儿童体外循环内源性类洋地黄物质变化

目的 探讨内源性类洋地黄物质与儿童心功能之间的关系及其在体外循环期间变化的临床意义。方法 80例先天性心脏病患儿根据心功能分级分为4组，按先天性心脏病分类分为2组，于术前，主动脉阻断后，转流30min，心脏复苏后和术后24h测定血浆EDLS浓度。结果 (1)儿童血浆EDLS浓度在心功能代偿期与正常对照组无显著性差异，而心功能不全各级分别显著高于正常组，并随心功能不全程度加重而增加；(2)体外循环期间以循环阻断后EDLS浓度最低，随着心脏复苏EDLS浓度逐渐升高，至术后24h EDLS浓度恢复至术前水平；(3)青紫型先天性心脏病与非青紫型先天性心脏病比EDLS血清浓度有显著性差异，而转流期间变化不大。结论 (1)EDLS血浆浓度与心功能不全的严重程度呈密切相关，成为维持心脏功能代偿的因素之一；(2)EDLS随容量负荷增加而增加；(3)儿童心脏病术后适当应用洋地黄制剂是安全的。     

自体冷血停搏液对紫绀型先心病婴儿心脏的保护作用

目的：探讨自体冷血停搏液对紫绀型先心病婴儿心脏的保护作用。 方法：96例行体外循环术的紫绀型先心病婴儿随机分为：HTK液组（HTK组,32例）、非自体冷血停搏液组（非自体组,32例）和自体冷血停搏液组（自体组,32例）,阻断前和主动脉开放30 min取右心耳组织,检测ATP的含量及能量储备（EC）;术前和术后即刻取静脉血,测肌酸激酶同工酶（CK-MB）和心肌肌钙蛋白I（cTnI）的浓度;记录术中复跳时间、复跳率、术后 2 h心脏指数（CI）、正性肌力药物依赖情况和左室射血分数、术后24 h心律失常发生率、术后并发症及病死率等。结果：主动脉开放30 min后,3组ATP 和EC含量均显著降低（P<0.05）,其中自体组较HTK组和非自体组高,差异有统计学意义（P<0.05）。3组术后即刻CK MB和cTnI血清浓度均显著增加（P<0.05）,其中自体组较HTK组和非自体组低,差异具有统计学意义（P<0.05）。自体组在复跳时间、术后2 h正性肌力药物依赖及左心射血分数上优于HTK组和非自体组,差异具有统计学意义（P<0.05）。结论：在紫绀型先心病婴儿体外循环下,自体冷血停搏液比非自体冷血停搏液与HTK液能更好地保存心肌细胞能量,减轻心肌损伤,对心脏具有较好的保护作用。     

先心病患儿心脏手术前后血清神经元特异性烯醇化酶、脑干听觉诱发电位检测的意义

目的 评价脑干听觉诱发电位（BAEP）和血清神经元特异性烯醇化酶（s-NSE)在小儿先心病（CHD）心脏手术前后检测的意义。方法 1．测定体外循环开放式不停跳手术的CHD患儿20例手术前后BAEP的变化；2．测定心脏不停跳、停跳手术患儿各20例术前24h、术后2h、7d的s-NSE水平。结果 1．CHD患儿与对照组比较和手术前或后紫绀型先心病（CCHD）和非紫绀型先心病（NCCHD）比较，CCHD组Ⅲ-Ⅳ、Ⅰ－Ⅴ波和I－V波峰间潜伏期（IPL）显著延长（P＜0．05）。2．手术组术后2h血s-NSE水平显著增高，不停跳组和停跳组平均值与术前比较差异非常显著（P＜0．001）；术后7d s-NSE水平上述两组与术前比较差异显著（P＜0．05）。3．术后2h s-NSE水平停跳组明显高于不停跳组（P＜0．001），且术后7d s-NSE水平两组比较差异显著（P＜0．05）。结论 1．CCHD可伴随脑功能损害；2．s-NSE的变化可提示体外循环手术过程中的脑功能受损；3．就脑保护而言，心不停跳术式优于停跳术式。     

