Objectives: Humanitarian program has emerged as a critical support mechanism for the management of congenital heart disease (CHD), yet its cost-effectiveness at the hospital level receives little investigation. Methods: We reviewed 2759 patients underwent CHD surgery at Fuwai Hospital, National Center for Cardiovascular Diseases from 2012 to 2022, and received financial aid from nongovernmental organizations. Analyzed data included baseline characteristics, surgical complexity, hospitalization costs, nongovernmental organization funding proportions, and disability-adjusted life years (DALYs) averted. Cost-effectiveness was calculated as funding expenditure per DALY averted, with adjustments for natural disease history and surgical outcomes. Results: Charitable fundings covered a median of 31% of hospitalization costs (¥36,564.34/USD $5424.98 per patient), averting 50.82 DALYs per patient at a cost of ¥952.19 ($141.27) per DALY. Neonates and complex CHD cases showed higher number of DALYs averted (59.44 and 54.42, respectively) but lower charitable funding proportions (24% and 32%, respectively). Patients with single-ventricle physiology and patients with previous surgeries exhibited relatively lower cost-effectiveness (¥1627.30 and ¥1349.08 per DALY). Unexpected reinterventions correlated with higher in-hospital mortality (9.6% vs 1%) and lower funding support (19% vs 33%). Conclusions: Charitable funding reduces financial barriers for surgical CHD treatment in China, demonstrating high cost-effectiveness. Funding models should evolve to incorporate surgical complexity and patient age, ensuring more reasonable support in the future. More importantly, the demonstrated cost-effectiveness should encourage more organizations to join the effort of funding CHD surgery, which is the most pivotal action at present.

OBJECTIVES:We aimed to summarize the clinical implications and outcomes of the Ross II procedure for infants and young children. METHODS:This study retrospectively reviewed consecutive paediatric patients who underwent Ross II procedure from 2011 to 2022 at Fuwai Hospital. Baseline, operative, postoperative characteristics, and outcomes were summarized. Postoperative all-cause mortality was defined as the primary outcome, and reoperation as the secondary outcome. RESULTS:Ten patients who underwent the Ross II procedure were included in the final analysis, with 2 cases of late mortality and 3 cases of reoperation during a median follow-up of 36 months (range: 3.5 months to 9 years). The median age at surgery was 1.8 years (interquartile range [IQR]: 0.65 to 3.5), and the median mitral annulus diameter was 16 mm (IQR: 15 to 18). Patients who underwent the Ross II procedure experienced a prolonged postoperative course, with a median intensive care unit stay of 11.5 days (IQR: 8.5 to 19.75) and postoperative hospital stay of 24 days (IQR: 20.25 to 34.75). Mitral stenosis emerged as the predominant late complication following the Ross II procedure, affecting 80% patients, as evidenced by the increase in mean gradient 3 years postoperatively compared with discharge. CONCLUSIONS:The Ross II procedure may represent a safe option for infants and young children when prosthetic valves are unavailable, despite the limited growth potential of the pulmonary autograft.

AbstractAimsHeritable dilated cardiomyopathy (DCM) or DCM associated with congenital or acquired left ventricular diseases carries a significant mortality risk. Pulmonary artery banding (PAB) has been proposed as an alternative to heart transplantation. This study aimed to delineate the clinical development, ventricular reverse remodelling, and functional regeneration of the dilated left ventricle, presenting as a pioneering approach in China.Methods and resultsThis prospective study was initiated in November 2021, involving paediatric patients with a significant dilated left ventricle and preserved right ventricle who underwent surgical PAB. The baseline characteristics and clinical information during follow‐up were collected. Seven patients (five boys) with a median age of 240 (148, 1028) days have been included thus far. No procedural or follow‐up mortality was observed. The modified Ross functional class improved from treatment to follow‐up of 348 (200, 629) days, and the median left ventricular ejection fraction increased from 27.0 (15.0, 34.0) % before surgery to 61.0 (52.0, 68.0) % (P < 0.05); the median left ventricular end‐diastolic diameter and corresponding Z‐scores decreased from 43.0 (40.0, 55.0) mm [+9.4 (+7.7, +11.7)] to 33.0 (29.0, 39.0) mm [+1.8 (+1.3, +3.8)] (P < 0.05). Functional regeneration of the left ventricle was observed in five patients. Three of them underwent balloon dilation of the PAB to relieve excessively elevated right ventricular pressures.ConclusionsThe application of PAB should adhere to strict criteria. Initial results are promising for infants and even toddlers with a dilated left ventricle and limited probability of spontaneous recovery. PAB can be an alternative when there is a shortage of donor transplants and assist devices, especially for low‐ and middle‐income countries.

