左冠状动脉起源于肺动脉7例临床分析

左冠状动脉起源于肺动脉(anomalous origin of left coronary artery from pulmonary artery,LCAPA)是罕见的先天性心脏畸形,国外报道约占先天性心脏病的0.4%[1].本病预后极差,未行手术治疗者死亡率高.由于对疾病的认识和检查手段方面的原因,早期难以发现,临床误诊率高,尤其易误诊为"心内膜弹力纤维增生症"、"扩张性心肌病"等.而本病经手术治疗能改善预后,故对其早期识别有重要的临床价值.现将近2年收治的7例LCAPA资料总结报告如下.

小儿肺动脉发育异常影像学诊断价值探讨

单侧肺动脉缺如( unilateral absence of pulmonary artery, UAPA)或发育不良是小儿时期罕见的先天性肺血管畸形,因临床缺乏特异性表现,常导致误诊.诊断的主要依据为影像学检查,为提高对本病的认识,现将我院 5例肺动脉发育异常患儿的临床及影像学资料并结合有关文献予以分析,兹报道如下.

动脉导管未闭合并右肺动脉缺如一例

患儿男,1岁10个月.因有轻微活动后心率显著加快、气促,反复肺部感染入院;体格检查:发育尚可,无紫绀,气管居中,双肺呼吸音增粗,未闻哕音;心律齐,肺动脉瓣区明显连续性杂音;胸部X线:左肺纹理增多;心电图:窦性心动过速;心脏超声:动脉导管未闭(PDA),右室稍扩大;心血管造影:PDA=18mm×6mm,降主动脉--左肺动脉(图1,主动脉造影);右肺动脉未显影,提示右肺动脉缺如(absence ofright pulmonary artery APA),左肺动脉发育正常(图2,右室造影,导管经PDA至左肺动脉再进入右心室);肺动脉压84/36/57mmHg,左、右肺静脉发育可.

1. Biventricular hypertrophy has been described in a high blood pressure variability (BPV) model of sinoaortic-denervated (SAD) rats without systemic hypertension. To explore the possible involvement of the lung in SAD-induced right ventricular hypertrophy (RVH), we examined lung morphology, in addition to systemic haemodynamics and ventricle morphology, in Wistar-Kyoto rats 32 weeks after SAD. 2. In Wistar-Kyoto rats 32 weeks after SAD, there existed a substantial elevation in BPV, with no change in the average level of arterial pressure. Biventricular hypertrophy following SAD was characterized by a greater hypertrophy in right than left ventricles; both absolute and normalized right ventricular weights were significantly increased by 22 and 27%, respectively, and only normalized left ventricular weight was significantly increased by 12%. No infarcts were found in any ventricles examined. 3. In the lung, the most prominent change following SAD was pulmonary vasculopathy, including wall thickening, perivascular fibrosis and cell infiltration. In pulmonary arteries with an internal diameter of 70-130 microm, the external diameter, wall thickness and wall thickness to internal diameter ratio were increased in SAD compared with control rats. 4. There was no correlation between right and left ventricular weights. In contrast with BPV-correlated left ventricular weight, right ventricular weight was correlated with the wall thickness of the pulmonary artery, but not with BPV. 5. These findings suggest that greater RVH following SAD is associated with pulmonary vasculopathy, but is not secondary to the left ventricular problems or high BPV.

左肺动脉吊带畸形1例

患儿,男,4月21 d.因咳嗽7 d、气喘4 d入院.当地医院诊断为"支气管肺炎"合并"心力衰竭",给予头孢唑肟抗感染、西地兰强心和速尿治疗,患儿咳嗽、气喘仍无明显好转而转入我院.患儿为第1胎第1产,足月剖宫产,新生儿期健康,于生后1个月出现咳、喘,当地医院诊断先天性喉喘鸣,予头孢类抗生素治疗后症状恢复慢,且反复出现.

