小婴儿重症主动脉缩窄经皮球囊扩张成形术治疗6例随访观察

目的 分析经皮球囊扩张成形术在小婴儿重症主动脉缩窄中的即刻治疗效果及6~49个月的随访情况。方法 对2006至2009年于复旦大学附属儿科医院施行经皮球囊扩张成形术的重症主动脉缩窄的<6月龄患儿行主动脉形态学和血液动力学指标的评价,于术后~2周、~6个月、~12个月及之后每年1次临床随访,随访内容包括股动脉损伤、主动脉再狭窄、左室收缩功能和是否有动脉瘤形成。结果 重症主动脉缩窄行经皮球囊扩张成形术6例患儿中,男4例,女2例;年龄32~153 d,平均（91±44）d;体重3.0~6.0 kg,平均（4.7±1.0）kg。6例患儿术前均表现为不同程度心功能不全,2例出现心源性休克合并严重代谢性酸中毒。经皮球囊扩张术后即刻,导管测量缩窄前后压力阶差由术前的（46±11）mmHg（1 mmHg=0.133 kPa）降至术后的（14±7）mmHg,5例术后跨缩窄压差均＜20 mmHg,1例极重度主动脉缩窄患儿由术前的76 mmHg降至术后的32 mmHg,下降幅度＞50%,6例均即刻成功。经皮球囊扩张术后主动脉缩窄部直径由术前的（2.18±0.48）mm扩大至术后的（4.00±0.83）mm,1例患儿术后有动脉瘤形成。随访6~49个月,6例患儿临床心功能不全症状均消失;2例因主动脉再狭窄行第2次经皮球囊扩张术;1例并发动脉瘤患儿在随访18个月后动脉瘤自行消失。结论 对重症主动脉缩窄合并左心功能不全的<6月龄患儿,经皮球囊扩张成形术可作为一种安全有效的替代外科手术的治疗方法,但其远期疗效及并发症尚有待进一步随访。     

婴幼儿室间隔缺损合并肺动脉高压的外科治疗

目的 总结婴幼儿大型室间隔缺损（Ventricular Septal Defects,VSD）合并肺动脉高压的外科治疗经验.方法 2008年10月至2009年5月共收治婴幼儿大型VSD 98例,男56例,女42例,年龄4～24个月,体重5.1～15.3 kg;均为单发,其中合并房间隔缺损（Atrial Septal Defect,ASD）26例,合并三尖瓣关闭不全（Tricuspid Insufficiency,TI）18例,动脉导管未闭（Patent Ductus Arteriosus,PDA）12例.患儿均在全麻低温体外循环下实施一期矫治术.结果 治愈95例,死亡3例,术后并发症16例,其中低心排出量综合征6例,肺不张4例,肺炎3例,心律紊乱2例,气胸1例,术后早期复查彩超,除5例有少量残余分流外,其余患儿心功能及瓣膜关闭均正常,患儿均痊愈出院.结论 低体重、低年龄的大型室间隔缺损应尽早手术根治;术中尽量减轻体外循环损伤,保护脏器功能;术后加强呼吸道管理、预防低心排出量综合征和肺动脉高压危象是降低死亡率的关键.     

肺动脉压监测在幼婴室间隔缺损并中、重度肺高压修补术中的意义探讨

目的 探讨幼婴先天性心脏病间隔缺损（ＶＳＤ）合并肺动脉高压手术前,后肺动脉压力（ＰＡＰ）改变与预后的关系。方法 １９９４年１月－１９９８年１２月我科施行幼婴ＶＳＤ并中,重度肺动脉高压心内直视手术７０例,对比缺损修补前,后ＰＡＰ改变,持续监测其中３５例术后ＰＡＰ变化。结果 术前未作心志管检查者,术中测ＰＡＰ可初步判断其肺高压程度。     

儿童大型动脉导管未闭介入封堵术治疗的临床效果

目的：探讨Amplatzer法介入治疗大型动脉导管未闭(PDA)的临床效果。方法：227例大型PDA患儿,其中合并肺动脉高压者63例,应用Amplatzer封堵器行动脉导管封堵术,年龄中位数为3.2岁,体重中位数为10.6 kg,PDA最窄处直径中位数5.7 mm。结果：227例患儿中,216例封堵成功（95.2％）。合并肺动脉高压者治疗前肺动脉平均压为45±19 mm Hg,封堵术后降为22±12 mm Hg（P<0.05）。216例封堵成功患儿中,术后造影显示即刻完全封堵109例（50.5％）,术后24 h超声心动图检查完全封堵者181例（83.8％）。术后6个月和12个月超声心动图检查示封堵成功的患儿均无残余分流。结论：Amplatzer法介入封堵治疗儿童大型PDA是一种安全有效的方法。     

