Contemporary treatments and outcomes of preterm infants with critical congenital heart disease in a tertiary cardiovascular institute in China

Background The survival rate of preterm infants with critical congenital heart disease(P-CCHD) has been improved by medicine advances. The aims of this study were to investigate the contemporary treatments for shortterm outcomes of P-CCHD and to evaluate risk factors associated with the outcomes. Methods Sixty-four PCCHD patients admitted to Guangdong General Hospital between 2011 and 2015 were included in this study. Demographic characteristics and patient records were reviewed. Logistic regression was used to analyze the risk factors of P-CCHD outcome. Results Thirty-six patients underwent surgical treatments for cardiac anomalies.Moreover, 31.25% of the P-CCHD infants did not receive surgery because these parents refused further treatment. The in-hospital mortality rate was 8.3% for the patients who underwent surgeries. During a median followup of 1.2 years, the survivors were basically healthy. However, mental and physical growth retardation remained.Conclusions Compared to infants in developed Western countries, the treatments and short-term outcomes of P-CCHD infants were satisfactory. However, the long-term outcomes remain to be determined.     

胎儿严重肺动脉瓣狭窄或闭锁的产前超声心动图诊断评估、右心室发育评估及宫内介入治疗的结局随访

目的探讨胎儿室间隔完整型严重肺动脉狭窄(critical pulmonary stenosis with intact ventricular septum,CPS/IVS)或室间隔完整型肺动脉闭锁(pulmonary atresia with intact ventricular septum,PA/IVS)的产前超声心动图诊断、右心室发育评估及宫内介入治疗的结局随访。方法回顾性分析广东省人民医院2016年9月至2018年12月6例产前超声心动图诊断为PA/IVS或CPS/IVS(1例诊断PA/IVS,5例为CPS/IVS)行胎儿肺动脉瓣球囊成形术(fetal pulmonary valvuloplasty,FPV)胎儿的产前超声心动图诊断、右心室发育评估及结局随访资料。结果 6例胎儿诊断孕周为(26.48±2.15)周。6例胎儿术前三尖瓣环/二尖瓣环比值(tricuspid valve annulus/mitral valve annulus,TV/MV)分别为0.53、0.82、0.71、0.85、0.77、0.71,右心室纵径/左心室纵径比值(right ventricle length/left ventricle length,RV/LV)分别为0.42、0.63、0.52、0.61、0.75、0.61,三尖瓣流入时间/心动周期长度比值(tricuspid valve inflow duration/cardiac cycle length,TVID/CCL)比值分别为0.26、0.35、0.39、0.44、0.44、0.35,肺动脉瓣环/主动脉瓣环(pulmonary valve annulus/aortic annulus,PV/AV)比值分别为0.85、1.03、0.85、0.86、1.20、0.78。动态观察2周后,6例胎儿右心室各指标未见明显增长,于(29.45±1.19)周行FPV术,均取得技术性成功(100%),无宫内死亡,孕妇及胎儿无严重并发症。FPV术后,6例胎儿术后1～2周内TV/MV、RV/LV、TVID/CCL明显增长,术后2～6周增长趋于稳定,而PV/AV则在术后2～4周增长较明显。6例患儿分娩胎龄为(37.74±1.17)周,出生体质量(3.04±0.24)kg,于生后(15.33±7.31)d完成行一期手术,其中2例行外科手术,4例12的严重感染所致的多器官功能衰竭。存活的5例患儿血氧饱和度95%,无右心衰竭症状。结论产前可通过超声评估CPS/IVS、PA/IVS胎儿右心室发育状况,筛选宫内介入治疗适应证,适时进行FPV,可促进右心室小梁部及三尖瓣发育,争取患儿生后实现双心室循环。     