体外循环中低温Perfadex液对室间隔缺损肺动脉高压患者的肺保护作用

目的探讨体外循环中肺动脉灌注低温Perfadex液对合并肺动脉高压婴幼儿的肺功能保护作用：方法将30例合并肺动脉高压的室间隔缺损患儿分为对照组（15例）和肺保护组（15例）,均在体外循环下行心内直视手术。肺保护组体外循环期间于心脏阻断后向肺动脉灌注低温Perfadex液,对照组不灌注。分别于转流前、转流结束、手术结束、术后6h及术后24h测定两组白介素6（IL-6）、白介素8（IL-8）、白介素10（IL-10）以及血浆脂质过氧化物丙二醛（MDA）水平;计算氧合指数（PaO2/FiO2）和肺静态顺应性（TV/Pause）。结果两组体外循环后IL-6、IL-8、IL-10及MDA水平均升高,手术结束时达峰值,以后逐渐下降,术后24h仍高于术前;肺保护组各时点IL-6、IL-8及MDA上升幅度低于对照组（P〈0．01）,各时点IL-10上升幅度高于对照组（P〈0．01）;肺保护组手术结束、术后6h及24h血氧指数高于对照组,P〈0.05或0.01,两组肺静态顺应性较术前明显降低,实验组变化程度明显低于对照组（P〈0．05）。结论肺动脉灌注低温Perfadex液能明显保护肺动脉高压患者术后呼吸功能。     

改良超滤在婴幼儿体外循环中的应用

目的：婴幼儿各组织器官发育尚未成熟,体外循环后各种并发症也相对较为严重。改良超滤技术被证明能减轻婴幼儿体外循环所带来的不利影响,该文分析改良超滤在幼儿体外循环手术中的应用情况。方法：分析261名1岁以下在体外循环下行心脏手术的患者,其中205例（超滤组）体外循环后行改良超滤,56例（对照组）不行改良超滤。比较两组患者的转流时间、主动脉阻断时间、术后引流量、术后输血量、术后呼吸机通气时间、术后24 h时的血球压积以及氧合指数等围手术期资料。结果：超滤组没有发生与超滤有关的并发症,两组患者转流时间和主动脉阻断时间无明显差异,超滤组患者术后引流量和输血量较对照组少,呼吸机通气时间较短,而术后血球压积以及氧合指数较对照组高。结论：改良超滤技术在幼儿体外循环心脏手术后可以明显减少出血量和输血量,可以提高肺的氧合功能,改善呼吸功能,缩短呼吸机支持时间,有利于患者术后恢复。     

心脏直视术后心肌肌钙蛋白Ⅰ的转归及其影响因素相关性分析

目的探讨心脏直视术后心肌肌钙蛋白Ⅰ(TnTi)的转归,并就其与各影响因素作相关性分析。方法收集57例中低温体外循环下行心内畸形纠治术后患儿,年龄(2.34±2.11)岁.体重(10.92±4.59)kg。按病种不同分为A组(法洛氏四联症组,n=31)和B组(室间隔缺损组,n=26)。分别测定术前、主动脉开放5min、6h、12h、24h、48h、72h7个时相点血清TnTi浓度和心功能评分(score),记录体外转流时间(TT)、主动脉阻断时间(CT)以及监护窒预后情况。结果①与术前相比,主动脉开放5min时TnTi显著升高达到峰值(P<0.01),72h后仍高于术前水平(P<0.01);A组患儿血清TnTi显著高于B组患儿,两组cTnTi峰值分别是术前的118和55倍。②患儿TT、CT、手术机械损伤部位及程度与TnTi浓度呈正相关,相关系数分别为0.51、0.51、0.35(P均<0.01)。score与TnTi浓度呈负相关,相关系数为-0.52(P<0.01)。③2.3μg／L是预估患儿术后心功能恢复以及指导临床治疗的临界值。结论TnTi是心脏直视术后评价心肌受损的可靠指标,对反映患儿术后心功能的恢复有一定参考价值。     