先天性心脏病重度肺动脉高压性质的综合评价

目的探讨先天性心脏病（简称先心病）合并重度肺动脉高压患儿器质性肺动脉高压（简称肺高压）的诊断标准。方法３７例经手术治疗后早期肺动脉压力降至正常的患儿作为动力性肺高压组；７例经手术治疗后仍持续性肺动脉高压及６例临床诊断为器质性肺高压而未予手术的共１３例患儿作为器质性肺高压组，对比两组心导管检查血液动力学指标。结果两组肺血管阻力、肺小动脉楔压、肺循环血流量与体循环血流量之比（Ｑｐ／Ｑｓ）及降主动脉血氧饱和度（ＳａＯ２）差异均有显著意义，如按年龄大于２岁、肺动脉阻力＞７２ｋＰａ·ｓ－１·Ｌ－１（９Ｗｏｏｄ单位）、肺小动脉楔压≤１．６ｋＰａ（１２ｍｍＨｇ）、Ｑｐ／Ｑｓ＜２和动脉血氧饱和度＜０．９０作为临床诊断器质性肺高压的指标，则本组动力性肺高压组仅有５．４％的患儿符合上述指标３项或３项以上，而器质性肺高压组所有病例均符合上述指标３项或３项以上。结论先心病合并重度肺动脉高压存在上述５项指标中３项或３项以上，高度提示患儿存在器质性肺动脉高压     

婴幼儿室间隔缺损合并肺动脉高压的麻醉处理

目的探讨婴幼儿先天性心脏病室间隔缺损合并肺动脉高压行室缺修补术的麻醉处理要点。方法室间隔缺损伴肺动脉高压的婴幼儿86例,均在静吸复合全麻、低温体外循环下行室缺修补术。全组持续监测有创血压(ABP)、CVP、ECG、SpO2、体温、尿量、电解质、血气,术中加测右房压(RVP)、平均肺动脉压(PAP)。结果本组体外循环时间27min～114min,主动脉阻断时间15min～87min,74例自动复跳;12例电击除颤后复跳;除1例外,肺动脉压力均较术前下降;术后并发低心排综合征12例;术后出现心律失常2例;无1例出现肺动脉高压危象。结论麻醉前做好充分准备,提高患儿耐受能力;麻醉诱导平顺,配合术者尽快建立体外循环;麻醉深度适宜,维持血流动力学平稳,是保证肺动脉压不进一步增高的重要因素;合理应用血管活性药物,防治肺动脉高压危象鸦积极防范及处理术后并发症,可使患儿安全度过手术期。     

先天性心脏病伴肺动脉高压患儿围术期中性粒细胞明胶酶相关脂质运载蛋白水平的变化

目的：探讨先天性心脏病（CHD）合并肺动脉高压患儿手术前后中性粒细胞明胶酶相关脂质运载蛋白（NGAL）水平的变化及意义。方法：将80例CHD患儿按其肺动脉收缩压分为4组：无肺动脉高压组及轻、中、重度肺动脉高压组。测定各组术前、术后即刻、术后24 h血清NGAL含量,分析血清NGAL含量与肺动脉高压及早期预后的关系。结果：CHD伴轻、中、重度肺动脉高压患儿血清NGAL含量均高于不伴肺动脉高压组,血清NGAL浓度随着肺动脉高压程度的加重而升高（P<0.01）。各组患儿术后血清NGAL含量均较术前降低（P<0.01）。血清NGAL含量与肺动脉高压的程度、重症监护病房滞留时间呈正相关（P<0.01）。结论：CHD所致肺动脉高压时,血清NGAL浓度增高;手术中止异常分流后,血清NGAL浓度逐渐降低。血清NGAL水平或可作为衡量肺动脉高压程度和评价手术效果的血清学参考指标。     