一期或分期全腔静脉-肺动脉连接术治疗功能性单心室

目的总结、比较一期和分期全腔静脉-肺动脉连接术(total cavopulmonary connection,TCPC)治疗复杂先天性心脏病的疗效。方法分析2003年12月至2007年9月在广东省人民医院住院,适合一期生理矫治并施行了一期TCPC术的患儿25例和已先期完成第一阶段姑息手术并施行了二期TCPC术的患儿17例的临床资料。前期手术包括15例双向Glenn手术、1例体-肺分流术、1例三尖瓣下移畸形矫治加双向Glenn手术。一期TCPC组中14例采用外管道,11例肺动脉直接下拉与下腔静脉吻合。二期TCPC组中15例采用外管道,1例采用心房内通道,1例肺动脉直接与下腔静脉吻合。结果早期死亡4例,病死率9.5%(4/42)。一期TCPC组死亡3例,病死率12.0%(3/25);二期TCPC组死亡1例,病死率5.9%(1/17)。二期TCPC组的辅助通气时间、胸管停留时间、住监护室时间、术后住院天数比一期TCPC组短,但差异无统计学意义(P0.05)。随访8个月到4.8年。1例于术后8个月死于严重的房室瓣反流、心力衰竭。结论一期和分期TCPC手术治疗复杂先心病都能达到良好的效果。     

体外循环心脏手术对婴幼儿期患儿S-100β与神经元特异性烯醇化酶的影响

目的 探讨体外循环心脏手术对婴幼儿期患儿中枢神经系统的影响.方法 3岁以下先天性心脏病患儿30例,分为非紫绀组(n=14)和紫绀组(n=16),进行心脏手术.将两组患儿的临床资料和不同时点血S-100β蛋白和神经元特异性烯醇化酶(NSE)浓度进行比较.结果 紫绀组患儿主动脉阻断和体外循环时间比非紫绀组长(P<0.05);两组患儿术后即刻S-100β浓度比术前高(P＜0.05),组间比较差异无统计学意义(P＞0.05);非紫绀组患儿NSE术后所有时点比术前高(P＜0.05),紫绀组患儿术后6、24 h比术前高(P＜0.05),组间比较差异无统计学意义(P＞0.05).术后即刻S-100β浓度峰值与清醒时间(r=0.452,P<0.05)、机械通气时间(r=0.450,P<0.05)呈正相关;术后即刻NSE浓度峰值与主动脉阻断时间(r=0.462,P<0.05)呈正相关.术后随访,所有患儿智力正常,生长发育良好.结论 婴幼儿心脏手术,对中枢神经系统有一定影响,这些影响多为可逆的;在法洛四联症和室间隔缺损伴肺动脉高压患儿之间没有明显差别.     

应用无内膜接触缝合技术治疗心上型全肺静脉异位引流

Objective Retrospectively analyze surgical correction supracardiac total anomalous pulmonary venous con-nection (TAPVC) using sutureless technique to prevent post-repair pulmonary vein stenosis. Methods Between December 2007 and December 2008, 25 children cases of total anomalous pulmonary venous connection underwent primary surgical cor-rection. The anatomic types of TAPVC were supra cardiac in nine patients, inha cardiac in one, mixed in 3 and intra cardiac in 12. Five of nine supra cardiac TAPVC underwent correction using sutureless technique. There were 2 male and 3 female. Their age at surgeries ranged from 2 months to 13 years, and the body weight were from 4.5 kg to 2.1 kg with an average of (7.9±6.4) kg. After median sternotomy and opening the pericardium, the heart was arrested by delivering cold crystal ear-dioplegia. The heart was then positioned toward the patient' s right and under the right henri sternum. A generous incision across the posterior wall of the left atrium and one on the common pulmonary vein was made. The latter was extended upwards to the midpoint of the vertical vein. The left atrium was subsequently connected to the pulmonary venous confluence by suturing the edge of the atrium to the posterior mediastinal pericardium that surrounding the common pulmonary vein and the vertical vein with 7-0 PDS. The vertical vein was partially ligated after conclusion of CPB, leaving a diameter of 5mm shunt. Routine follow-up with echocardiogram were at diacharging, 3 months, 6 months and 1 year after surgery. Results All 5 cases survived uneventfully excopt 1 baby on ventilation over 7 days after surgery, who had bilateral lung consolidation before the operation. Echocardiogram showed satisfactory results with maxium velocity acrossing the anastomosis of 0.65 -0.85 m/s. Conclusion Sutureleas technique can avoid trauma to the pulmonary venous endothelium and minimize the tension of anastomasis. It may play an important role to prevent post-repair pulmonary vein stenosis. More patients with long-term follow-up are necessary to draw a definite conclusion of this technique.     

