肺静脉指数对肺血减少型先天性心脏病肺血管发育的评估

目的 探索能更准确反映肺血管发育及肺血流情况的指标,为外科手术方案的选择提供依据.方法 采用74例肺血减少型先天性心脏病心血管造影序列,测量左右肺动脉及4根肺静脉直径,分别计算Nakata指数,McGoon指数,肺静脉指数(PVI),分别与术后情况进行相关分析.结果 左、右侧肺静脉大小分别与左右肺动脉大小高度相关,左侧肺静脉与左肺动脉远端的相关性为0.73,左侧肺静脉与左肺动脉近端的相关性为0.72,右侧肺静脉与右肺动脉远端的相关性为0.67,右侧肺静脉与右肺动脉近端的相关性为0.71.PVI与术后监护时间,呼吸机维持时间,正性肌力药物用量的相关性分别为-0.51,-0.478和-0.693,均比Nakata指数,McGoon指数明显增高,能更准确的反映整个肺血管的发育情况.右室流出道重建术后无低心排组与低心排组的McGoon指数分别为1.97±0.58与1.36±0.51(t=2.347,P＜0.05),两组Nakata指数分别为(269±124)mm2/m2和(164±106)mm2/m2(t=2.218,P＜0.05),PVI分别为(273±125)mm2/m2和(152±77)mm2/m2(t=2.936,P＜0.01),低心排组肺血管值均明显小于无低心排组.当PVI小于180 mm2/m2时,术后易出现血流动力学不稳定,低心排,甚至死亡.结论 肺动脉、肺静脉发育彼此相关,PVI能更准确反映肺血管发育及肺血流情况的形态学指标,为外科手术方案的选择提供有效依据.     

肺血减少先天性心脏病肺血管发育的评估

通过核磁共振成像、超声心动图、心血管造影、螺旋CT等各种方法测量肺血管大小、计算肺动脉总干／主动脉比值、McGoon指数－即左肺动脉（LPA）与右肺动脉（RPA）直径之和／降主动脉直径、Nakata指数（LPA、RPA近第一支分支处血管截面积相加／体表面积）,对各类肺血减少先天性心脏病手术预后进行评估。肺血管大小与右心室／左心室峰压比、肺血管顺应性密切相关,并可影响肺血管阻力,McGoon指数、Nakata指数、PVI指数与术后血液动力学、术后低心排的发生、术后生存率高度相关。     

多排螺旋CT在小儿法乐四联症诊治中的应用价值

目的 探讨多排螺旋CT血管造影(MUCTA)三维重建应用于小儿法乐四联症(TOF)诊断及治疗中的价值.方法 2004年12月至2008年12月38例经胸二维超声心动图(TEE)诊断TOF患儿,为进一步明确肺动脉发育状况行MUCTA检查,均经外科手术证实.MUCTA扫描后应用三维重建处理,测算全肺动脉指数(Nakata),确定肺动脉发育状况,冠状动脉走向,主、肺动脉侧枝循环位置,设计手术方案.结果 38例患儿检查过程顺利均为TOF,其中合并房间隔缺损(ASD)3例、动脉导管未闭(PDA)2例、室间隔缺损(VSD)嵴下型38例,单纯右室流出道狭窄7例、合并肺动脉狭窄31例,冠状动脉走向异常2例、主、肺动脉侧枝循环19例、其中粗大侧枝循环3例.35例康复出院,3例术后死亡.结论 MUCTA对于诊断TOF心外畸形的显示清晰度超过其它影像学检查,特别是对观察肺动脉发育形态、肺动脉狭窄的部位、冠状动脉起源与走向异常、PDA、主肺动脉侧枝循环,精确测算Nakata指数、McGoon指数均有明显帮助.与TEE相结合能达到优势互补、能够更加完整、准确的诊断TOF,为手术决策,方案的制定提供了有效的依据,对提高TOF患儿的治疗效果有重要价值.     