婴儿肺动脉瓣狭窄的介入治疗

目的　总结经皮球囊肺动脉瓣成形术（PBPV）治疗婴儿肺动脉瓣狭窄（PS）的经验,评价其必要性、安全性和有效性。方法　回顾性分析2009年1月至2018年12月青岛市妇女儿童医院心脏中心行PBPV治疗68例婴儿PS的临床资料。结果　患儿平均手术年龄为（6.34±3.6）个月,平均手术体重为（7.81±2.11） kg,术前体重/年龄Z值为-0.80±1.38,提示生长受限为22例（32.4%）,合并紫绀15例（22.1%）,呼吸急促23例（33.8%）。超声心动图评估右室长径/左室长径比值平均值为0.56±0.73,胸部正位片心胸比平均值为0.56±0.43,术前血氧饱和度平均值为94.74%±6.10%。术前右室/左室收缩压力比值平均值为0.68±0.26。球囊扩张后,肺动脉瓣平均压力梯度由（75.57±18.61） mmHg降至（22.37±5.21） mmHg,右心室收缩压平均值由（83.78±22.44） mmHg降至（54.16±20.81） mmHg。手术即刻成功率为100%。术中无严重心律失常、心腔穿孔、腱索断裂、血管损伤等并发症。术后早期患儿轻-中度肺动脉瓣反流比例为53.3%,无重度反流。随访3个月～10年间再狭窄率2.94%,32例随访5年以上患儿肺动脉瓣反流程度未进一步加重,无特殊处理。所有患者均未出现明显的右心室功能障碍。与接受PBPV治疗幼儿PS相比,婴儿PS临床症状明显,病情较重,术后早期及中远期随访手术效果满意。对本研究婴儿PS进行分组比较分析发现低体重、低龄儿可获得同样良好手术效果。结论　PBPV治疗婴儿肺动脉瓣狭窄十分必要,技术操作安全可行,随访结果持续有效。     

209例体重5kg以下婴儿先天性心脏病的外科治疗及围术期处理

目的总结本院体重5kg以下婴儿先天性心脏病的外科治疗效果及围术期处理方法。方法2002年11月至2008年2月本院手术治疗5kg以下先天性心脏病患儿209例，男162例，女47例。年龄10d至10个月，其中年龄〈28d9例，28d至3个月110例，3-10个月90例。体重2．3～5妇，其中2．3～3kg10例，3～5kg199例。病种包括：法洛氏四联症（TOF）8例，完全性房室间隔缺损（CAVSD）6例，右室双出口（DORV）9例，完全性肺静脉异位引流（TAPVC）19例，完全性大血管转位（TGA）9例，主动脉弓中断（IAA）2例，室间隔缺损（VSD）和（或）房间隔缺损（ASD）156例，其中部分伴动脉导管未闭（PDA）和（或）肺动脉高压（PH）。全组均在体外循环下行心内直视手术治疗，术后均给予改良超滤，2例延迟关胸，8例放置临时起搏导线，术中及术后依据病情予米力农、多巴胺、异丙肾上腺素等持续静脉滴注，呼吸机支持时间20．5—362．0h，撤机后全部用鼻塞式持续气道正压给氧。结果本组早期（术后30d内）死亡16例（7．9％），死亡原因包括低心排出量综合征、急性肾功能衰竭、肺高压危象、多器官功能衰竭等；术后主要严重并发症包括毛细血管渗漏综合征、低心排出量综合征、肺高压危象、肾功能衰竭、低氧血症等。193例存活者随访12个月，172例肺炎及心力衰竭的发生明显减少，体重明显增加，无远期死亡及并发症发生。结论低体重先天性心脏病患儿手术风险大，应严格把握手术指征及手术时机，重视和改进围术期各个环节，可有效降低手术死亡率和术后并发症。     

