先天性冠状动脉瘘是指冠状动脉和心腔或大血管间存在异常交通，是一种少见疾病，瘘入右心室壁内极为罕见。本文报道1例6岁男性儿童右冠状动脉右心室壁内瘘形成巨大夹层瘤，准确诊断并成功手术治疗的病例。冠状动脉心室壁内瘘存在破裂风险，发现后应尽早手术；超声心动图及冠状动脉造影对冠状动脉瘘诊断具有重要意义。

Objective: To examine the early and midterm surgical outcome of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) using revised surgical strategies. Methods: A retrospective analysis of clinical data, surgical methods, and follow-up results was performed of 104 cases of PA/VSD/MAPCA in Department of Cardiovascular Surgery, Guangzhou Women and Children's Medical Center from January 2017 to September 2022. There were 55 males and 49 females, aged (M(IQR)) 33.9(84.0) months (range: 0.5 to 209.6 months) at the first surgical procedures. The anatomical classification included 89 cases of type B and 15 cases of type C. The number of major aortopulmonary collateral arteries was 4.2 (3.0) (range: 1 to 8). The Kaplan-Meier method was used for survival estimation. Results: In the first stage of surgery, 50 patients underwent a complete primary repair, 12 patients underwent partial repair, 32 patients underwent palliative right ventricular-pulmonary artery connection, and only 10 patients chose the Blalock-Taussig shunt. There were 10 cases of early death. In the second stage, 14 patients underwent complete repair and 4 patients underwent partial repair with no early death. The interval between the two surgeries was 19 (10) months (range: 9 to 48 months). Finally, during the 40 (34) months follow-up period, a total of 64 patients were complete repair and the right/left ventricular pressure ratio after complete repair was 0.63±0.16 (range: 0.36 to 1.00). Survival analysis showed that survival rates at 1 and 5 years after first-stage surgery were both 89.4% (95%CI: 83.5% to 95.3%). At 28 (34) months (range: 1 to 67 months) of follow-up after complete repair, the survival analysis showed that the survival rates at 1 and 5 years were both 95.2% (95%CI: 89.9% to 100%). Conclusions: Using combined approaches tailored to individual patients and optimized unifocalization strategy, the complete repair rate at one stage and the cumulative complete repair rate at 5 years improved significantly with a lower right/left ventricular pressure ratio and satisfactory early and intermediate survival.

目的 探讨婴儿期外科治疗完全性肺静脉异位连接(total anomalous pulmonary venous connection,TAPVC)的风险因素,分析不同类型TAPVC外科治疗随访结果的差异.方法 总结广州市妇女儿童医疗中心2009年1月至2019年12月婴儿期手术治疗的TAPVC患儿的资料,同时对这些患儿进行随访.将术前有关因素与住院手术死亡进行单因素相关性分析和多因素相关性分析;将全部患儿按TAPVC的类型分组,分别用Kaplan-Meier法生成术后各组的近-中期生存曲线,并对这些生存曲线用log-rank法进行总体差异性检验,同时对各组间的差异性进行检验.结果 手术患儿246例,手术死亡16例,手术病死率为6.50%.心内型、心上型、心下型、混合型分别为91例、106例、34例、15例,分别手术死亡2例、5例、6例、3例,病死率分别2.20%、4.72%、17.65%、20.00%.单因素分析显示与手术死亡有相关性的因子有TAPVC类型、术前休克/酸中毒、体外循环时间、主动脉阻断时间、手术过程中有深低温停循环;多因素分析显示与手术死亡有相关性的因子有TAPVC类型、术前休克/酸中毒、体外循环时间、主动脉阻断时间、手术过程中有深低温停循环、手术时低体质量.全部手术存活患儿随访死亡19例,用Kaplan-Meier法生成生存曲线,术后1年、3年、6年、9年总体累积生存率分别为89%、86%、85%、85%.随访死亡患儿中,心上型、心内型、心下型、混合型分别为6、5、8、0例.各组术后1年、3年、6年、9年累积生存率分别为心内型92%、92%、92%、92%;心上型93%、90%、89%、89%;心下型70%、54%、54%、54%;混合型80%、80%、80%、80%.术后各组生存曲线比较,差异有统计学意义(P<0.05):心下型术后累计生存率显著低于心内型和心上型.结论 婴儿期TAPVC的外科治疗独立风险因子有TAPVC类型、术前休克/酸中毒、体外循环时间、主动脉阻断时间、手术过程中有深低温停循环、手术时低体质量;婴儿期TAPVC的心内型、心上型、混合型、心下型术后近中期累计生存率总体上有显著差异;心下型术后累计生存率显著低于心内型、心上型.