库存红细胞清洗在新生儿及小婴儿体外循环手术中的应用

目的 对比体外循环(CPB)心脏手术中接受洗涤及未洗涤的库存红细胞(PRBC)对血清钾离子浓度(K+)及乳酸浓度(LAC)的影响.方法 选取复杂先天性心脏病(先心病)行CPB心脏手术的新生儿及小婴儿为研究对象,排除术前血乳酸大于3.0mmol/L的病婴.对照组(15例)接受未洗涤的PRBC进行CPB预充及术中添加,试验组(15例)接受用血液回收机(Medtronic Autolog)洗涤过的PRBC.在CPB前、CPB 3、15min、CPB复温、停止CPB前、术毕、术后4、24h比较血K+及LAC浓度.结果 洗涤PRBC显著降低供血中K+浓度[从(19.3±0.9)mmol/L降至(1.1±0.3)mmol/L,p<0.001]及IAC浓度[从>15mmol/L降至(7.8±1.2)mmol/L,P<0.001].对照组预充液的K+及ILAC浓度显著高于试验组[K+(9.0±0.5)rranol/L对(2.6±0.1)mmol/L,P<0.001;LAC浓度(9.5±0.5)mmol/L对(4.7±1.1)nrml/L,P<0.001].CPB 3min、CPB复温时对照组血清K+浓度显著高于试验组[CPB 3 min(5.6±0.9)mmol/L对(3.5±0.4)mmol/L,P<0.01;CPB复温时(4.8±0.7)mmol/L对(3.7±0.6)mmol/L,P<0.01];其余时点对照组K+浓度高于试验组,差异无统计学意义.CPB 3、15 min、CPB复温、停止CPB前、术毕、术后4 h对照组IAC浓度高于试验组,但差异无统计学意义.结论 洗涤PRBC降低血清钾及乳酸负荷,可预防cPB中高血钾.在新生儿及小婴儿复杂先心病CPB手术中应考虑应用清洗PRBC.     

深低温停循环与区域性脑灌注对新生儿及小婴儿的脑保护效果

目的分析新生儿及小婴儿主动脉手术中深低温停循环（DHCA）与区域性脑灌注（RBP）对血清S100B蛋白与神经元特异性烯醇化酶（NSE）表达的影响,评价两种方式的脑保护效果。方法主动脉缩窄（CoA）或主动脉弓离断（IAA）合并心内畸形患儿24例,其中男20例,女4例,年龄6～197d,平均（78&#177;58）d,体重2．3—5．6kg,平均（4．0&#177;0．8）k,随机分为DHCA组（15例）和RBP组（9例）。麻醉后经颈内静脉逆行穿刺保留置管,分别于麻醉诱导后、DHCA／RBP前、DHCA／RBP结束时、体外循环（CPB）结束时、术后3h、术后12h、术后24h由颈内静脉插管留取血标本测定血清S100B蛋白与NSE含量。结果所有患儿均于术后24h内清醒,未出现近期神经系统并发症。两组患儿S100B蛋白与NSE均在CPB开始后持续升高,CPB结束时达峰,随后迅速下降,各时间点两组患儿S100B蛋白与NSE水平差异均无显著性（P〉0．05）。结论一定时限内,DHCA不会引起新生儿及小婴儿缺血缺氧性脑损伤,与RBP具有相似的脑保护作用。     

动脉导管未闭双面伞堵闭术后严重溶血的急诊手术三例

从1994年7月至1997年3月,我科先后急诊手术治疗3例动脉导管未闭(ＰＤＡ)双面伞堵闭术后严重溶血的患者,取得满意效果。资料与方法　3例4～6岁患儿,因自幼发现心脏杂音入院。经Ｘ线胸片、心电图、超声心动图检查,诊断为ＰＤＡ。在逆行主动脉造影下,显示ＰＤＡ均为漏斗型,最窄处内径为436～598ｍｍ。均选用17ｍｍＲａｓｈｋｉｎｄ双面伞堵闭系统,经股静脉穿刺置放双面伞器堵闭ＰＤＡ成功。术后15分钟再次主动脉造影均显示有少量残余分流。3例患儿均在堵闭术后4～8小时,出现酱油色尿,血红蛋白进行性下降,经各项检查诊断为急性机械性重度溶血。…     

