心病肺高压患儿血浆及肺组织ET-1含量明显高于肺动脉压正常者(P<0.01)。肺高压患儿肺组织Ⅰ、Ⅲ型前胶原mRNA的表达与肺动脉压正常者相比明显增加(P<0.05)。结论ET-1可能通过刺激肺动脉平滑肌细胞胶原合成增加参与肺高压的形成。     

白细胞介素-1受体拮抗剂对NZB/WF_1小鼠狼疮肺损害防治作用的研究

目的　探讨白细胞介素 1受体拮抗剂 (IL 1ra)在狼疮性肺损害防治中的价值。方法对有狼疮倾向的NZB/WF1小鼠腹腔内注射生理盐水和IL 1ra,对比观察其对细胞因子活性和肺部病理形态学改变的影响。结果　实验组和对照组IL 2活性分别为 (2 3± 4)U/ml和 (18± 3)U/ml(P <0 .0 5 ) ;NK细胞活性分别为 (76± 2 1) %和 (45± 4) % (P <0 .0 5 ) ;IL 1α分别为 (173± 78)pg/ml和 (44 2±10 0 )pg/ml(P <0 .0 1)。对照组小鼠肺组织病理变化重 ,表现为细支气管及小血管周围重度慢性炎性细胞浸润 ,毛细血管扩张充血 ,肺泡间质明显增厚及纤维化 ,而实验组小鼠毛细支气管和小血管周围炎性细胞浸润、毛细血管扩张 ,以及肺泡间质增厚均较对照组明显为轻。结论　IL 1ra在预防和治疗系统性红斑狼疮所致的肺损害方面有较好的效果。     

不伴体-肺动脉侧支和动脉导管未闭的婴幼儿肺血减少型复杂先天性心脏病的肺组织病理改变

目的：婴幼儿肺发育过程中,肺循环血量的改变影响肺外周血管的发育,导致肺组织的结构出现病理性改变。该研究利用形态半定量分析方法,了解不伴体-肺动脉侧支和动脉导管未闭的肺血减少型复杂先天性心脏病的婴幼儿肺细小动脉和肺泡间质的病理改变特点。方法选择有肺组织病理切片资料的56例婴幼儿不伴体-肺动脉侧支和动脉导管未闭的肺血减少型复杂先心病患儿作为病变组,年龄4～36月,包括法洛四联症34例和合并肺动脉狭窄的右室双出口7例、单心室9例、三尖瓣闭锁4例和完全性心内膜垫缺损2例。5例年龄4～18月非心、肺源性疾病死亡的婴幼儿作为对照组。采用显微镜形态半定量分析技术测量肺细小动脉内、外弹力板间距,计算平均中膜厚度百分比（MT%）和中膜面积百分比（MS%）,单位面积肺细小动脉数目（APSC）,单位面积肺泡数（MAN）,平均肺泡内衬间隔(MLI),肺实质占同切片肺总面积比例(PPA%)和单位面积肺泡数/肺细小动脉数比（AAR）。结果：先心病组的MT%,MS%,APSC和MAN降低,肺细小动脉内弹力板间距、AAR和MLI升高,与对照组比较差异有显著性,其余参数差异没有显著性。先心病组多数肺细小动脉形状不规则。结论：不伴体-肺动脉侧支和动脉导管未闭的肺血减少型复杂先心病的婴幼儿外周肺细小动脉中膜变薄、管腔扩张,单位面积肺细小动脉数目和单位面积肺泡数目减少,肺泡内径增大。     

先天性心脏病合并肺动脉高压患儿肺血管中维甲酸受体表达的变化

目的 通过观察不同肺血流和(或)肺动脉压力情况下先天性心脏病(先心病)患儿手术时活检肺组织中肺血管维甲酸受体(RARα、β、γ亚型)的表达情况,探讨其在先心病合并肺动脉高压发病机制中的可能作用.方法 收集先心病及非先心病患儿开胸手术病例共31例,其中无高肺血流及肺动脉高压的非先心病手术对照组病例6例;左向右分流先心病高肺血流组7例;左向右分流先心病肺动脉高压组18例.手术时取右下肺组织,连续切片后采用小鼠抗人RARα、β、γ亚型单克隆抗体进行免疫组化显色,图像分析观察三种血型相对表达面积,同时分析其与肺高压组肺动脉压力、HE病理分级的相关性.结果 肺动脉高压组肺动脉中RAR α、β、γ亚型阳性表达面积均较对照组及高肺血流组明显减少(P均<0.01),而对照组与高肺血流组之间差别无统计学意义(P>0.05);肺高压组RAR各亚型相对表达面积与肺动脉压力无相关性(r=0.03～0.42,P均>0.05);RARα、β表达与HE病理分级有相关性(r=0.59、0.52,P均<0.01),RARγ与HE病理分组无相关性(r=0.21,P>0.05).结论 先心病合并肺动脉高压时肺动脉中RAR各亚型表达均有减少,且RARα、β表达减少随肺血管病变程度加重而愈加明显,提示RAR可能参与了先心病肺动脉高压的肺血管重建过程.     