主肺动脉窗25例临床分析

目的 分析主肺动脉窗（APW）的发病、分型和伴发畸形情况,总结诊断及治疗经验。方法 回顾性分析复旦大学附属儿科医院2003至2013年诊治的25例APW患儿的临床症状、病理分型、合并畸形、诊断、治疗和预后资料。结果 25例APW患儿进入分析,男14例,女11例,年龄5 d至13岁（6月龄以下12例）,占同期住院先天性心脏病患儿的0.18%。Ⅰ型5例（20%）,Ⅱ型16例（60%）,Ⅲ型1例（4%）,Ⅴ型3例（12%）。单纯APW 10例,APW合并单纯卵圆孔未闭3例;余12例（48.0%）均合并其他需纠正的心血管畸形,其中合并单纯房间隔缺损（ASD）1例,单纯动脉导管未闭（PDA）2例,单纯室间隔缺损(VSD)3例,右室双出口和ASD 1例,主动脉瓣狭窄和右位主动脉弓1例,肺动脉瓣闭锁伴VSD、右位主动脉弓、永存左上腔静脉1例, 主动脉弓离断及PDA 3例。超声心动图诊断17例,误诊或漏诊8例（32%）中5例通过心导管检查及造影诊断,3例外科术中诊断。25例均有不同程度肺动脉高压,其中2例心导管检查评估为阻力性肺动脉高压,4例家长放弃诊治,其余19例施行一期根治术,术后死亡2例（10.5%）。17例术后随访3个月至9年3个月,平均（23±27）个月,其中15例临床无症状,复查超声心动图APW修补无残余,肺动脉压力逐渐下降至正常。结论 APW缺损主要发生在主动脉远端,常伴有间隔缺损、PDA和主动脉弓病变等;超声心动图为首选检查方法,但必要时需行心导管检查及造影。APW患儿易早期发生肺动脉高压,一经诊断尽早手术,可获得较好的中长期疗效。     

复杂畸形伴肌部多发室间隔缺损的直视镶嵌封堵

目的 介绍复杂畸形伴肌部多发室间隔缺损心内直视镶嵌封堵的经验.方法 全组10例,男7例,女3例.复杂心内外畸形包括右心室双出口Taussig-Bing畸形2例、右心室双出口主动脉瓣下室缺2例、完全性大动脉错位伴室间隔缺损肺动脉高压2例、法乐四联症1例、室间隔缺损伴重度二尖瓣反流2例、室间隔缺损伴主动脉重度缩窄1例.其他伴发畸形有:二尖瓣反流、单支右冠状动脉畸形、房间隔缺损、冠状窦隔缺损、主动脉弓发育不良、动脉导管未闭等.全组均解剖纠治镶嵌心内直视封堵肌部多发室间隔缺损,平均手术年龄(8.8±5.4)个月,平均手术体重(6.3±2.0)kg.结果 手术无死亡.无心内直视镶嵌封堵相关并发症.3例术后并发肺动脉高压危象;1例术后3 d延迟关胸;1例拔肺动脉测压管出血,心包填塞,床边开胸止血.随访无死亡,2例封堵器下缘残留少量分流.结论 心内直视封堵肌部多发室间隔缺损,可避免传统手术纠治需心室切口、心室内肌束切开,对心功能影响大的缺点;可缩短体外循环时间,同时镶嵌复杂畸形Ⅰ期解剖纠治,使手术简化、安全、尤其适用于婴幼儿,可获得较佳中长期疗效.

Abstract：

Objective To present our experience using hybrid approaches to repair muscular ventricular septal defects (mVSDs) associated with additional congenital heart diseases in children.Methods From April 2006 to September 2010, 10 patients with mVSDs associated with additional congenital heart diseases underwent corrective surgery at this center. Their mean age was 8. 8 month old, and their mean body weight was 6. 3 kg. The additional congenital heart defects included 2 patients with D-transposition of great arteries, 2 with double outlet right ventricle-Taussig-Bing anomaly, 1 with Tetralogy of Fallot, 2 with double outlet right ventricle, 1 with severe coarctation of aorta,and 2 with severe mitral valve regurgitation. Results All patients underwent the single stage hybrid approach repair under direct vision and all recovered from surgery. After surgery, 3 patients developed pulmonary hypertension crisis among whom 1 had delayed chest closure and 1 underwent emergence surgery to relief cardiac tapenade. The patients were followed up for 3 months to 3 years No late deaths were noted. Complete closure of muscular ventricular septal defects was achieved on 5 patients.Clinically insignificant residual shunt was observed on 2 patients. Conclusions It is safe and effective using hybrid approaches to repair muscular ventricular septal defects (mVSDs) associated with additional congenital heart diseases in children.     