Objective:To study the clinical characteristics of double aortic arch (DAA) combined with aortoesophageal fistula (AEF), and summarize the diagnosis and treatment experience.Methods:Retrospective analysis was performed on the diagnosis and treatment of a newborn with hemorrhagic shock caused by DAA combined with AEF in the Guangzhou Women and Children's Medical Center. The key searching words included "double aortic arch", "aortoesophageal fistula", "vascular ring", "newborn or neonate", and "infant, newborn". The relevant reports were retrieved from databases of CNKI, Wanfang, VIP, PubMed, Springer Link, Google Scholar, Web of Science, Embase, Cochrane Library and OVID, to summarize the clinical features, diagnosis and treatment experience of neonates with DAA and AEF. The retrieval deadline was December 31, 2020.Results:A full-term female newborn was hospitalized for dyspnea immediately after birth, and failed to evacuate from the ventilator for several times. The patient was fed with nasogastric tube and transferred to our hospital because of hemorrhagic shock occurring in 32 days after birth, and gastrointestinal bleeding occurred repeatedly with the maximum bleeding volume reaching 200 ml/time. DAA was diagnosed by cardiac ultrasound and CT, AEF hemorrhage was finally confirmed by gastroscopy, aortography and operation. DAA correction and esophagus repair were successfully performed, and the infant recovered well after the operation. At 9-month old, the infant grew and developed well. At present, no reports of DAA combined with AEF neonates have yet to be published in medical literatures in China. Seven English language literatures included 7 cases of AEF complicated with DAA in neonatal period, 5 cases survived and 2 cases died have so far been reported. All patients have a long history of gastric tube indwelling.Conclusions:The incidence of DAA combined with AEF is rare in the newborn with respiratory and swallowing difficulties as the first manifestation. The disease symptoms progressed rapidly, and life-threatening digestive tract hemorrhage may occur, which often requires surgical treatment. Prolonged gastric tube retention should be avoided in DAA children to prevent the occurrence of AEF.

Objective:To summarize the early and middle terms of the revascularization of remnant pulmonary artery in unilateral absent intrapericardial pulmonary artery.Methods:We retrospectively analyzed the medical records of 14 patients(7 males and 7 females) with unilateral absent pulmonary artery, in which 10 were right and 4 were left, the median age at surgery was 5 months. The patients received operation from January 2009 to December 2020. 14 patients, 2 cases associated with tetralogy of Fallot, and 1 case with aortopulmonary window. The diagnosis was made by enhanced CT scan or pulmonary vein wedge angiography. The median diameter of the affected hilar pulmonary artery remnants was(3.20±0.94)mm, and the Z value was -3.92±1.64. All the patients received single-stage revascularization: group A: tube graft interposition in 3 patients, autologous pericardial roll in 5; group B: direct anastomosis in 2, unifocalization in one and main pulmonary artery flap angioplasty in the rest 3.Results:No hospital death occurred. There were no difference finds in the age and weight at operation, the cardiopulmonary bypass time, the mechanical ventilation time, and the length of intensive care unit stay between the two groups. All the patients took aspirin for anticoagulation for 6 months after the operation. The follow-up period was 1 month to 68 months. Because the neo-PA stenosis at the anastomosis was found in one patient in group B, transcatheter balloon angioplasty was performed at 41 months after surgery. Nonetheless, the results were encouraging, symptoms have improved in all patients. The diameter and Z value of the latest ipsilateral pulmonary artery was(6.25±0.99)mm and -2.34±1.18 respectively, significantly improved when compared to the preoperative value. Residual pulmonary artery hypertension was not found. The Z value of the affected side of the pulmonary artery in group B was significantly improved than that in group A.Conclusion:Early and aggressive pulmonary artery revascularization is effective at restoring normal antegrade flow to the affected lung, resulting in improved diameter of the PA, and UAPA patient’s symptoms. The use of autologous pulmonary artery tissue angioplasty may reach a more satisfying result. However, transcatheter intervention may diminish the new pulmonary artery stenosis temporarily, reoperation is still needed in the long-term follow-up.

Objective:To summarize the institutional experiences of treating severe bradycardia with epicardial permanent pacemaker implantation.Methods:From July 2010 to July 2020, clinical data were retrospectively reviewed for 52 hospitalized children with severe bradycardia. There were 25 boys and 27 girls with a median age of 338(143-1696) days and a median weight of 7.3(5.1-14.3) kg. All operations were performed by the same group of surgeons. Clinical data were processed by SPSS 20.0.Results:There were complete atrioventricular block complicated by cardiac surgery (n=40, 76.9%) , congenital complete atrioventricular block (n=11 , 21.2%) and sick sinus syndrome (n=1). The median follow-up period was 35.3 (11.5-71.4) months. Among these patients 5 (9.6 %) developed complications, 4 (7.7%) died. As compared with immediate postoperative data, pacing impedance declined ( P=0.019) and capture threshold spiked markedly at the last follow-up ( P=0.009). However, pacing function was decent. Conclusion:Epicardial permanent pacemaker implantation is both effective and safe for children with severe bradycardia. There is a low risk of complications and mortality and medium/long-term outcomes are satisfactory. However, clinicians should pay close attention to the problems of pacemaker leading to fracture and dysfunctions.