60例低出生体重新生儿及早产儿心脏手术术后监护治疗

目的 总结60例低出生体重新生儿(low birth weight infant,LBWI)及早产儿先天性心脏病外科手术的术后监护治疗经验.方法 2003年5月至2011年10月共为60例2 500g以下LBWI先天性心脏病新生儿进行外科手术治疗.本组病例孕周26～42周,平均(33.50±4.07)周,出生体重640～2 500 g,平均(1729.3±522.45)g,其中47例为早产儿.手术时日龄4～55 d,平均(24.88±12.49)d;手术时体重650～2712 g,平均体重(1953.2±463.57)g.术前所有患儿均在NICU接受治疗.患儿均在全麻下接受先心病矫治手术,其巾非体外循环组29例,体外循环组31例(不停跳3例,深低温停循环4例).全组患儿术后进入心外小儿ICU进行监护治疗.结果 全组患儿住院期间死亡13例,总病死率为21.7％.其中术中死亡4例,手术病死率6.7％;术后早期死亡(72h内)6例,占总例数10.0％;术后家属放弃治疗3例,占总例数5.0 ％.体外循环时间35～326min,平均(121.0±74.6)min;主动脉阻断时间27～173min,平均(74.8±44.7)min.术后机械通气时间12～648 h,中位数96 h.全组患儿术后延期关胸13例,术后床旁开胸探查止血8例.其余主要并发症包括重症肺炎10例,肺高压危象2例,低心排综合症8例.结论 早产儿或低体重儿可早期实施先心病外科手术治疗,良好的术后管理有助于降低术后病死率和并发症发生率.     

小儿体外循环对血浆细胞因子水平的影响

目的　评价小儿体外循环心内直视术对血浆细胞因子IL 6、IL 8、IL 10水平的影响。方法　 30例房室水平左向右分流型先心病患儿 (体外循环组 ) ,行体外循环心内直视先天性心脏病手术治疗。分别于麻醉诱导后、转流后 10～ 15min、转流结束、术后 2h、术后 1、2、3d采集外周血 ,双抗夹心法酶联吸附试验 (ELISA)定量测定血浆中IL 6、IL 8、IL 10水平。随机抽取同期非CPB胸科或腹部手术患儿 2 0例 (对照组 )。结果　CPB组体外循环停机后血奖IL 6、IL 8、IL 10水平开始明显升高并于术后 2h达到峰值 (P <0 0 5 )。对照组血浆IL 6、IL 8、IL 10水平于术后 2h达到峰值 (P <0 0 5 ) ,但其参数的变化幅度显著小于体外循环组相应时间点参数值的增高程度。体外循环组引起血浆IL 6、IL 8、IL 10水平的升高幅度显著高于对照组 (P <0 0 5 )。结论　小儿体外循环心内直视手术可导致IL 6、IL 8、IL 10等促炎症和抗炎症介质的释放 ,在体外循环诱发的全身炎症反应中可能发挥重要作用。     

先天性心脏病术后近期严重神经系统异常的相关因素分析

目的总结先天性心脏病(简称先心病)体外循环术后近期(1个月内)发生神经系统异常患儿的临床特征, 分析术后近期发生严重神经系统异常的高危因素。方法回顾性分析2016年2月至2020年4月浙江大学医学院附属儿童医院心脏重症监护室(cardiac intensive care unit, CICU)收治的73例因先心病行体外循环下心脏手术, 术后1个月内发生神经系统异常患儿的临床资料。根据是否需要接受抗癫痫治疗、外科手术干预及高压氧康复治疗将患儿分为严重神经系统异常组(n=15)和非严重神经系统异常组(n=58), 对比两组患儿先心病类型、年龄、体重、体外循环时间、主动脉阻断时间、CICU滞留时间、住院时间、是否存在撤机后再插管以及围手术期心肺复苏(cardiopulmonary resuscitation, CPR)史等资料, 采用单因素分析和二元Logistic回归分析先心病患儿手术后近斯发生严重神经系统异常的影响因素。结果经单因素分析发现, 体外循环时间和主动脉阻断时间长、应用体外膜肺氧合(extracorporeal membrane oxygenation, ECMO)、合并气道狭...     