库存红细胞清洗在新生儿及小婴儿体外循环手术中的应用

Objective Priming blood in cardiopulmonary circuit is necessary for neonates and small infants. However, high concentration of potassium and lactate in pecked red blood cells transfused during cardiopulmonary bypass may have detrimental effects on meonstea and infants undergoing cardiac surgery. This study is to cornice the effects of trasfusing washed end unwashed peeked red blood cells for cardiopulmonary circuit on serum potassium and lactate concentrations preoperatively. Methods 30 neonates and small infants with complex congenitai heart disease undergoing open heart surgery were divided into 2 groups randomly. Unwashed group (n = 15) received unwashed pecked red blood cells and washed group (n=15) received packed red blood cells washed in a cell saver (Medtronic Autolog). Potasium and lactate concertrations were compared before, during and after bypass. Resuils Wash-packed red blood cells reduced donor blood [K+] from (19.3±0.9)mmd/L to (1.1 +0.3) mmol/L, and lactate from 15 mmo/L to (7.8±1.2) mmol/L (P<0.001). The [K+]in the prime solution was significantly higher [(9.0±0.5) mmol/L vs. (2.6±0.1) mmol/L, P<0.001] in umwashed group than that of washed group, so did the lactate [(9.5±2.6) mmol/L vs. (4.7±1.1) mmol/L, P相似文献   

Konno-Rastan手术治疗儿童复杂性多水平左心室流出道梗阻

目的 总结Konno-Rastan手术治疗复杂多水平左心室流出道梗阻的疗效、并发症和常见失误及预防.方法 1996年1月至2012年8月,13例患儿行主动脉根部及左心室流出道扩大、人工机械瓣膜主动瓣置换(Konno-Rastan术).男8例,女5例;年龄5～13岁;体质量12～51 kg,中位值21 kg.诊断先天性主动脉瓣狭窄8例,先天性主动脉瓣狭窄合并主动脉瓣上狭窄3例,先天性主动脉瓣狭窄合并室间隔缺损、主动脉缩窄及右心室流出道狭窄1例,先天性主动脉瓣狭窄、主动脉瓣球囊扩张术后再狭窄1例.患儿均合并继发性室间隔增厚、左心室流出道梗阻.主动脉瓣环直径12.0 ～ 16.4 mm,术前跨主动脉瓣压差90～ 151 mm Hg(1 mm Hg =0.133 kPa).置入St.Jude AG19号机械瓣膜8例,AG17号5例.结果 无手术死亡.呼吸机辅助4～74 h,中位时间6h;ICU停留1～6天.1例术后第3天出现突发性晕厥,确诊为Ⅲ度房室传导阻滞,植入永久性心外膜起搏器,术后7天恢复窦性心律,传导功能正常,将起搏器设定为60次/min,VVI模式备用.2例术后3个月复查发现新生心室水平残余分流,其中1例因反复心功能衰竭再次手术,修补残余分流,术后心功能状态良好,无残余心内畸形;1例因无明显症状,门诊随访,术后3年患类风湿,感染性心内膜炎及肾病综合征,拟择期行残余分流修补术.其余患儿无不适症状.全部患儿随访1 ～78个月,终生服用华法林抗凝,控制国际标准比值(INR) 1.8 ～2.5.随访期内无死亡,未发现人工瓣膜相关性并发症发生.完全性房室传导阻滞发生率为7.7％;残余分流发生率为15.4％;术后早期感染性心内膜炎发生率为0,远期发生率为7.7％.结论 Konno-Rastan手术可以有效地治疗患儿复杂的多水平左心室流出道梗阻,但由于手术操作复杂,可导致较高比例的手术并发症;完善手术操作是提高手术成功率的关键因素之一.