Objective:Extracorporeal membrane pulmonary oxygenation (ECMO) is widely applied in neonates with cardiopulmonary failure. This study was intended to explore the effect of enteral nutrition (EN) on the prognosis of ECMO treatment for children.Methods:Through a retrospective case-control design, 59 ECMO cases of heart failure in the past 7 years were selected and divided into two groups of ECMO weaning success (n=38) and failure (n=21). Clinical and nutritional related parameters of two groups were compared. And the area under the curve was calculated for seeking a threshold for predicting ECMO weaning success. Based upon Cox regression model, independent risk factors were assessed for ECMO weaning failure.Results:Thirty-eight cases(64.4%) were weaned successfully and 36 cases(61.0%)were survival for discharge (n=36, 61.0%). The results of repeated measures ANOVA indicated that input/output balance and maximal lactic acid value in the first 5 days of ECMO support were significantly lower in successful group than those in failed group and the inter-group differences were statistically significant ( P=0.037 and P<0.001). In successful group, the ratio of total calorie intake to target calorie at Day 2 of EN/PN and EN energy intake to target calorie at Day 2 of EN/PN during ECMO were (38.2±24.0)% and (31.7±25.3)% respectively, which were significantly higher than those in failed group (18.6±19.8)% and (14.9±20.7)%. The inter-group difference was statistically significant ( P=0.041, P=0.047). ROC curve analysis indicated that the ratio of calorie intake to target calorie intake at Day 2 of EN≥11.445% could effectively predict a successful withdrawal of ECMO with a sensitivity of 78.8% and a specificity of 69.2%. The risk of ECMO evacuation failure spiked 4 folds at Day 2 of EN when the ratio was <11.445% of target calorie. Univariate analysis of Cox regression model indicated that sufficient calorie and EN were negatively correlated with ECMO evacuation failure and the relative risks were 0.172 and 0.264 respectively. The relative risk of ECMO evacuation failure was 4.095, 18.921, 3.126, 4.233 and 3.130 respectively with a calorie-caloric ratio <11.445% at Day 2 of EN. Severe complications and lactic acid continued increasing from Day 2 to Day 4 during ECMO. Multivariate analysis revealed that severe complications during ECMO and elevation of lactic acid at Day 2 were independent risk factors for ECMO evacuation failure. Conclusions:An early application of enteral nutrition for providing a certain amount of calorie has a positive effect on a successful withdrawal of ECMO.

目的 总结本中心近年来以扩大端侧吻合法一期矫治小儿主动脉缩窄合并室间隔缺损的手术疗效及经验.方法 选取2010年1月-2017年10月在本院行一期手术治疗的主动脉缩窄合并室间隔缺损的患儿,共208例,其中男性137例,女性71例,中位年龄54(5～730)天,中位体质量4.0(1.8～9.9)kg.胸骨正中单一切口下,以扩大端侧吻合矫治主动脉缩窄,同期修补室间隔缺损.所有手术由同一组外科医师完成.应用SPSS 20.0统计软件进行数据录入和分析.结果 术后早期死亡6例(2.9％),晚期死亡3例(1.4％);再缩窄16例(7.7％),再手术19例(9.1％).脑钠肽在术前即存在明显升高,但术后未发生明显变化;主动脉缩窄段压差在术后明显下降,经皮血氧饱和度在术后明显上升.C反应蛋白、乳酸和肌酐水平在术后明显上升.术前合并高血压104例(53.0％),术后血压降至正常30例(28.8％),术后血压继续升高35例(33.7％).术后吻合口压差较高是术后再缩窄的高危因素.结论 胸骨正中单一切口下,扩大端侧吻合法一期矫治主动脉缩窄合并室间隔缺损,死亡率和再缩窄率低,取得良好的近中期疗效.术后吻合口压差较高是主动脉缩窄复发的高危因素.