复杂性先天性心脏病的介入治疗

目的探讨先天性心脏病(简称先心病)介入治疗在复杂性先心病中的应用.方法应用Rashkind球囊导管对59例复杂性先心病患儿行球囊房隔造口术(BAS);对2例右室发育不良综合征患儿进行静态球囊房隔扩张造口术;对1例在大动脉转位术后出现肺动脉吻合口狭窄的完全性大动脉转位(TGA)的患儿,采用球囊扩张导管进行扩张;对15例法洛四联症(TOF)患儿进行经皮球囊肺动脉瓣成形术(PBPV)、球囊血管成形术;对18例合并主动脉至肺动脉侧支血管的患儿以及5例B-T分流术的患儿在外科根治术前采用弹簧圈进行侧支血管和分流管道的堵塞术;对1例Fontan手术后的先心病患儿采用Amplatzer房间隔缺损(简称房缺)堵塞装置进行窗孔堵塞术.结果46例TGA患儿BAS后动脉血氧饱和度(SaO2)由0.57±0.17上升至0.76±0.13(t=14.58, P<0.01),左右心房压差﹤2 mmHg(1 mmHg=0.133 kPa),术后房缺5～20 mm;10例肺动脉闭锁伴室间隔完整的患儿BAS后SaO2变化不大(t=1.57, P﹥0.05),左右心房压差﹤2 mmHg;TOF患儿经PBPV及球囊血管成形术后SaO2平均上升15%,肺动脉瓣狭窄解除;18例主动脉至肺动脉侧支血管堵塞术,14例效果良好被完全堵塞;5例B-T分流管道堵塞术,全部封堵成功;1例Fontan手术后窗孔堵塞术,获完全封堵.结论复杂性先心病应用介入治疗和外科手术相配合,可获得更好的疗效.     

High-altitude pulmonary edema with absent right pulmonary artery

High-altitude pulmonary edema potentially is fatal. Adults with unilateral absence of a right pulmonary artery are particularly susceptible to high-altitude pulmonary edema. The occurrence of high-altitude pulmonary edema was documented in a child with congenital absence of the right pulmonary artery. Improvement occurred only upon descent to low altitude. Physicians should be aware of this life-threatening condition in children ascending to high altitude, particularly in individuals with unilateral absence of a pulmonary artery.     

Common pulmonary vein atresia without anomalous pulmonary venous connection

Common pulmonary vein atresia without pulmonary venous connection is a rare form of congenital heart disease. No communication exists between the confluence of the pulmonary veins and the heart or a major systemic vessel. A case diagnosed antemortem is presented. Correct early diagnosis is imperative as surgery may be corrective.     

Liposteroid against refractory pulmonary haemorrhage in idiopathic pulmonary haemosiderosis

We describe two Japanese children with idiopathic pulmonary haemosiderosis (IPH), whose refractory haemorrhages were treated with an intravenous lipid emulsion containing dexamethasone (liposteroid). A 22-month-old boy and a 14-month-old girl have been observed with similar symptoms; periodic bouts of anaemia, reticulocytosis, diffuse infiltrates on chest X-ray and the finding of siderophages in sputum or gastric lavage fluid. The MRI of the lung was useful for the diagnosis. Methylprednisolone pulse therapy was successful in treating acute massive bleeding. Subsequent oral prednisolone could not prevent chronic recurrent haemorrhages. However, the intermittent administration of liposteroid (0.05 mg/kg/dose IV) led to a cessation of bleeding; the haemoglobin concentration rose to normal levels. This observation emphasizes the usefulness of liposteroid in the management of refractory IPH.     

Congenital pulmonary arteriovenous fistula producing pulmonary arterial steal syndrome

Summary This report describes a congenital pulmonary arterial steal syndrome manifested as cyanosis and acidosis in a newborn. A fistulous connection between the right pulmonary artery and a large, anomalous right common pulmonary vein stole blood from the pulmonary arteries. The anomaly was suspected because of a pericardiac shadow on frontal and lateral chest films, substantiated by M-mode echocardiogram, confirmed at cardiac catheterization with angiocardiography, and analyzed at postmortem examination. Supported in part by a grant from The Rose M. Badgeley Charitable Trust     

Intralobar pulmonary sequestration occurring without alteration of pulmonary parenchyma

Typical changes of lung sequestration, including the scimitar sign, were seen on plain films in the left lower lobe. At aortography vasculature of a pulmonary sequestration was seen located paravertebrally in the left posterobasal segment. The arterial supply arose from a division of the thoracic aorta above the diaphragm; venous drainage was to the pulmonary veins. No pulmonary parenchymal changes were detected in the sequestration after resection, nor was there an extrapleural component of the sequestration.     

Pediatric pulmonary CT-scanning

Forty three lung scans, obtained in 29 anaesthetized children, were evaluated and compared with 85 scans performed in 52 sedated children. Confluent high absorptive areas in the lower parts of the lungs were found in 35 (81%) of the scans performed in children under general anaesthesia but such areas were not found in the scans performed under sedation. — For general anaesthesia, halothane-N₂O-O₂ was used in all but one patient. The radiological changes are presumably due to a fall in functional residual capacity with consequent airway closure. — It is important not to misinterpret these anaesthesia-induced pulmonary changes which may obscure or mimic true pathological lesions, such as parenchymal and pleural metastases.     

Visualization of pulmonary vein obstruction by pulmonary artery wedge injection

Summary One of the major postoperative complications of total anomalous pulmonary venous connection is obstruction of the pulmonary veins. This event may be apparent immediately after surgery when it is usually due to congenitally obstructed veins or later when it may be the result of failure of commensurate growth of the left atrium-common vein anastomosis or of scarring at the anastomotic site. The exact location of the obstruction becomes, then, of paramount importance in deciding if the patient can be helped by further surgery. At cardiac catheterization, pulmonary artery wedge injection was the only angiographic technique that visualized the site and degree of pulmonary venous obstruction in the case reported here.