平衡超滤联合改良超滤对婴幼儿体外循环术后肺功能的影响

目的研究平衡超滤联合改良超滤对婴幼儿体外循环术后肺功能的影响。方法60例先天性心脏病患儿分为对照组（C）、改良超滤组（M）和平衡超滤联合改良超滤组（U）。C组体外循环中不使用超滤，M组在转流结束后应用改良超滤，U组在转流开始后进行平衡超滤，转流结束后进行改良超滤。分别在转流前（T1），转流结束后20min（T2），术后2h（T3），术后6h（T4），术后12h（T5）测定记录气道峰压（Ppeak）、呼吸停顿压（Ppause）、潮气量（TV）、呼吸频率（F）、吸入氧浓度（Fi02）和吸气比例（insp％）、动脉血中自介素6（IL-6）和白介素8（IL-8）的浓度。结果各组体外循环术后较术前的肺静态顺应性（Cstat）、气道阻力（Raw）、肺泡-动脉氧分压（AaDCh）、氧合指数（OI）、IL-6及IL-8浓度都有明显变化。在T5时间U组的Cstat明显高于M组和C组，Raw明显低于M组和C组；在T4、T5时间U组的AaD02明显低于M组和C组，OI明显高于M组和C组；在T2、T3、T4、T5时间U组的IL-6和IL-8浓度明显低于M组和C组。结论体外循环手术中联合应用平衡超滤和改良超滤能改善患儿肺的通气功能和换气功能，并能降低血浆中的炎性因子的浓度，减轻炎性反应，保护肺功能。     

先天性心脏病并肺动脉高压患儿痰液及血液亚硝酸盐/硝酸盐的变化

目的探讨围手术期先天性心脏病(CHD)患儿痰液及血清亚硝酸盐/硝酸盐(NO2-/NO3-)变化及体外循环转流(CPB)和术后、外源性吸入一氧化氮(NO)对肺血管的影响。方法选择CHD并肺动脉高压(PH)患儿36例(A组),CHD未并PH患儿30例(B组),无CHD患者32例(C组)。3组术前及A、B组术后分别测定痰液及血清NO2-/NO3-,A组术后分别经NO吸入及静脉予硝普钠30 min,26、h测定痰液及血液NO2-/NO3-。结果术前痰液及血液NO2-/NO3-A组明显低于B、C组,术后A、B组痰液及血清NO2-/NO3-均减低,吸入NO后痰液NO2-/NO3-增加明显。相关分析示吸入NO后痰液和血液NO2-/NO3-呈明显正相关。结论吸入NO治疗对CHD并PH患儿效果更明显,反映在痰液和血液变化基本一致,对局部肺组织中NO2-/NO3-变化研究,痰液较血清标本更具针对性。     

The effect of corrective surgery on serum IGF-1, IGFBP-3 levels and growth in children with congenital heart disease

The aim of this study is to evaluate growth and insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding protein 3 (IGFBP-3) levels in infants with congenital heart disease (CHD) pre- and postoperatively over a period of a year. Anthropometric values and serum levels of IGF-1 and IGFBP-3 of 40 infants with CHD (20 cyanotic and 20 acyanotic) were compared with 32 healthy controls. Acyanotic infants and infants with pulmonary hypertension (PH) presented significantly more growth failure. Preoperatively, serum IGF-1 and IGFBP-3 levels were lower in the acyanotic group than the cyanotic and the control groups (p = 0.22; p < 0.01). The upward trend in IGF-1 and IGFBP-3 levels in this year-long study demonstrated that the values in the third month and the first year were higher than the preoperative values (p < 0.05). The parallel increase of weight gain and IGF-1, IGFBP-3 levels were the best evidence that these parameters are good nutritional indicators. Timing the corrective surgery before chronic malnutrition or PH develops is an important issue to maintain a normal growth for children with CHD.     