目的 评估心肺转流(CPB)联合深低温停循环(DHCA)对小儿孤立性主动脉缩窄(CoA)术后早期恢复及远期预后的影响.方法 选取2010年4月至2017年9月期间在本中心行外科手术治疗的孤立性CoA患儿共26例,通过年龄和性别匹配,分为非心肺转流组(Non-CPB)和CPB+DHCA组.对两组病例的临床资料和随访情况进行总结分析.所有手术由同一组外科医师完成.应用SPSS 20.0统计软件进行数据录入和分析.结果 在Non-CPB组,全部病例均采取左胸后外侧切口,进行扩大端端吻合;在CPB+DHCA组,全部病例均采取胸骨正中切口,3例进行扩大端端吻合,10例进行扩大端侧吻合.在术后早期,两组病例的主动脉缩窄段压差均出现明显下降;经皮血氧饱和度在CPB+DHCA组出现明显上升;平均动脉压在两组病例中均未发生明显变化.在术后早期,CPB+DHCA组的血中乳酸水平出现明显上升;而B型脑钠肽、C反应蛋白和肌酐水平在两组病例中均未发生明显变化.与Non-CPB组相比,CPB+DHCA组在ICU停留时间明显延长.CPB+DHCA组在住院期间有2例接受再手术.随访期间,Non-CPB组出现再缩窄5例,CPB+DHCA组出现再缩窄1例,均不需要再次手术干预.早期和晚期均无一例死亡.结论 CPB联合DHCA导致小儿孤立性CoA术后早期的乳酸水平明显升高及ICU停留时间显著延长,但对术后通气时间、总住院时间、住院期间再手术、远期再缩窄及早期和晚期死亡无明显影响.CPB联合DHCA在小儿孤立性CoA纠正手术中安全有效,取得良好的外科治疗效果.

Objective: To evaluate the early results of pulmonary autograft mitral valve replacement (Ross Ⅱ procedure) in infants with intractable congenital mitral valve lesions. Methods: Between August 2018 and September 2019, 6 infants underwent mitral valve replacement with a pulmonary autograft in Department of Cardiovascular Surgery, Guangzhou Women and Children's Medical Center. There were 2 males and 4 females.The age at surgery ranged from 50 days to 1 year old.Preoperative diagnoses included severe to critic mitral valve insuffiency in all patients, moderate mitral valve stenosis in 3 patients, and mitral valve prolapse in one. When the pulmonary autograft was harvested, a cuff of bovine or autologous pericardium was sewn onto the proximal (infundibular) end of the autograft ( "top-hat" configuration). The distal (pulmonary) end of the autograft was secured to the mitral annulus.At the level of the left atrial free wall, the pericardial cuff was progressively tailored and sewn onto the atrial wall to remain away from the ostia of the pulmonary veins and to maintain normal morphology of the autograft. The bovine jugular valved conduit was used to reconstruct the right ventricular outflow tract. Results: There was one early death due to sudden cardiac arrest at the night of surgery day. The remaining 5 patients were successfully recovered and discharged. Follow-up of survivors ranged from 3 to 13 months. Echocardiographic follow-up demonstrated the flow velocity across the mitral valve position was 1.5 to 2.3 m/s, with a means gradient of 4 to 6 mmHg (1 mmHg=0.133 kPa). Four patients showed mild mitral insuffiency, normal left atrium and ventricle size and left ventricle ejection fraction.One patient had moderate mitral insuffiency, pulmonary valve endocarditis, and reduced left ventricle ejection fraction. The clinical symptoms of all survivals improved significantly and the weight gain were satisfactory. Conclusion: Pulmonary autograft mitral valve replacement may be a feasible and effective remedial surgical strategy for young infants with intractable congenital mitral valve lesions.

Objective:To summarize the diagnostic and surgical experience of children with scimitar syndrome and to explore the immediate/mid-term outcomes of these operations.Methods:Retrospective analysis was performed for 14 clinical records of children with scimitar syndrome from January 2010 to December 2017. There were 7 boys and 7 girls with a mean age of (18.23±28.55 months)(23 days to 9 years). The complications included other cardiac abnormalities ( n=10), mesocardia ( n=8), right pulmonary hypoplasia ( n=7) and collaterall circulation ( n=8). Follow-ups were conducted for analyzing the immediate/mid-term outcomes of survival and stenosis of corrected scimitar vein (SV). And postoperative survival curves were plotted. Results:SVs were dissected from inferior caval vein (IVC) and anastomosed to left artrium ( n=9); SVs (2 SVs) were resected together with partial IVC wall and anastomosed to left artrium ( n=1); SVs (2 SVs) were resected respectively and anastomosed side-to-side and then anastomosed to left artrium ( n=1); SVs were anastomosed to right atrium and interatrium septum be rebuilt ( n=2) and SV was connected to artificial conduit and then connected to left atrium ( n=1) owing to that SV was too far to left atrium. In the same term of operation, complicated cardiac abnormality was cured. And 2/14 cases died immediately postoperatively with a mortality rate of 14.29%; 1/12 survivors died of pneumonia and respiratory failure at 14 months postoperatively and 1/3/5-year postoperative cumulative survival rates were 86%, 73% and 73% respectively. One corrected SV of 12 survivors was stenosed during discharge and 3 corrected SVs of 11 survivors became stenotic during follow-ups. Conclusions:Scimitar syndrome may be complicated with other cardiac abnormality, mesocardia and pulmonary hypoplasia, etc. Most SVs of scimitar syndrome may be cured by anastomosing SV to left artrium. SV should be anastomosed to right atrium instead of left atrium and interatrium septum. Collateral circulation of scimitar syndrome may be plugged during cardiac interventions and even resected intraoperatively.