Congenital tracheal stenosis (CTS) is a rare congenital malformation.And 69% of CTS children also suffer from congenital heart diseases (CHDs). Proper management of dual malformations has been a focus of clinical attention.With a rapid pace of technical development, the diagnosis and treatment of such patients have also changed.This systematic review summarized the diagnosis and treatment of CHD children with CTS.It was intended to provide clinical references and assist cardiothoracic surgeons in the proper management of CTS.

Background:The modified L-shaped incision technique (MLIT) was successfully applied to the repair of supracardiac total anomalous pulmonary venous connection (TAPVC) with promising mid-term outcomes. It is, however, unclear whether or not MLIT could be an alternative to sutureless technique (ST).Methods:All patients (n=141) who underwent MLIT or ST repair for supracardiac TAPVC between June 2009 and June 2022 were included and a propensity score-matched analysis was performed to reduce the heterogeneity.Results:MLIT was performed in 80.9% (114/141), whereas ST was performed in 19.1% (27/141). Patients who underwent MLIT repair had a lower incidence of pulmonary veinous obstruction (PVO)-related reintervention (1.8 vs. 18.5%, P=0.002), and late mortality (2.6 vs. 18.2%, P=0.006). Overall survival at 10 years was 92.5% (87.7-97.7%) for MLIT and 66.8% (44.4-100%) for ST (P=0.012). Freedom from postoperative PVO at 10 years was 89.1% (83.2-95.5%) for MLIT and 79.9% (65.6-97.4%) for ST (P=0.12). Cox proportional hazards regression identified prolonged mechanical ventilation duration, postoperative PVO, respiratory dysfunction, and low cardiac output syndrome were associated with postoperative death and PVO-related reintervention.Conclusions:The MLIT strategy is a safe, technologically feasible, and effective approach for supracardiac TAPVC, which is associated with more favorable and promising freedom from death and PVO-related reintervention.

OBJECTIVES Coronary artery atresia is a rare coronary artery anomaly in children and has a high rate of misdiagnosis. We aimed to summarize the profile and early outcomes after the surgical reconstruction of coronary artery atresia in children. METHODS A retrospective analysis was performed in 12 consecutive patients with coronary artery atresia who were admitted to the Department of Paediatric Cardiac Surgery of Fuwai Hospital between October 2016 and September 2020. Ten patients underwent surgical reconstruction of the coronary artery with the pulmonary artery anterior wall, and 8 patients underwent concomitant mitral valvuloplasty. RESULTS There were 6 females and 6 males, with an age of 1.75 years [interquartile range (IQR), 1.0-3.5] and weight of 10.0 kg (IQR, 8.9-14.75). There were 10 cases of left coronary artery atresia and 2 cases of right coronary artery atresia. All the patients were initially misdiagnosed in the outpatient clinic, but further nonselective coronary angiography confirmed the diagnosis of coronary artery atresia. In all 10 patients with mitral regurgitation, echocardiogram of the mitral valve chordae and papillary muscle revealed ischaemic changes. The clamp time was 89.0 min (IQR, 75.0-101.0), the pump time was 126.0 min (IQR, 119.0-132.0) and the intensive care unit stay time was 1.5 days (IQR, 1.0-3.0). No perioperative deaths were noted. After 9.5 months (IQR, 5.5-13.5) of follow-up, 2 patients with fractional shortening that significantly decreased to 14.1% and 14.8% died and 1 patient had moderate pericardial effusion that improved after treatment with oral diuretics. Coronary artery ultrasound and enhanced computed tomography showed a patent main coronary artery in all the patients. CONCLUSIONS Coronary artery atresia in children is often associated with mitral regurgitation, and mitral valve chordae and papillary muscle exhibit ischaemic changes. Coronary artery reconstruction is safe and effective in children with coronary artery atresia.