新生儿先天性右肺动脉起源异常1例

患儿,女,足月出生,出生体重3 000 g.生后第5天因发现全身皮肤欠红润,四肢肌张力低而入院.入院时T 36.8℃,R 48次/min,HR 145次/min,全身皮肤欠红润,双肺呼吸音清,心律齐,心音过有力,未闻及明显杂音.入院后患儿在安静时呼吸、心率尚平稳,但稍有活动后呼吸增至65～85次/min,心率150～160次/min,皮肤无明显青紫.生后第9天动脉血气分析提示低氧血症(PO246 mmHg),但全身皮肤发绀不明显.

Pinacidil 对低氧性肺动脉高压大鼠肺动脉压和血浆内皮素-1的影响

目的: 探讨钾通道开放剂pinacidil对低氧性肺动脉高压(HPH)及对血浆内皮素-1(ET-1)含量的影响.方法: 将45只雄性Wistar大鼠分为3组:①对照组;②低氧组,每天低氧8 h,共4周;③治疗组,每天低氧前半小时腹腔注射pinacidil 3 mg/kg,共4周,4周后观察3组平均肺动脉压(mPAP),右心室肥厚指标、血浆中ET-1的水平.结果: ①低氧组大鼠mPAP明显高于对照组.右心室肥厚显著,血浆中ET-1含量明显高于对照组;②治疗组mPAP明显低于低氧组,右心室肥厚轻于低氧组,血浆中ET-1含量明显低于低氧组.结论:pinacidil 能有效降低HPH中肺动脉压力、阻抑右心室肥厚,并影响血浆中ET-1的水平.

This experiment, using cultured bovine pulmonary artery endothelial cells (BPAEC), was undertaken to investigate roles of endogenous ONOO- in lipopolysaccharide (LPS)-caused injury to endothelial cells. The fluorescent intensity of nitrotyrosine (NT), a marker of ONOO- generation, in BPAEC represented content of endogenous ONOO- generation. The fluorescent intensity of NT and number of NT positive cells were detected with flow cytometry, and the percentage of NT positive cells was calculated. Results were as follows. (1) LPS (1 mg/L, 5 mg/L and 10 mg/L) caused marked increase in fluorescent intensity of NT in a dose dependent manner. The number and percentage of NT positive cells were markedly increased (P＜0.05). Aminoguanidine (AG), a selective inhibitor of inducible nitric oxide synthase, inhibited the increase in fluorescent intensity of NT in BPAEC induced by LPS. However, the number and percentage of NT positive cells had tendency to reduce. (2) LPS caused the enhancement of MDA content and activity of LDH in cultured supernatant (P＜0.01). AG reversed the enhancement of MDA content induced by LPS (P＜0.01). In contrast, AG had marginal effect on activity of LDH. (3) LPS induced the increase in apoptotic rate in BPAEC in a dose dependent manner. Some BPAEC stained with fluorescent probe ethidium bromide showed morphological features of apoptosis with chromatin condensation and nuclear fragmentation. AG reduced the apoptotic rate and number of apoptotic cells, both of which were still higher than those of vehicle group (P＜0.05). (4) LPS inhibited mitochondrial respiration. Effect of LPS on mitochondrial membrane potential (ΔΨ) depended on the doses of LPS. 1 mg/L LPS led to a little increase in ΔΨ, while 5 mg/L and 10 mg/L LPS significantly reduced ΔΨ. In conclusion, LPS caused injury to cultured BPAEC and increased production of ONOO-. Cytotoxicity of LPS may be mediated by endogenous ONOO-.