目的 讨论经胸小切口室间隔缺损封堵术治疗室间隔缺损患儿的疗效及安全性,并对其中期随访结局进行分析.方法 221例室间隔缺损患儿,均行经胸小切口室间隔缺损封堵术治疗,术后对所有患儿进行随访观察,统计其封堵完成情况,并对失败案例进行分析,患儿需定期复查超声心动图和心电图,记录患儿随访期间的心脏B超检测结果 以及并发症发生情况,总结分析患儿的中期随访结局.结果187例完成随访患儿中,共177例(94.7%)封堵成功,10例(5.3％)封堵失败.177例封堵成功患儿中,围术期内无血管活性药物使用、输血、重症加强护理病房(ICU)停留,在随访期间无死亡、严重瓣膜返流、封堵器脱落、栓塞、溶血发生;10例封堵失败患儿中,6例术中立即中转开胸行传统体外循环下室间隔缺损修补术,其中3例为残余漏,1例为术中导丝无法通过室间隔缺损,1例因封堵器释放后术中经食管超声心动图(TEE)提示主动脉瓣重度返流,1例封堵后主动脉瓣变形;4例于术后因并发症行封堵器取出并行体外循环下室间隔缺损修补术,其中1例为小残余漏,家长强烈要求行修补术,1例于术后半个月因感染性心内膜炎而行修补术,2例分别于术后第3、5天发现Ⅲ度房室传导阻滞及左束支传导阻滞,分别于术后第4、6天行封堵器取出+体外循环下室间隔缺损修补术.177例封堵成功患儿中截止到随访结束时,其中共10例患儿发生传导阻滞,2例为完全性右束支传导阻滞(CRBB),6例为不完全性右束支传导阻滞(IRBB),1例为完全性左束支传导阻滞(CLBB),1例为完全性房室传导阻滞(CAVB).以上10例患儿中的2例在随访第3个月时恢复正常,其余8例患儿在随访期间未发现更严重的传导阻滞以及进一步治疗或起搏器置入.187例患儿术后1周行经胸心脏超声(TTE)提示左心房(LA)、左室舒张末期直径(LVDD)较术前变小,差异具有统计学意义(P<0.05).187例患儿随访期间均未发生主动脉瓣返流,发生三尖瓣返流加重30例(16.0％),均于术后2个月内发现,2例为局限性返流,28例为轻度返流,随访期间无进一步加重.结论 经胸小切口室间隔缺损封堵术是一项安全、可选择的替代治疗方式,中期随访结局提示并发症少、安全性高,但对于该术式是否因中期结局良好而扩大适应证以及远期并发症种类及严重性问题,仍需持续关注.

Objective:To summarize effect of poly L-lactic acid absorbable bone fixation system for preventing poor wound healing after pediatric cardiac operation.Methods:From January 2014 to December 2017, 4 424 patients underwent cardiac operations at our center. There were 1 967 patients included. According to the usage of poly L-lactic acid absorbable bone fixation system, they were classified into group A (1 015 cases) and group B (952 cases). The age and body weight at surgery was 1 month to 4.7 years and 3.6-20.0 kg in group A, and the age and body weight at surgery was 1 month to 4.2 years and 3.4-20.0 kg in group B.Results:After operation, the incidence of debridement and suturing operation for poor wound healing was 0.29% in group A, and 1.89% in group B. Two groups of incidence had significant difference ( P=0.001). Conclusion:Poly L-lactic acid absorbable bone fixation system decreased the incidence of poor wound healing after pediatric cardiac operation.

