肺动脉闭锁合并室间隔缺损行肺动脉下拉重建右心室流出道

目的明确肺动脉闭锁合并室间隔缺损行肺动脉下拉重建右心室流出道后肺动脉发育、瓣膜反流及右心功能情况。方法回顾性分析2002年11月至2012年9月广东省人民医院31例肺动脉闭锁合并室间隔缺损患者的临床资料,其中男20例,女11例;患儿手术时14d～14．50岁（47．90±53．84）个月,均采用肺动脉下拉重建右心室流出道。随访评估其吻合口及肺动脉发育情况、肺动脉与三尖瓣反流及其与随访时间的相关关系;采用右心室应变等指标评估右心功能。结果术后早期死亡3例。随访27例（27／31,87．1％）,随访时间4～129（35．97±28．24）个月。随访期间无死亡。根治术后元吻合口再狭窄,姑息术后吻合口相对狭窄4例。吻合口及左、右肺动脉的直径均明显大于术后早期;肺动脉反流及i尖瓣反流加重,但肺动脉反流量与随访时间无相关关系。远期右心功能整体良好。结论肺动脉闭锁合并室间隔缺损行肺动脉下拉重建右心室流出道效果良好,吻合口及肺动脉分支可生长发育,术后中远期右心功能良好。     

应用肺动脉管道矫治主动脉弓离断后管道重度扩张一例

临床资料患者,男,18岁,体重59.5 kg。主动脉弓离断合并室间隔缺损（VSD）矫治术后2年。患者于2年前发现心脏杂音16年,劳力后心悸、气促1年,入院行手术治疗。经胸骨正中切口径路手术,深低温停循环下用自体肺动脉片制作管道（Neville管,直径20 mm）行A型主动脉弓离断矫治术（图1）、VSD修补和动脉导管结扎术。     

心上型完全性肺静脉异位引流手术132例的治疗效果分析

目的总结无内膜接触缝合（Sutureless）技术应用于矫治心上型完全性肺静脉异位引流（TAPVC）的临床疗效。方法2007年7月至2013年12月在广东省心血管病研究所行外科矫治术的所有心上型TAPVC婴幼儿共132例,排除合并单心室、心房异构等患儿,均行双心室根治性手术。全部患儿均行超声心动图检查,绝大部分患儿行CT检查了解肺静脉发育情况,进行术前评估,并术中探查确诊。按不同的手术方式分为传统手术组和Sutureless手术组。其中传统手术组69例,男54例（78．3％）、女15例（21．7％）;中位年龄60（30,225）d;中位体重4．85（3．50,6．35）kg。Sutureless手术组63例,男48例（76．2％）、女15例（23．8％）,中位年龄90（30,210）d;中位体重4．58（3．72,6．20）蝇。随访至2014年1月1日。结果Sutureless手术组住院死亡率（4．8％VS．7．2％,x^2=1．414,P=-0．720）、术后总死亡率（4．8％VS．13．0％,x^2=2．733,P=-0．098）均低于传统手术组,但差异无统计学意义。Sutureless手术组术后梗阻率低于传统手术组（1．6％VS．10．1％,x^2=4．236,P=-0．040）,差异有统计学意义。COX多因素分析发现,传统手术方式、术前肺静脉梗阻与术后肺静脉梗阻的发生显著相关（P=0．023,P=0．016）。传统手术方式与术后死亡无明显相关性（P=0．060）。结论对于心上型TAPVC,Sutureless技术可明显降低术后肺静脉梗阻发生率。     

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。     

One-time pulmonary artery reconstruction to repair infant unilateral absence of pulmonary artery: A case report

<正>INTRODUCTION Unilateral Absence of Pulmonary Artery(UAPA) is a rare complicated congenital heart disease firstly described by Frentzel in 1868.An absent pulmonary artery is caused by the involution of the proximal sixth aortic arch     

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.     

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.     

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.     

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.     

中国Williams综合征婴儿合并先天性心血管病研究

目的 分析1岁前诊断为Williams综合征患者合并先天性心血管病的发病率、类型及中期预后。方法 回顾性分析2008年1月至2020年8月诊断为Williams综合征患者的临床资料,通过全外显子测序技术明确诊断,心脏超声评估患者心血管发病情况,并比较随访初始和最近或术前的血管狭窄处压差值。结果 本次研究共纳入110名在1岁前被确诊并进行了心血管系统检查的Williams综合征患者,确诊年龄（130±105）d,随访时间（3±2.2）年,其中男性64例,女性46例。99名（99/110,90%）患者合并有先天性心血管畸形,其中最常见的为主动脉瓣上狭窄,其次为外周肺动脉狭窄。在随访期间,主动脉瓣上狭窄处压差基本保持不变,而外周肺动脉狭窄压差可随时间的推移而自发降低[从（56±24）mm Hg降至（27±18）mm Hg,P＜0.001]。18例患者接受了外科手术矫治主动脉瓣上狭窄,压差由术前的（105.17±39.84）mm Hg降至术后7天的（18.42±8.73）mm Hg (P<0.001),其中2名患者术后再发狭窄,1名合并重度双侧心室流出道梗阻患者死亡。 结论 Williams综合征患者心血管疾病发病率高,主动脉瓣上狭窄和外周肺动脉狭窄为心血管畸形的主要类型,也常合并其它心内畸形。其中主动脉瓣上狭窄严重程度常保持不变,而大部分外周肺动脉狭窄包括重度狭窄均可自发地得到改善。手术治疗Williams综合征合并主动脉瓣上狭窄的疗效满意,但有再狭窄可能。