In this study we found: 1\, There was endogenous ONOO- formation in lungs in the early stage of endotoxic shock. Exogenous ONOO- led to increase in microvascular permeability, severe lung pathological changes and enhanced MDA content. 2\, It was, for the first time, found that responses of isolated pulmonary artery preincubated with ONOO- showed abnormal manifestations. (1) Low dose of ONOO- let to the inhibition of endothelial dependent relaxation, but enhacement of contractile response, both of which were similar to changes of reactivity in isolated pulmonary artery induced by LPS. (2) High dose of ONOO- reduced contractile response to PE and relaxation to SNP. 3\, ONOO- had direct effect for relaxation of precontracted isolated pulmonary artery. The relaxing action of ONOO- was weak and was negtively regulated by endothelial cells, supporting the notion that ONOO- may be involved in pulmonary hypertension in the early stage of endotoxic shock. 4\, It was, for the first time, found that LPS-induced increase in endogenous ONOO- generation in BPAEC and that endogenous ONOO- mediated injury to BPAEC induced by LPS, which may be a novel mechanism for endotoxin-elicited damage to endothelial cells. 5\, Exposure of pulmonary artery to LPS led to reduction in endothelial dependent relaxation but enhancement in contractile response, both of which were reversed by concomitant exposure to CCK and LPS. 6\, CCK protected cultured BPAEC against the detrimental effects of LPS such as lipoperoxide damages and cellular apoptosis as well as LPS-induced endogenous ONOO- formation. The underlying mechanism of CCK for cytoprotection may be mediated by its receptors and related to its reduced ability of endothelia to generate ONOO- induced by LPS.

In order to investigate the possible role of ONOO- in regulatory disorder of pulmonary arterial tension in endotoxic shock, the responses of rabbit pulmonary arterial rings (PARs) preincubated with ONOO- to endothelial dependent and receptor dependent relaxants acetylcholine (ACh) and adenosine diphosphate (ADP), endothelial dependent and receptor independent relaxant A23187, endothelial independent relaxant sodium nitroprusside (SNP) and α1-adrenoceptor agonist phenylephrine (PE) were observed in vitro in accumulative manner. Results were as follow: (1) Relaxations of PARs to ACh, A23187 and ADP were markedly impaired with shift of accumulative dose response curve of each agonist to the right. Inhibition of endothelial dependent and receptor dependent or independent relaxation by ONOO- was dose dependent. (2) ONOO- incubation inhibited SNP-induced relaxation in a dose dependent manner. Accumulative dose response curve of SNP was right shift to some degree depending on the doses of ONOO-. (3) Contractile response of PARs to PE varied with the different doses of ONOO-. In PARs preincubated with 0.5 mmol/L ONOO-, contractile reponse was significantly enhanced with shift of PE accumulative dose response curve to the left, while in PARs preincubated with 1.0 mmol/L or 2.0 mmol/L ONOO-, it was markedly reduced with right shift of PE accumulative dose response curve. (4) Vehicle of ONOO- had no effect on responses to every agonist, whereas decomposed ONOO- had minimal effect on the response to PE and ADP. In contrast, relaxation of PARs to ACh, A23187 and SNP were enhanced. These results suggested that direct effect of ONOO- on pulmonary artery may be a key factor contributing to regulatory disorder of pulmonary arterial tension induced by LPS and pulmonary hypertension in the early stage of endotoxic shock.