Objective:To explore the diagnosis and surgical treatment of complete vascular ring or semi-vascular ring in children and analyze the surgical outcomes.Methods:From January 2010 to June 2018, retrospective analysis was performed for medical records for 121 children with complete vascular ring or semi-vascular ring undergoing operations. Their clinical symptoms, preoperative examinations of chest radiograph, echocardiography, cardiac computed tomography (CT), fiberoptic bronchoscopy and esophagography, anesthetic modes, surgical approaches, postoperative mortality & morbility and complications were recorded during the follow-ups of surviving cases and postoperative survival status. Raw data were analyzed with SPSS16.0. Kaplan-Meier method was utilized for plotting the postoperative survival curve and immediate/middle-term survival status evaluated.Results:Vascular ring was complete (n=35) and partial (n=86). For complete vascular ring, there were double aortic arch (n=22) and right arch & right descending aorta & left ductus arteriosus/ductus arteriosus ligament (n=13). For semi-vascular ring, there were pulmonary artery sling (n=83) and left aortic arch & aberrant right subclavian artery (n=3). For double aortic arch, weak arch was resected and sutured. Two cases were complicated with tracheal stenosis and 1 symptomatic child underwent tracheal stenosis resection and end-to-end anastomosis. For right arch & right descending aorta & left ductus arteriosus/ductus arteriosus ligament, there was Kommerell diverticulum (n=8). There were aberrant left subclavian (n=11) and mirror image branching (n=2). Ductus arteriosus/ductus arteriosus ligament was resected. Kommerell diverticulum was resected and left subclavian artery transplanted into left common carotid artery (n=6) owing to the diameter of Kommerell diverticulum were 1.5 times larger than that of left subclavian artery. For 83 cases of pulmonary artery sling, the complications included tracheal stenosis (n=24), ventricular septal defect (VSD, n=4), atrial septal defect (ASD, n=10) and complete atrioventricular septal defect (CAVSD, n=1). Left pulmonary artery transplanted into major pulmonary artery and 13 symptomatic children of tracheal stenosis underwent sliding tracheoplasty. The complications of cardiac malformations were simultaneously corrected. For 3 children of left aortic arch and aberrant right subclavian artery, there were aortic arch dissection (IAA, n=1), VSD (n=1) and tetralogy of Fallot (TOF, n=1). Aberrant right subclavian artery was transplanted into right common carotid artery and the complications of cardiac malformations were simultaneously corrected. Two died during tracheoplasty. One case was complicated with postoperative mediastinal infection and another child was abandoned by parents due to tracheal anastomotic fistula. Surgical complications included massive hemorrhage (double-aortic arch correction & median thoracotomy, n=1), laryngeal nerve injury (complete vascular ring surgery, n=2) and left pulmonary artery stenosis (PA-Sling surgery, n=4). For double aortic arch, extubation was completed via day operation (n=20), with intraoperative hemorrhoea & next day (n=1) and Day 3 postoperatively after tracheal stenosis resection & end-to-end anastomosis (n=1). Thirteen children of right arch and right descending aorta and left ductus arteriosus/ductus arteriosus ligament were extubated in day operation. And 13/83 children of pulmonary sling underwent sliding tracheoplasty due to severe tracheal stenosis. The average mechanical ventilation time was (70.08±31.04)(40-130) hours. The other 70 were extubated in day operation. Three cases of aberrant right subclavian artery complicated with cardiac malformations were extubated in day operation. The sympoms of esophageal compression were all eliminated. Two children had mild sympotoms of airway stenosis. The follow-up period was from 3 to 78 months. One child died from recurrent granuloma at anastomotic site after end-to-end anastomosis during resection of tracheal stenosis (double aortic arch). The 1/3/5-year survival rate of complete vascular ring was 97%, 97% and 97% and 1/3/5-year survival rate of semi-vascular ring 98%, 98% and 98% respectively. Postoperative complications included intermittent tracheal stenosis after tracheoplasty (n=4). However, severity decreased significantly after surgery and mild-to-moderate left pulmonary artery stenosis persisted after PA-Sling surgery (n=6).Conclusions:The immediate/middle-term postoperative outcomes are excellent for children with complete vascular ring or semi-vascular ring. Vascular ring is frequently complicated with tracheal stenosis and it may impair the surgical outcomes of vascular ring.

Objective To summarize the experience of perioperative management for repair of congenital diaphragmatic hernia (CDH) supported by extracorporeal membrane oxygenation (ECMO). Method Retrospective review was conducted for the clinical data of CDH patients who received surgical repair on ECMO from December 2016 to June 2018 in Guangzhou Women and Children's Medical Center. Result Four fetus with prenatal diagnosis of left-side CDH were transferred to our Center and received standardized perinatal management. Moderate-severe pulmonary hypoplasia was recognized after evaluation by fetal imaging. Four cases were initiated with veno-arterial ECMO at 3, 35, 41, 11 h of life, respectively. Repair of the diaphragmatic defect was performed within two weeks after cannulation of ECMO. Furthermore, activated clotting time goals were adjusted to 180～220 s, activated partial thromboplastin time were stabilized between 50～80 s, platelets count were maintained>100×109/L and hematocrit was kept>30％before the surgery. The surgeries of four patients were completed on the 0.9th, 0.5th, 3.6th, 5.1th day of life on ECMO, respectively. The defect was repaired by parachute patch. The operative time was 85～210 min. According to CDH Staging System defect size (A to D), there were two with defects at grade C and other two at grade D. Postoperative total volume of drainage was 215～1301 ml and ECMO duration was 3.0～39.3 d. Three of them survived during neonatal period, while one died. Conclusion Repair of CDH on ECMO is feasible and help to improve neonatal survival, especially for those with moderate-severe pulmonary hypoplasia.