婴儿先天性心脏病1387例外科治疗结果分析

目的 回顾性分析我院近11年来外科治疗的≤6个月小婴儿先天性心脏病(先心病)1387例,探讨其病种、手术时机和治疗观念的变迁,以期进一步提高小婴儿先心病的就诊和救治率.方法 1997年1月至2007年12月,在我院行手术治疗的≤6个月先心病患儿1387例,主要病种包括:室间隔缺损合并肺动脉高压(VSD/PH)、法洛四联症(TOF)、完全性大动脉转位(TGA)、完全性肺静脉异位引流(TAPVC)、主动脉缩窄或主动脉弓中断合并室间隔缺损[CoA(IAA)/VSD]、右心室双出口(DORV)、合并室间隔缺损的肺动脉闭锁(PA/VSD)、室间隔完整的肺动脉闭锁(PA/IVS)等,根据病情采取相应的手术方法矫治,部分复杂型先心病进行了随访.结果 手术死亡110例,总手术死亡率7.9%.从历年手术治疗分析,手术死亡率1997至2003年为11.5%～14.4%,2004至2005年降至8.6%～&9%,2006至2007年降至3.3%～3.8%.对TGA、TAPVC、TOF、PA/VSD、PA/IVS患儿进行了随访,随访率分别为83.8%(98/117)、87.8%(79/90)、48.2%(68/141)、65%(13/20)和95%(19/20),随访期限为3～86个月.晚期死亡16例.随访中绝大多数患儿无症状,心功能和生长发育正常.结论 绝大部分早期出现症状的危重先心病可以在小婴儿期进行矫治,手术效果接近国际水平.不能进行一期矫治的可以先做姑息手术,改善缺氧、促进肺动脉发育,为以后的根治手术创造条件.     

先天性心脏病肺高压患儿血浆肾上腺髓质素浓度的测定及其临床意义:附33例报告

用特异性放射免疫非平衡法测定左向右分流型先天性心脏病 (CHD)患儿血浆肾上腺髓质素 (AM )水平 ,并观察心脏手术后AM的动态变化。结果显示 :CHD肺高压组AM明显高于无肺高压组及正常对照组 (P<0.05 ,P<0.01)。且AM随着肺高压病情加重而升高 ;中、重度肺高压组AM高于轻度肺高压组 ,差异有显著性 (P<0.01)。肺高压组术后AM明显下降 ,与术前比较差异有显著性 (P<0.05 ,P<0.01)。CHD组患儿AM与肺动脉收缩压 (PASP)呈显著正相关 (r=0.77,P<0.05)。本资料表明 ,AM参与了左向右分流型CHD合并肺高压的病理生理过程 ,AM的升高是机体的一种防御性反应 ,对维持肺循环稳定 ,减缓肺高压的发展有积极作用     

儿童先天性心脏病血清Apelin水平与肺动脉压关系的初步探讨

目的 初步探讨先天性心脏病患儿血清Apelin 水平与肺动脉压的关系.方法 手术治疗的先心病患儿126 例,检测患儿术前及术后第7 天的血清Apelin 水平.建立体外循环前检测并计算肺动脉收缩压/体循环收缩压(Pp/Ps)的比值,依据Pp/Ps 分组:无肺动脉高组压(PAH)组、轻度PAH 组、中度PAH 组和重度PAH 组;术后第7 天超声心动图估测肺动脉平均压(PAMP).结果 无PAH,以及轻、中、重度PAH 各组术前及术后的血清Apelin 水平依次降低,差异有统计学意义(PPr=-0.51,-0.54,P结论 先心病患儿并发肺动脉高压及其发展与血清Apelin 水平降低有关系,血清Apelin 对诊断先心病患儿是否并发肺动脉高压及其程度的意义值得深入研究.     

Nitric oxide-related compounds in patients with congenital heart defects and pulmonary hypertension

BACKGROUND: The relationship between blood levels of nitric oxide (NO)-related compounds in patients with congenital heart defects (CHD) and degree of pulmonary hypertension (PH) has not yet been described. METHODS: Thirty-six patients (aged 6 months to 19 years) with CHD were divided into three groups on the basis of their hemodynamic characteristics: group 1 (control, n = 5), left-to-right shunt (-) without PH (pulmonary to systemic artery peak pressure ratio, Pp/Ps < 0.4); group 2 (n = 14), left-to-right shunt (+) without PH; group 3 (n = 17), left-to-right shunt (+) with PH (Pp/Ps > 0.4). Blood samples were obtained from the right atrium, pulmonary artery, left atrium or pulmonary capillary wedge and aorta during cardiac catheterization. All NO-related compounds in whole blood were measured by chemiluminescent assay using Sievers NO analyzer. RESULTS: The sampling site had no significant impact on NO-related compound levels. However, the patients with PH (group 3) had significantly higher (P < 0.01) blood levels of NO-related compounds (117.3 +/- 14.7 mumol/L) than the patients without PH (groups 1 and 2, 23.9 +/- 3.2 and 38.4 +/- 4.8 mumol/L, respectively). In addition, pulmonary artery resistance (Rp) values of less than 6 Wood U/m2 were directly correlated with levels of NO-related compounds, whereas Rp values greater than 6 Wood U/m2 were inversely correlated with blood levels of NO-related compounds. CONCLUSION: The present results suggest that the hemodynamic status of the pulmonary circulation in CHD affects, at least partly, blood levels of NO-related compounds.     