Long-Term Behavior and Quality of Life After Corrective Cardiac Surgery in Infancy for Tetralogy of Fallot or Ventricular Septal Defect

The objective of this study was to evaluate behavior and quality of life in children after corrective cardiac surgery in infancy. Twenty cyanotic (tetralogy of Fallot) and 20 acyanotic children (ventricular septal defect), operated at a mean age of 0.7 years with deep hypothermic circulatory arrest (DHCA) and low-flow cardiopulmonary bypass (CPB), were assessed at a mean age of 7.4 years by the Child Behavior Checklist (CBCL) and the German KINDL. Test results were related to perioperative and neurodevelopmental outcome. Compared to healthy children and not significantly different between the groups, internalizing and externalizing problems were elevated, school performance and total competence were reduced, and self- and parent-reported quality of life was not reduced. Parent-reported problems and reduced physical status were correlated with longer durations of DHCA and CPB. Internalizing and externalizing problems, reduced school competence, and reduced self-esteem were associated with reduced endurance capacity. Externalizing problems were related to reduced gross motor function. Poor school competence was related to reduced intelligence and academic achievement. Children with preoperative hypoxemia in infancy due to cyanotic cardiac defects are not at significantly higher risk for behavioral problems and reduced quality of life than those with acyanotic heart defects. The risk of long-term psychosocial maladjustment after corrective surgery in infancy is increased compared to that for normal children and related to the presence of neurodevelopmental dysfunction.     

Correlation between vascular endothelial growth factor and leptin in children with cyanotic congenital heart disease

The objective in this study was to determine whether there was any relation between leptin and vascular endothelial growth factor (VEGF) in children with cyanotic and acyanotic heart anomalies. The study group consisted of 18 children with cyanotic congenital heart disease (CHD) and 20 age-adjusted children with acyanotic CHD as controls. Serum VEGF and leptin levels were determined by enzyme-linked immunosorbent assay (ELISA). The mean VEGF level was 149.25+/-42.93 pg/ml (range 80.66-217.00) in the cyanotic group and 88.18+/-20.94 pg/ml (range 48.44-112.71) in the acyanotic group (p<0.001). The mean leptin level was 7.55+/-1.46 ng/ml (range 4.08-10.25) in the cyanotic group and 6.89+/-1.43 ng/ml (range 2.67-8.57) in the acyanotic group (p=0.168). There was a significant positive correlation (r=0.723, p<0.001) between VEGF and leptin levels in the cyanotic group while there was no correlation (r=0.235, p=0.348) in the acyanotic group. Arterial oxygen saturation (SaO2) was negatively correlated (r=-0.625, p<0.001) with VEGF, but not correlated with leptin (r=-0.207, p=0.211) in the cyanotic group. There was no correlation between VEGF, leptin and SaO2 in the acyanotic group. We conclude that it is likely that both VEGF and leptin have a role in the pathogenesis of angiogenesis in cyanotic CHD.     

吸入一氧化氮对危重先天性心脏病体外循环婴幼儿肺表面活性物质的影响

目的 探讨吸入一氧化氮(NO)对婴幼儿危重先天性心脏病(先心病)体外循环术(CPB)中肺表面活性物质的影响.方法 将30例室间隔缺损伴重度肺动脉高压的婴幼儿随机分为对照组和NO组,NO组在CPB期间吸入40×10-6NO.CPB前和术后气管插管未拔前0～1 h,1～2 h,2～3 h测定气道压、吸入氧浓度和呼气末二氧化碳浓度,并分别在同时点采动脉血进行血气分析,计算肺泡死腔率(VDhD/VT)、肺泡动脉血氧分压差[P(A-a)O2]、动脉血氧含量(CaO2)和肺泡氧合指数(OI),记录术后呼吸机支持时间.同时各时相点以少量生理盐水灌洗气道,分别测定气道吸出物中总磷脂(TPL)、饱和卵磷脂(SatPC)、总蛋白(TP)值,计算SatPC/TPL和SatPC/TP.结果 与对照组相比,NO组VD/VTP(A-a)O2明显下降(P<0.01),OI、CaO2升高(P<0.01);两组CPB后SatPC/TPL和SatPC/TP较CPB前明显降低(P<0.01).NO组SatPC/TPL和SatPC/TP下降的幅度明显小于对照组(P<0.01).结论 婴幼儿危重先心病CPB中存在明显的肺损害,表现为一些亚临床性肺功能损伤.吸入一定浓度的NO对CPB期间肺功能有保护作用.     