Objective: To examine the early- and midterm outcomes of pulmonary artery banding as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow. Methods: Between January 2008 and December 2017, 49 patients with single ventricle and unrestricted pulmonary blood flow underwent pulmonary artery banding at Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University. There were 29 males and 20 females. The age at the time of surgery was 5.6 (11.5) months (M(Q(R))), and the weight was 5.2 (3.9) kg. The medical records and results after pulmonary artery banding (death/reoperation, transition to the Glenn procedure) and subsequently after the Glenn procedure (death, transition to the Fontan procedure) were reviewed retrospectively. Actuarial survivals were estimated by the Kaplan-Meier curve. Relative factors for affecting outcomes were analyzed using the Cox regression hazard model. Results: There were 8 early deaths, with a mortality of 16.3%, including 4 cases who received simultaneous arch repair. There were 5 late deaths. During the follow-up of 47(62) (M(Q(R))) months, 11 patients (22.4%) underwent pulmonary artery banding adjustment, 29 patients (59.2%) underwent the Glenn procedure, 21 patients (42.8%) underwent the Fontan procedure. The survival of patients after the initial pulmonary artery banding were 77.4% (95%CI: 65.6% to 89.2%) and 72.6% (95%CI: 59.9% to 85.3%) at 1 year and 5 years, respectively. Multivariate Cox regression analysis revealed that systemic ventricular outflow tract obstruction (HR=4.25, 95%CI: 1.50 to 12.03, P=0.006) and total anomalous pulmonary venous connection (HR=6.49, 95%CI: 3.24 to 12.98, P=0.000) were relative factors for death. Conclusions: The early and midterm outcomes of pulmonary artery banding as an initial palliative strategy is not satisfactory. Systemic ventricular outflow tract obstruction and total anomalous pulmonary venous connection are associated with high mortality.

1病例资料 女,3月龄,因"咳嗽伴发热1周"于2017年8月至广州医科大学附属广州市妇女儿童医疗中心(我院)就诊.出生时体检发现心脏杂音,当地医院诊断为"左冠状动脉-右室瘘、动脉导管未闭、卵圆孔未闭",未行处理.患儿平素无反复呼吸道感染;吃奶时有气促,有间断,无呛咳;大小便未见异常. 患儿系G1P1,足月剖宫产,无窒息抢救史,出生体重3000g.父母均体健,否认近亲结婚,否认遗传病史.家族成员无类似病史.

Objective: To investigate the efficacy of multistage surgery in patients with functional single ventricle (FSV) and risk factors of postoperative death. Methods: The clinical data of all consecutive patients with FSV undergoing multistage single ventricle palliation surgery in Guangzhou women and children's medical center from January 2008 to December 2017 were retrospectively reviewed. The study included 289 patients. The age was 10.0 (6.0, 35.4) months,and there were 198 male and 91 female patients. The patients were followed up at outpatient clinic. Survival rates were calculated with Kaplan-Meier. Multivariate Cox regression analysis was made to determine the risk factors of postoperative death. Results: Seventy-nine patients required the first stage palliation surgery, 232 patients received the Glenn shunt surgery, and 162 patients completed the Fontan procedure. Overall, postoperative death occurred in 39 patients including 21 after the first stage palliation surgery (early stage 13 cases, late stage 8 cases) , 10 after the Glenn shunt surgery (early stage 5 cases, late stage 5 cases) , and 8 following the Fontan procedure (early stage 6 cases, late stage 2 cases) . Kaplan-Meier analysis showed that survival rate of the entire cohort was 90.2% (95%CI 86.7%-93.7%) , 85.9% (95%CI 81.8%-90.0%) ,and 84.6% (95%CI 79.7%-89.5%) at 1 year, 5 years and 10 years post operation. Survival rate was 74.4% (95%CI 64.8%-84.0%) , 73.0% (95%CI 63.2%-82.8%) , and 73.0% (95%CI 63.2%-82.8%) at 1 year, 5 years, and 10 years post the first stage palliation surgery, 97.8% (95%CI 95.8%-99.7%) , 95.2% (95%CI 92.3%-98.1%) , and 95.2% (95%CI 92.3%-98.1%) at 1 year, 5 years, and 10 years post Glenn shunt surgery, 95.6% (95%CI 92.5%-98.7%) and 93.7% (95%CI 88.8%-98.6%) at 1 year and 5 years post Fontan surgery. Multivariate Cox regression analysis revealed that total anomalous pulmonary venous connection (HR=5.47, 95%CI 2.71-11.04, P<0.001), atrioventricular valve regurgitation more than moderate (HR=2.52, 95%CI 1.32-4.79, P=0.005), systemic ventricular outflow tract obstruction (HR=3.47, 95%CI 1.30-9.29, P=0.013), and required the first palliation surgery (HR=3.12, 95%CI 1.59-6.15, P=0.001) were risk factors of postoperative death. Conclusions: The multistage surgery can effectively improve the survival of patientswith functional single ventricle and is associated with satisfactory long-term prognosis. Total anomalous pulmonary venous connection, atrioventricular valve regurgitation more than moderate, systemic ventricular outflow tract obstruction, and required the first palliation surgery are risk factors of postoperative death in these patients.