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。     

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目的研究与法洛四联症(TOF)一期根治手术早期死亡相关的危险因素。方法回顾1995年4月至2004年3月在复旦大学附属儿科医院心血管中心行一期根治术的TOF病例152例,术后早期死亡17例,对其术前、术中27个可能的危险因素指标与术后早期死亡的关系进行单因素和多因素分析。结果单因素分析结果显示与术后早期死亡有关的指标包括手术时体重、发生青紫年龄、动脉血氧饱和度、升主动脉与肺动脉干直径比AO/MPA、McGoon比值、Nakata指数、肺体循环血流量之比Qp/Qs、右向左分流量占体循环血流量比例(QseQp)/Qs、体肺侧支循环和跨瓣补片。多因素分析结果显示AO/MPA和McGoon比值与手术早期死亡有关。结论肺动脉的发育情况是TOF一期根治术后能否生存的关键。手术病例的选择需要综合考虑多种危险因素。     

肺血减少先天性心脏病肺血管发育的评估

通过核磁共振成像、超声心动图、心血管造影、螺旋ＣＴ等各种方法测量肺血管大小,计算肺动脉总干／主动脉比值、ＭｃＧｏｏｎ指数——即左肺动脉（ＬＰＡ）与右肺动脉（ＲＰＡ）直径之和／降主动脉直径、Ｎａｋａｔａ指数（ＬＰＡ、ＲＰＡ近第一支分支处血管截面积相加／体表面积）、ＰＶＩ指数（所有肺静脉入心房近端处的截面积相加／体表面积）,对各类肺血减少先天性心脏病手术预后进行评估。肺血管大小与右心室／左心室峰压比、肺血管顺应性密切相关,并可影响肺血管阻力。ＭｃＧｏｏｎ指数、Ｎａｋａｔａ指数、ＰＶＩ指数与术后血液动力学、术后低心排的发生、术后生存率高度相关。     

婴幼儿法乐四联症手术治疗近期疗效的影响因素

目的 探讨影响婴幼儿法乐四联症手术治疗近期疗效的各相关因素.方法 将2003年8月至2011年2月经作者一期手术纠治的117例年龄≤3岁的法乐四联症患儿分为疗效良好、疗效较差两组.分析手术时患儿年龄、体重、术前HCT值、McGoon指数、EDVI、主动脉阻断时间、转流时间、室间隔缺损大小、升主动脉与肺动脉干直径比、...     

肺动脉发育不良型婴幼儿法乐四联症的手术指征及策略

目的 探讨肺动脉发育不良型婴幼儿法乐四联症手术适应证.方法 回顾性分析2009年1月至2013年4月间行手术治疗的98例法洛四联症患儿(McGoon≤1.2)的临床资料,收集与肺动脉关联数据,根据术式分为根治术组和体肺分流组.根据影像学资料,分别测定McGoon指数(MI)、左下肺动脉直径/左肺动脉近端直径(LBPA/LPA1)、左下肺动脉直径/左肺动脉远端直径(LBPA/LPA2)、左右下肺动脉直径之和/左右肺动脉近端直径之和(MBPA/MPA1)、左右下肺动脉直径之和/左右肺动脉远端直径之和(MBPA/MPA2)、右下肺动脉/气管(RBPA/TR)及临床数据;通过统计学分析,发现MBPA/MPA1在体肺分流组明显低于根治组,差异有统计学意义(P =0.004),按照MBPA/MPA1 =0.70为界结合McGoon指数将病例分为根治组(82例)、体肺分流组(16例)比较实际病死率.结果 根治组死亡6例,经皮血氧饱和度由术前(73.3±10.2)％升至(95.6±1.8)％,血红蛋白由术前(149.4±30.1)g/L降至(121.6±8.0)g/L.体肺分流组死亡2例,经皮血氧饱和度由术前(67.4±11.8)％升至(84.6±2.2)％,血红蛋白由术前(158.6±32.7)g/L降至(120.8±11.6)g/L,MI由1.0±0.2升至1.2±0.1.LBPA/LPA1在体肺分流组为0.6±0.1,明显低于根治组0.8±0.1;MBPA/MPA1在体肺分流组为0.6±0.1,明显低于根治组0.8±0.1,差异均有统计学意义(P =0.004);MI和MBPA/MPA1相结合的分组的实际根治病死率明显降低.结论 对于大多MI≤1.2的肺动脉发育不良型法乐四联症患儿可早期行法乐四联症根治术.MBPA/MPA1可能可以更好的反映肺内血管发育情况,而MI和MBPA/MPA1相结合能为法乐四联症根治手术适应证的选择提供重要依据.     