Dynamics of Factor XIII Levels After Open Heart Surgery for Congenital Heart Defects: Do Cyanotic and Acyanotic Patients Differ?

Cardiopulmonary bypass (CPB) reduces coagulation factor levels through hemodilution and consumption. Differences in CPB-induced alterations of factor XIII (FXIII) levels in children with cyanotic and acyanotic congenital heart defects (CHDs) are not well characterized. FXIII activity (determined by Berichrom assay), prothrombin index, activated partial thromboplastin time, and fibrinogen were measured before open heart surgery with CPB and 5 days postoperatively for children older than 3 years with acyanotic (n = 30) and cyanotic (n = 30) CHDs. The preoperative FXIII levels did not differ significantly among the children of the compared groups. The cyanotic patients showed a significantly longer duration of CPB (111.4 ± 45.8 vs 71.5 ± 33.6 min; p = 0.026) and aortic cross-clamp (68.0 ± 27.1 vs 45.4 ± 31.4 min; p = 0.034). The drop in FXIII levels after termination of CPB was more profound for the children with cyanotic CHDs (87.1 ± 13.4 to 49.1 ± 13.2 vs 81.5 ± 12.6 to 58.6 ± 11.1 %, respectively; p = 0.018). The cyanotc patients also were restored to their baseline FXIII levels later than the children with acyanotic CHDs (at 48 vs 24 h). The post-CPB dynamics of the majority of the other coagulation parameters in the compared groups of patients were similar. The cyanotic patients experienced significantly greater postoperative blood loss than the acyanotic patients (12.6 ± 4.9 vs 5.0 ± 2.1 mL/kg; p < 0.001) and were transfused with larger volumes of red blood cells (10.4 ± 6.5 vs 4.2 ± 2.5 mL/kg; p = 0.007). The decrease in FXIII levels after CPB is more profound and lasts longer in children with cyanotic CHDs than in acyanotic patients. The rational strategy of postoperative FXIII replacement therapy for these categories of patients needs to be determined.     

先天性心脏病伴肺动脉高压患儿血清血管内皮生长因子和一氧化氮水平变化的研究

目的 探讨血管内皮生长因子(VEGF)与一氧化氮(NO)在先天性心脏病(CHD)伴肺动脉高压(PH)形成和发展中的作用及二者之间的关系.方法 采用酶联免疫吸附法和分光光度法分别检测25例CHD伴PH(PH组)及16例CHD不伴PH(对照组)患儿静脉血血清VEGF和NO的含量,并进行比较分析.结果 (1)PH组血清VEGF和NO含量明显高于对照组,差异有非常显著性(P＜0.01);(2)PH组血清VEGF和NO含量与平均肺动脉压力呈正相关(r=0.712,P＜0.01;r=0.602,P＜0.01);(3)PH组血清中VEGF与NO含量呈正相关(r=0.801,P＜0.01).结论 VEGF和NO在CHD伴PH的形成和发展中起保护作用;VEGF可能通过NO介导而发挥其血管重构作用.     

Malnutrition and growth failure in cyanotic and acyanotic congenital heart disease with and without pulmonary hypertension.