Wild type fragment of erythropoietin(Epo)3'-enhancer (W18) and its mutant type fragment (M18) were synthesized. Primary cultures of endothelial cells of different sources (ECs) and human umbilical venous endothelial cell line (HUVEC) as well as the primary culture of pulmonary artery smooth muscle cells (PASMC) were transfected with either W18 or M18. RT-PCR was performed to detect mRNA and investigate the effect of Epo3'-enhancer fragment on the hypoxia- induced gene expression in ECs. Electrophoretic mobility shift assay(EMSA) was used to test DNA-binding activity of extracted nuclear protein. ［3H］-TdR incorporation, MTT test and flow-cytometry were used to determine the proliferation of PASMC and the effect of Epo3'-enhancer fragment on it. The results showed as follows:(1)The OD value of COX-2 mRNA expressed in hypoxic rat aortic EC was 2.34±0.32, the OD values of COX-2 and VEGF mRNAs expressed in hypoxic EC of pulmonary microvasculature were 1.78±0.21 and 4.71±0.52, the OD value of TXS and ET-1 mRNAs in hypoxic HUVEC were 13.01±4.27 and 1.01±0.05, they were all higher than their counterpart normoxic group (P＜0.05). If the cells were pretransfected with W18, the OD values in these hypoxic groups became 1.28±0.25,0.77±0.09, 2.29±0.41, 4.88±1.05. and 0.51±0.15, respectively, just as low as those of the relevant normoxic groups; (2)The conditioned medium of hypoxic pulmonary artery endothelial cell (PAEC) caused proliferation of PASMC, the cpm values of ［3H］-TdR incorporation was 917.00±527.11, higher than that of normoxic control (P＜0.05). In addition, it was found by using flow-cytometry an increase in the percentage of cells of phases S and G2/M in PASMC incubated in hypoxic EC conditioned medium in comparison with normoxia group and group pretransfected with W18; (3)Hypoxia could induce proliferation of PASMC directly, transfection of W18 could decrease its effect. Their cpm values were 829.50±228.10 and 497.00±52.45, respectively(P＜0.05).The results of MTT test was similar; (4)The inducer of HIF-1, CoCl2 could increase the expression of COX-2 and TXS mRNA in HUVEC, which could also be inhibited by W18 but not by M18;(5)The HIF-1 DNA binding activity was found in hypoxic HUVEC in EMSA with　32　　　　P labeled W18, but not found with 　32　　　　P M18. These results suggest that there might be a common pathway in hypoxic responses of different cells, i.e. regulating the transcription of genes by binding of HIF-1 to a sequence similar to Epo3'-enhancer.

AIM:Increasing evidence suggests that carbohydrate-binding proteins play an essential role in tumor growth and metastasis .Ga-lectin-3, a multifunctional protein of an expanding family of β-galactoside-binding animal lectins , is the major nonintegrin cellular laminin-binding protein , and is implicated in a variety of biologic events , such as inflammation and angiogenesis .Because galectin-3 expression was shown to participate in mediating tumor angiogenesis and initiate signaling cascades in several diseases .We hypothe-sized that galectin-3 may promote pulmonary vascular endothelial neovascularization .METHODS:Hypoxic and MCT rat model of pul-monary artery remodeling was used .The mRNA and protein levels of galectin-3 in rats were measured by in situ hybrization and West-ern blot analysis.Endothelial cell (EC) proliferation, migration and tube formation were measured using MTT , cell scratch and Matri-gel assays, respectively.Protein expression was quantitated by Western blot analysis .LC 3A/B staining was detected with cellular im-munofluorescence staining .RESULTS:We found that galectin-3 was localized on the intima and adventitial wall .Galectin-3 was in-creased after rat hypoxia and MCT administration .Galectin-3 promoted EC proliferation , migration and tube formation , while its roles were reversed by RNA interference.Galectin-3 induced Atg 5, Beclin-1, LAMP-2, and LC 3A/B expression increases.Galectin-3 al-so increased LC 3A/B staining in ECs.Akt/mTOR and GSK-3βsignaling pathways were activated after galectin-3 treated ECs using its specific phosphorylation antibodies , while blocked it with LY294002 inhibited cell autophagy and EC dynamic alterations induced by galectin-3.CONCLUSION:These findings demonstrate that galectin-3 can induce an Akt signaling cascade leading to cell autoph-agy, and then the differentiation and angiogenesis of pulmonary artery endothelial cells .

静脉注射L-NAME对肉鸡肺动脉压的影响

目的:观察静脉注射一氧化氮合酶抑制剂L-NAME对肉鸡肺动脉压的影响.方法:AA肉鸡40羽随机等分为A、B、C、D组,按常规条件饲养至44d龄进行实验.A组为对照组,B、C、D组分别为静脉注射一氧化氮合酶抑制剂L-NAME 1、2、4 h的3个实验组.应用右心导管技术逐羽测定肺动脉压,并采集血样作一氧化氮(nitric ox-ide,NO)和内皮素(endothelin-1,ET-1)水平测定.结果:与A组相比,静注L-NAME 1-2 h后肉鸡血浆NO含量低于A组(P＜0.05),肺动脉压高于A组(P＜0.05),血浆ET-1未见明显差异;静注4 h后,血浆NO含量、肺动脉压与A组水平无明显差异,但血浆ET-1水平明显高于A、B、C组(P＜0.05).结论:L-NAME可能通过抑制NO的合成从而促进了肉鸡肺动脉高压的形成.