Increased turnover of serotonin in children with pulmonary hypertension secondary to congenital heart disease

Serotonin (5HT) is a potent vasoconstrictor of the pulmonary vascular bed and may be involved in the pathophysiology of secondary pulmonary hypertension in children with a left-to-right shunt due to a congenital heart defect. To test this hypothesis we measured the total and free 5HT concentration in blood as well as the urinary excretion of its main metabolite 5-hydroxyin-doleacetic acid (HIAA) in children showing a left-to-right shunt with (n=10) and without (n=18) pulmonary hypertension. 5HT and HIAA were also measured in children after corrective cardiac surgery using cardiopulmonary bypass (n=14) and in controls without congenital heart disease (n=18). The concentrations of total and free 5HT were not significantly different between controls and patients with a left-to-right shunt. After cardiac surgery total 5HT concentration was significantly reduced by about 65% owing to a postoperatively reduced platelet count. In patients with a left-to-right shunt the total 5HT content was similar in the right atrium (204.0±17.3 ng/ml), pulmonary artery (189.0±19.1 ng/ml), and aorta (195.0±19.3 ng/ml), as was the free 5HT concentration. Therefore no net release of 5HT from platelets occurred between these sampling sites. In patients with pulmonary hypertension, the urinary excretion of HIAA was significantly increased when compared with controls and patients without pulmonary hypertension. It is concluded that turbulent blood flow in children with a left-to-right shunt does not lead to a significant release of 5HT from platelets. However, the increased urinary excretion of HIAA in patients with pulmonary hypertension indicates an increased turnover of 5HT, probably due to an increased number of intrapulmonary neuroepithelial cells or a higher metabolic rate of 5HT within those cells.     

Nitric Oxide in the Evaluation of Congenital Heart Disease with Pulmonary Hypertension: Factors Related to Nitric Oxide Response

Inhaled nitric oxide (NO) has been used in the preoperative evaluation of patients with congenital heart disease and pulmonary hypertension. The purpose of this study was to characterize responses in pulmonary vascular resistance (PVR) to oxygen and increasing doses of NO during cardiac catheterization and to determine if any related factors affect the response of the pulmonary vascular bed to NO. A prospective analysis of 42 patients (median age, 3.0 years) with congenital heart disease and pulmonary hypertension who underwent NO testing was performed. Systemic vascular resistance (SVR) and PVR were assessed in room air, 100% oxygen, and oxygen plus 20, 40, and 80 parts per million (ppm) NO. Changes in pulmonary artery pressure, PVR, and SVR were assessed. The response to NO was then correlated to individual patient’s age, gender, type of heart defect, the presence of trisomy 21, and baseline PVR/SVR. There was a greater decrease in PVR and PVR/SVR with 20 ppm NO than with oxygen alone. There was no additional decrease at 40 or 80 ppm NO. There was no correlation between age, gender, type of congenital heart disease, and baseline PVR/SVR ratio with the degree of response to NO. Patients with trisomy 21 had less of a response to NO (p = 0.017) than patients without trisomy 21. There is no difference in determining PVR response with doses of NO beyond 20 ppm during cardiac catheterization. Age, gender, and baseline PVR/SVR ratio are not associated with responsiveness to NO. Patients with trisomy 21 may be less responsive to NO.     

先天性心脏病肺动脉高压手术前后右心功能变化和治疗

目的 了解先天性心脏病左向右分流导致的重度肺动脉高压对右心功能的影响以及手术矫治后右心功能的恢复状态。方法 对室间隔缺损(室缺)合并重度肺动脉高压(全肺循环阻力增加)的40例患儿，用心导管的方法进行术前、术后5～7年右心功能、肺循环的血流动力学随访测定。结果 术前右心心搏指数、作功指数、心排指数显著高于术后；术前右房压、右室收缩压及舒张压、肺动脉压力和阻力均增高，缺损修补后右室舒张压恢复正常，收缩压的降低与肺动脉压力下降有关；大型室缺左向右分流重度肺动脉高压右心功能不全系继发性右室高排出量心力衰竭，且伴有舒张功能障碍。结论 治疗心力衰竭不宜首选正性心肌收缩药物；降低肺动脉压力、减少左向右分流、根治心内畸形是合理的选择；术后右室收缩压持续不能恢复至正常水平，提示继发性肺血管梗阻性病变存在。     