中央体肺分流姑息手术治疗肺血减少型复杂先天性心脏病

目的 总结中央体肺分流姑息手术在复杂先天性心脏病治疗中的应用效果.方法 对11例肺血减少型复杂先天性心脏病患儿实施中央分流姑息手术,患儿术前均明显发绀,超声McGoon指数0.74～1.01(0.90±0.22);术中选取直径4～6 mm的Gore-Tex人工血管,采用4-0或5-0的prolene滑线,选择连续缝合法将人工血管与肺动脉和升主动脉进行端侧吻合,比较手术前后动脉血氧饱和度的变化,并进行术后随访,分析该术式的疗效.结果 本组无死亡病例,患儿术后动脉血氧饱和度较术前显著升高(0.78±0.12 vs 0.89±0.10,t=2.637 P<0.05).分流血管通畅,无血栓形成和闭塞,左右肺动脉均扩大,患儿活动量均较术前有所增加.结论 中央体肺分流姑息手术损伤小、操作简单,手术病死率低,可为根治肺血减少型复杂先天性心脏病提供较好的基础.     

法乐四联症伴肺动脉闭锁的外科治疗

目的：通过回顾总结对法乐四联症伴肺动脉闭锁的外科治疗经验，着重讨论手术适应证。方法：根据患称肺动闭锁范围及分支发育情况，左右肺动脉与降主动脉直径比、左右肺动脉截面积与体表面比、术后２４小时测定右室与左室压力来选择手术方法，手术方法分根治术和姑息术。结果：２８例患儿中，１８便行     

Intact Right Ventricle–Pulmonary Artery Shunt After Stage 2 Palliation in Hypoplastic Left Heart Syndrome Improves Pulmonary Artery Growth

For patients with hypoplastic left heart syndrome who have undergone the Norwood procedure with a right ventricle–pulmonary artery (RV-PA) shunt, the shunt can either be removed or left intact at the time of the stage 2 procedure. This study aimed to determine the effects of an intact shunt on pulmonary artery growth and clinical outcomes after the stage 2 procedure. A retrospective review of patients who underwent Norwood with an RV-PA shunt from 2005 to 2010 was performed. Catheterization data, echocardiographic data, postoperative outcome variables, and mortality data were collected. Pulmonary artery size was measured at pre-stage 2 and pre-Fontan catheterizations using the Nakata Index and the McGoon Ratio. Of the 68 patients included in the study, 48 had the shunt removed at the time of stage 2 (group 1), and 20 had the shunt left intact (group 2). The two groups did not differ in terms of pre-stage 2 hemodynamics or pulmonary artery size. After stage 2, group 2 had higher oxygen saturations. The two groups did not differ regarding duration of chest tube drainage, length of hospital stay, need for unplanned interventions, or mortality. Before Fontan, the group 2 patients had higher superior vena cava (SVC) pressures and more venovenous collaterals closed. There was increased pulmonary artery growth between the pre-stage 2 and pre-Fontan catheterizations in group 2 using both the Nakata Index (+148.5 vs ?52.4 mm²/m²; p = 0.01) and the McGoon Ratio (+0.36 vs +0.01; p = 0.01). These findings indicate that patients with an intact RV-PA shunt after stage 2 have greater pulmonary artery growth than patients with the shunt removed, with no increased risk of complications.     

An Angiographic Predictor of Pulmonary Artery Stenosis After the Norwood-Sano Operation for Hypoplastic Left Heart Syndrome

The objective of this study was to determine angiographic predictors of future pulmonary artery stenosis (PS) in patients with hypoplastic left heart syndrome (HLHS) at the time of pre-stage 2 cardiac catheterization (PS2C). The Sano modification of the Norwood operation (NSO) for HLHS includes placement of a right ventricle-to-pulmonary artery (RV-PA) conduit. Branch PS is a recognized complication. Data from patients with HLHS who underwent NSO from 2005 to 2009 and who underwent PS2C were reviewed retrospectively. Nakata and McGoon indices were calculated in the traditional fashion, and modified Nakata and McGoon indices were calculated using the narrowest branch PA diameters. Thirty-three patients underwent NSO and 28 patients underwent PS2C. Mean follow-up was 35.8?±?7.5?months. Ten (36?%) patients had significant left branch PS, with two requiring balloon angioplasty and eight requiring stent placement, a median of 15.2?months after PS2C (interquartile range 1.2, 32.8). The modified Nakata index was predictive of future intervention for left PS (receiver operating characteristic curve area under the curve 0.811), with a cut-off of 135?mm²/m² and a sensitivity of 100?% and specificity of 72.2?%. A modified Nakata index <135?mm²/m² at PS2C predicts future need for intervention on left-branch PS in patients with HLHS after the NSO. Surgical pulmonary arterioplasty at the time of stage 2 surgical palliation may obviate the need for future interventions.     