AIM: To investigate the effect of several types of congenital heart disease (CHD) on nutrition and growth. PATIENTS AND METHODS: The prevalence of malnutrition and growth failure was investigated in 89 patients with CHD aged 1-45 months. They were grouped according to cardiac diagnosis: group aP (n = 26), acyanotic patients with pulmonary hypertension; group ap (n = 5), acyanotic patients without pulmonary hypertension; group cp (n = 42), cyanotic patients without pulmonary hypertension; and group cP (n = 16), cyanotic patients with pulmonary hypertension. Information on socioeconomic level, parental education status, birth weight and nutrition history, number of siblings, and the timing, quality, and quantity of nutrients ingested during weaning period and at the time of the examination were obtained through interviews with parents. RESULTS: There was no significant difference between groups in terms of parental education status, socioeconomic level, duration of breast feeding, and number of siblings (p > 0.05). Group cP patients ingested fewer nutrients for their age compared to other groups. 37 of the 89 patients were below the 5th centile for both weight and length, and 58 of 89 patients were below the 5th centile for weight. Mild or borderline malnutrition was more common in group aP patients. Most group cp patients were in normal nutritional state, and stunting was more common than wasting. Both moderate to severe malnutrition and failure to thrive were more common in group cP patients. CONCLUSION: Patients with CHD are prone to malnutrition and growth failure. Pulmonary hypertension appears to be the most important factor, and cyanotic patients with pulmonary hypertension are the ones most severely affected. This study shows the additive effects of hypoxia and pulmonary hypertension on nutrition and growth of children with CHD.     

两种手术方法对肺动脉闭锁伴室间隔缺损预后影响的探讨

目的对近年来本院实施的肺动脉闭锁伴室间隔缺损(PA/VSD)一期根治术患儿手术方式及右室流出道重建方法进行总结,结合术后院内各项指标进行比较分析;对比同期一期姑息PA/VSD手术的术后生存率、并发症等情况,藉此对一期手术方案的选取进行评价。方法本院2008年1月至2014年3月施行单纯PA/VSD一期根治手术129例为根治组,收集患儿性别、手术时年龄、身高、体重、术前经皮氧饱和度、McGoon、Nakata指数、手术方式、右室流出道重建材料、术中体外循环、主动脉阻断时间、术后置CICU时间、住院时间、术后最大正性肌力药物评分、院内并发症及死亡率情况;另外收集本院120例P/VSD一期姑息手术患儿相应资料作为对比,为姑息组。结果一期根治组早期院内死亡10例,病死率7.8%;术前McGoon指数1.6±0.5,体外循环时间(130.0±45.6)min;主动脉阻断时间(77.2±23.5)min;呼吸机支持时间(87.0±92.8)h;住院时间(22.6±11.4)d。一期姑息组院内死亡11例,病死率9.2%;McGoon指数1.2±0.3,体外循环时间(89.7±46.4)min;呼吸机支持时间(47.4±50.5)h;住院时间(18.6±7.0)d;结论术前McGoon≥1.2,SpO_2≥75%可视为一期根治术的适应证;术中监测RVP/LVP比值是预防术后早期院内死亡的重要方法;对于伴有粗大主肺动脉侧支(MAPCAs)的患儿,提倡采取早期根治手术。     

先天性心脏病并肺动脉高压患儿血浆一氧化氮和硫化氢的变化

目的研究先天性心脏病(CHD)并肺动脉高压(PH)患者血浆一氧化氮(NO)和硫化氢(H2S)的变化及其与PH相关性,探论PH的形成机制,为临床有效治疗PH提供新的思路和理论依据。方法对全部研究对象行彩色超声心动图检查,明确CHD类型并测定肺动脉收缩压。按彩色超声心动图结果分为4组。正常儿童25例,CHD 75例,其中无PH 25例,轻度PH 25例,中、重度肺PH 25例。取CHD组术前静脉血4 mL,迅速分离血浆,采用分光光度法测定血浆NO水平,采用敏感硫电极法测定H2S水平。结果CHD患儿血浆NO水平明显高于对照组,但血浆NO水平增加到一定程度后不再随肺动脉收缩压增加而增加,肺动脉收缩压与血浆NO水平无相关性。CHD并中重度PH组血浆H2S水平明显低于CHD并轻度CHD组,CHD并轻度PH组血浆H2S水平明显低于对照组,肺动脉收缩压与血浆H2S水平呈负相关。结论CHD致PH形成时NO水平升高,代偿性内源性NO上调可能对缓解PH起一定作用。CHD致PH形成时H2S明显降低,内源性H2S下调可能在PH形成中起重要的作用。