肺动脉四瓣变异1例

作者在解剖一具2岁的男童标本时,发现其心脏肺动脉瓣异常,现报道如下:

Coronary Artery Fistulas: CT Findings

Normal coronary arteries terminate in broom-like arborizations,which penetrate the myocardium.CAF is defined as a direct precapillary connection between a branch of a coronary artery and the lumen of a cardiac chamber,the coronary sinus or superior vena cava,or a pulmonary artery or pulmonary vein close to the heart.CAF arises from the right coronary artery (RCA) in approximately 50% of patients,the left coronary artery (LCA) in approximately 42% of patients,and both the RCA and LCA in approximately 5% of patients.The most common drainage sites in order of decreasing frequency are the right ventricle (41%),right atrium (26%),pulmonary artery (17%),coronary sinus (7%),left atrium (5%),left ventricle (3%),and superior vena cava (1%).

成人左冠状动脉异常起源于肺动脉合并二尖瓣膜病变的诊断及手术治疗(附2例报告)

左冠状动脉(LCA)异常起源于肺动脉(anomalous origin of the 1eft coronary artery from the pulmonary artery,ALCAPA)是一种罕见的心血管畸形,每30万新生儿中仅有1例,占先天性心脏病的0.24%～0.46%[1],有87.7%的病儿在1岁蚋因心肌梗死而死亡[2].

多排螺旋CT诊断肺动脉吊带畸形1例

患者女,2岁8个月.因反复咳嗽、咳痰1年半入院.曾多次于当地医疗机构就治,拟诊肺炎、喉炎,但治疗效果不佳,之后渐出现喘息,哭闹时发绀.2009-04于华西医科大学附属医院就诊,纤支镜发现左、右主支气管狭窄,原因不明.2009-06-03于贵阳医学院附属医院行320排螺旋CT增强扫描检查.

左冠状动脉异常起源于肺动脉1例报告

冠状动脉起源于肺动脉是一种较为罕见的先天性心血管异常,约占先天性心脏病的0.26%～0.5%[1].我院近遇到1例,资料较完整,报道如下.

螺旋 CT 与 DSA 对成年型左冠状动脉异位起源于肺动脉的影像学诊断

先天性心脏病左冠状动脉异位起源于肺动脉(left coronary artery from the pulmonary artery,ALCAPA)又称为 Bland-White-Garland 综合征,是一种罕见的疾病,分为成人型(90%)及婴儿型(10%)2类,婴儿型患者往往早期死于严重的心肌缺血和心功能不全,而成人型 ALCAPA 患者猝死的发生率非常高[1-2]。过去由于 ALCAPA 仅能通过传统的冠状动脉造影术做出诊断,故对其做出早期确诊较为困难,近年来,随着检查设备及检查技术的迅速发展,尤其是配合心电门控技术的多排螺旋 CT 及高场 MRI 的快速发展,使得该病能够得到早期、全面、快捷、准确的诊断。由于 ALCAPA 特殊的冠状动脉血流动力学改变,在螺旋 CT 诊断的基础上,配合 DSA 成像检查,可以更加形象、全面地显示其异常的病理生理学改变。本文通过对一组成人型 ALCAPA 病例的螺旋 CT、DSA 影像及临床病理资料进行分析,总结其影像学特点。

小儿原发性肺动脉扩张的影像表现（附3例报告）

原发性肺动脉扩张(idiopathic dilatation of the pulmonary artery,IDPA)也称单纯性肺动脉扩张[1],是临床上一种少见的先天性心血管畸形,该疾病主要特点是肺动脉主干扩张伴或不伴其分支的扩张[2]。本文对3例经临床诊断的 IDPA 的影像学资料进行了分析,并对国内外相关文献进行复习,以期提高对IDPA 的影像诊断认识。