Short-Term Result of Ductus Arteriosus Stent Implantation Compared With Surgically Created Shunts

This report aims to compare the researchers?? early experience with the safety, efficacy, short-term outcomes, and complications of patent ductus arteriosus (PDA) stents in neonates having duct-dependent pulmonary circulation with those of surgically created shunts. Between April 2009 and April 2011, 18 infants with duct-dependent pulmonary circulation underwent cardiac catheterization for PDA stenting as the first palliative procedure in a referral center. For comparison, 20 infants who underwent surgical aortopulmonary shunt placement in another center were used. Follow-up assessment included clinical examination, echocardiography, oxygen saturation, and cardiac catheterization studies. Access and stenting for the PDA were successful in 15 patients (83.3?%). The mean procedure time was 58.43?±?41.25?min, and the mean fluoroscopy time was 18.81?±?5.64?min. Three patients (20?%) in the stented group and 6 patients (30?%) in the surgical group died (P?=?0.09). After a 6-month follow-up period, none of the patients had significant stent stenosis requiring reintervention. The oxygen saturation increase did not differ significantly between the two groups either immediately after the procedure or 6?months later (P?>?0.5). The left pulmonary artery diameter, McGoon ratio, and Nakata index did not differ significantly between the two groups (P?>?0.05), but the right pulmonary diameter was larger in the stented group (5.01?±?0.45 vs 4.1?±?0.49?mm; P?=?0.0001). Stenting for the PDA is an appropriate alternative to surgical shunt creation in many patients with duct-dependent circulation. In our sample, the two groups did not differ significantly in terms of outcome or mortality.     

手术治疗婴幼儿法洛四联症83例

目的 总结婴幼儿法洛四联症（TOF）的外科手术情况,初步探讨手术时机、术式及与近期疗效的关系,以提高手术矫治效果.方法 2003年8月至2008年5月本院共手术矫治年龄≤3岁的TOF患儿83例（86次）.年龄2～36个月,体重3.5～13 kg.17例有反复缺氧发作,1例术前需呼吸机支持.患儿术前均行心脏彩超检查,1例行心血管造影.75例采取一期根治手术,68例经右房修补室间隔缺损,62例跨肺动脉瓣扩大右室流出道.根据Mcgoon指数以及左心室发育情况,8例采取姑息治疗（中央性体肺分流）,其中3例于11～15个月后行二期根治手术.结果 患儿McGoon指数≥1.28时预后良好.全组存活79例,根治、姑息治疗后各死亡2例,病死率4.7%（4/86）;二期根治患儿中无一例死亡.术后随访3～62个月,无死亡病例.结论 TOF患儿婴幼儿期采取一期根治可获得满意的近期效果,少数重症患儿仍需分期矫治.     

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目的分析法洛四联症(TOF)根治术后急性肺损伤(ALI)的原因,总结治疗体会。方法2003年7月至2004年6月,上海第二医科大学附属新华医院上海儿童医学中心心胸外科共行TOF根治术161例,发生ALI5例。记录5例ALI患儿术前经皮氧饱和度(SpO2)、红细胞压积比(HCT)、McGoon比值、肺动脉指数(PAI)和手术、体外循环(CPB)资料,以及ALI发生时间、ALI发生后各项治疗措施、各治疗阶段肺功能指标、计算其肺损伤分数。结果5例ALI患儿,年龄7～24(13.8±3.1)个月,体重7.0～9.5(8.2±0.5)kg,均在静吸复合麻醉CPB下进行,术毕给予改良超滤,平均CPB时间(67.2±5.9)min,主动脉阻断时间(43.6±2.4)min,ALI发生率为3.1%,发生时间为术后8～60h,死亡2例。5例均施行腹膜透析术(PD),2例给予一氧化氮吸入,1例给予肺表面活性物质替代治疗。各治疗阶段肺功能指标逐步改善,至治疗后期,肺功能指标在统计学上有显著性差异(P<0.05)。结论TOF术后ALI治疗困难,合理机械通气、尽早维持体液平衡、改善肺通气和氧合功能有利于及时阻断低氧酸中毒导致的恶性循环。