Objective: To review the early and mid-term results of end-to-side anastomosis technique for interrupted aortic arch in neonates and infants. Methods: Clinic data of 46 patients were diagnosed as interrupted aortic arch in Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center between January 2010 and December 2016 were analyzed retrospectively. Twenty-six cases were neonates. The median age underwent surgery was 23 days (range: 2 days to 8 years). Anatomical subtypes included 36 cases of type A and 10 cases of type B. There was no type C case. The reconstruction of the aortic arch was completed by an extended end-to-side anastomosis technique between the descending aorta and the undersurface of the proximal aortic arch. In 42 patients, all with intracardiac anomalies, had concomitant complete repair of intracardiac anomalies through a median sternotomy. The remaining 4 patients, all without intracardiac anomalies, an end-to-side anastomosis was constructed through a left thoracotomy. During follow-up, aortic arch recurrent obstruction, left ventricular outflow tract obstruction (LVOTO) and tracheal stenosis were focused. Results: There were 3 surgical deaths, with amortality rate of 6.5%. The remaining 43 patients survived after surgery. In 39 of these patients, deep hypothermic cardiac arrest (DHCA) strategy was used for brain protection, and the mean time of DHCA was (16±3) minutes. Eight patients underwent delayed sternal closure. The mean mechanical ventilation time and ICU stay time were (3.4±1.6) days (range: 2 to 12 days) and (6.4±2.7) days (range: 3 to 16 days) respectively. In 16 patients, all with tracheal or bronchial stenosis before surgery, mechanical ventilation was successfully evacuated, and no new airway stenosis occurred. There was no residual pressure difference between upper and lower extremity arterial blood pressure at discharge. Echocardiography showed normal arterial blood flow velocity in aortic arch. At a mean follow-up of (36.2±18.9) months (range: 6 months to 7 years), there were two patients lost and one late date. Four patients developed a recurrent stenosis at the aortic arch, of which two were severe, and the other two were mild. In 2 patients, both with mild LVOTO before surgery, no significant increase in the degree of obstruction was found during the follow-up. Two patients developed new mild to moderate LVOTO without clinical symptoms, and continued to follow up. In all patients, the tracheal or bronchial stenosis were extenuated, and there was no new progressive airway stenosis by regular bronchoscopy. Conclusions: The end-to-side anastomosis technique for the reconstruction of the aortic arch achieved excellent early and mid-term results in neonates and infants suffered from interrupted aortic arch. Reducing the anastomotic tension by extensive mobilization is the key to prevent postoperative early complications and late recurrent arch obstruction.

Objective To explore the outcomes and risk factors for hospital mortality in low-weight neonates or small infants with congenital heart diseases.Methods Retrospective analysis were performed for low-weight neonates or small infants with congenital heart diseases during January 2011 and December 2016.Different groups were divided according to the criteria of RACHS-1.Single and multiple-factor correlations were analyzed between operative mortality and potential operative risk factors.Postoperative survival curves were plotted for all patients and those in different RACHS-1 ranks by Kaplan-Meier method,then differences between these curves were analyzed by Log-rank test.Results Among them,81 low-weight neonates or small infants had an operative mortality of 19/ 81(23.46％) while another 11 died during follow-ups.And 1,3,5-year postoperative survival rates were 65％,63％ and 58％ respectively.Both single-factor correlative and Logistic analyses indicated that operative age (days),preoperative mechanical ventilation,preoperative acidosis and palliative operation were correlated significantly with operative mortality.No significant difference existed in overall and individual comparisons of postoperative survival curves of different RACHS-1 groups.Conclusions Congenital heart diseases may be treated surgically in low-weight neonates or small infants.However,hospital mortality remains high.Operative age,preoperative mechanical ventilation,preoperative acidosis and palliative operation are independent operative risk factors for hospital mortality among low-weight neonates or small infants with congenital heart diseases.No significant difference exists in median-term survival rate among different RACHS-1 groups.