Regression of pulmonary arteriovenous malformations following heart transplantation

Pulmonary arteriovenous malformations (PAVMs) can occur following caval to pulmonary artery connection, Glenn and/or Fontan procedure, leading to severe cyanosis and exercise intolerance. It is unknown whether these abnormalities regress or persist following heart transplantation (HTx). Twenty patients with failed Fontan or Glenn procedures were screened for PAVMs prior to HTx by contrast echocardiography, selective pulmonary angiography, and pulmonary venous desaturation. Age at transplant, diagnosis, previous operations, time from Glenn to transplant, systemic oxygenation, hemoglobin level, and ventricular function were determined. The clinical course after HTx was characterized in three patients with significant PAVMs. Indications for HTx were exercise intolerance and severe cyanosis in one patient, and cyanosis and ventricular dysfunction in two. Pre-HTx, mean systemic saturation was 67%; mean pulmonary venous wedge saturation was 81%. Post-HTx, oxygen saturations were normal (> 96%) at 14, 40, and 180 days. Contrast echocardiography, performed 1 month to 3.3 yrs after HTx, showed no intrapulmonary shunting in two patients and minimal shunting in one. One patient suffered an embolic stroke from right-to-left shunting post-HTx. All patients are alive and well 35, 71, and 73 months post-HTx. In patients with single ventricle physiology, PAVMs are not an absolute contraindication to HTx. Heart-lung transplant may not be required for these patients.     

室间隔缺损术后反应性肺高压危险因素及疗效分析

目的 探求室间隔缺损、肺高压患儿术后反应性肺高压的发生率、危险因素.评估术后反应性肺高压的治疗效果.方法 对1991年至2006期间术前诊断室间隔缺损、肺高压在我科接受室间隔缺损修补术患儿的客观病史资料进行回顾性研究.搜集患儿围术期客观指标进行统计和分析,评估术后早期疗效.探求术后反应性肺高压的危险因素.结果 16年间共2 141例室间隔缺损合并肺高压的患儿纳入本课题.术后住院早期死亡20例,住院晚期死亡3例,术后病情平稳出院2 118例.术后反应性肺高压发生率为6.1%,术后肺高压危象的发生率为2.1%、死亡率为2.3%.术中体外循环时间≥90min(P＜0.01)、主动脉阻断时间≥75min(P＜0.01)、术后残余分流(P＜0.01)是发生术后反应性肺高压的相关危险因素.结论 室间隔缺损、肺高压的患儿术后反应性肺高压的独立危险因素有:术前年龄、Pp/Ps、存在充血性心力衰竭、术后二尖瓣反流中度以上.术后反应性肺高压的患儿更容易依赖儿茶酚胺类药物,左房途径输入儿茶酚胺类药物能增加其强心效果.

Abstract：

Objective To study the risk factors and management for reactive pulmonary hypertension (RPH) after corrective surgery of ventricular septal defect (VSD) in children. Methods From 1991 to 2006, 2141 patients who underwent corrective surgery for VSD were recruited in this study.The patients' clinical data, including patient's gender, age, diagnosis, the ratio of pulmonary pressure to systolic pressure (Pp/Ps), the time of cardiopulmonary bypass (CPB), postoperative RPH, pulmonary hypertension crisis (PHC) and other complications, and the treatment of RPH, were retrospectively analyzed. The risk factors of postoperative RPH were statistically analyzed. The outcomes of RPH treatment were also evaluated. Results Twenty patients died during the early in-hospital period, 3 patients died during the late in-hospital period, and the others were recovered after surgery. The mobility of postoperative RPH and PHC were 6. 12% and 2. 06%, respectively. The overall mortality of the patients was 2. 27%. The patients had CPB≥90 min, aortic clamp time≥75 min, postoperative catecholamines treatment, and residual intracardiac shunting after surgery were more likely to develop postoperative RPH. Conclusions The independent risk factors of postoperative RPH include patients'Pp/Ps ratio, age, congestive heart failure before surgery, moderate mitral regurgitation after surgery.Catecholamines treatment could effectively improve right ventricular function in the patients with RPH.