Acute myocardial infarction (AMI) is a severe and even fatal cardiovascular disease. The effect of transcription factors on AMI is intensively explored. Our experiment attempts to probe the role of Sox9 in cardiomyocyte apoptosis after AMI. AMI cell model was established in AC16 cells by hypoxia treatment. Cell viability and apoptosis were assessed. Then, the levels of BAX, Bcl-2, Sox9, miR-223-3p, and MEF2C were detected. The binding relation between Sox9 and miR-223-3p and between miR-223-3p and MEF2C was verified. The expression of miR-223-3p was upregulated using the miR-223-3p mimic, and collaborative experiments were conducted as si-Sox9 or si-MEF2C was transfected into cells to inhibit the expression of Sox9 or MEF2C. Sox9 was highly expressed in cardiomyocyte apoptosis after hypoxia, while Sox9 silencing protected hypoxia-treated cardiomyocytes from apoptosis by enhancing cell viability, quenching apoptosis, and reducing activity of caspase-3 and caspase-9. Essentially, Sox9 bound to the miR-223-3p promoter region to upregulate its expression. miR-223-3p targeted MEF2C transcription. miR-223-3p overexpression and MEF2C silencing could counteract the suppressive role of Sox9 silencing in hypoxia-treated cardiomyocyte apoptosis. Sox9 exacerbated hypoxia-induced cardiomyocyte apoptosis by promoting miR-223-3p expression and inhibiting MEF2C transcription.

目的 总结阜外医院标准化二尖瓣修复策略治疗完全房室间隔缺损(CAVSD)术后严重左侧房室瓣反流(LAVVR)的应用经验.方法 回顾性分析2016-2019年于我院通过标准化二尖瓣修复策略治疗CAVSD术后LAVVR的11例患者临床资料,其中男5例、女6例,中位年龄56(22～ 152)个月.总结二尖瓣大量反流病理特征、术中操作要点及近中期术后随访结果.结果 与初次手术时间间隔48(8～ 149)个月,主动脉阻断时间(54.6士21.5)min,体外循环时间(107.4士38.1)min,呼吸机使用时间(16.4士16.3)h.术后恢复顺利,无死亡病例.随访(29.0±-12.8)个月,超声心动图提示二尖瓣微少量反流5例,少量反流5例,中量反流1例.心功能分级(NYHA)均为Ⅰ级.结论 标准化二尖瓣修复策略治疗CAVSD术后大量LAVVR安全有效,近中期随访结果良好.

目的 总结超过手术建议年龄的完全房室间隔缺损(complete atrioventricular septal defect,CAVSD)患儿外科治疗的效果及经验.方法 回顾性分析2002～ 2013年163例7周岁以下单纯CAVSD患儿的临床资料,根据是否＞1周岁分为适龄组[n=84,其中男37例、女46例,平均年龄(7.6±2.7)个月]与超龄组[n=79,其中男30例、女49例,平均年龄(34.6±19.6)个月].结果 两组患者在平均主动脉阻断时间[(88.3±24.4)min vs.(106.1±35.4)min,P＜0.001]、平均体外循环时间[(123.6±31.1)min vs.(142.6±47.1)min,P=0.003]方面差异有统计学意义.术后随访过程中[适龄组(53.3±43.9)个月,超龄组(57.2±48.2)个月],两组在全因死亡率(10.7％ vs.8.9％,P=0.691)、中重度左侧房室瓣反流发生率(16.7％ vs.21.5％,P=0.430)及再干预率(3.6％ vs.0.0％,P=0.266)方面差异无统计学意义.两组均无左室流出道梗阻及房室传导阻滞发生.结论 经过合理的外科治疗,超过建议矫治年龄的患儿也可取得令人满意的结果.

目的 探索合并异位心及左室流出道狭窄的矫正型大动脉转位患者姑息性分流术后的二次手术治疗策略.方法 回顾性分析2011年6月至2019年5月连续性收治的姑息性分流术后合并异位心及左室流出道狭窄的矫正型大动脉转位54例患者的临床资料.所有患者均双心室发育均衡,按手术方式分为两组:一个半心室组,行一个半心室矫治术者24例,男16例、女8例,平均年龄(5.4±2.2)岁;单心室组,行单心室矫治术者30例,男19例、女11例,平均年龄(8.6±6.2)岁.通过电话随访并预约复查获取随访资料.结果 术前两组间体循环系统瓣膜反流率和体循环系统心室射血分数差异无统计学意义.单心室组的体外循环时间、主动脉阻断时间和机械通气时间均显著短于一个半心室组,但迁延性胸腔积液发生率明显高于一个半心室组.无院内死亡.一个半心室组中位随访时间49(17～83)个月,随访率93.9％,随访死亡1例.单心室组中位随访时间47(12～85)个月,随访率90.9％,随访死亡1例.随访终点时一个半心室组体循环系统心室射血分数显著性高于单心室组,而且心功能分级(NYHA)Ⅲ级和Ⅳ级比例低于单心室组,差异有统计学意义(P＜0.05).两组术后生存率及免除再干预率差异无统计学意义(P＞0.05).结论 对于姑息性分流术后的合并异位心及左室流出道狭窄的矫正型大动脉转位患者,一个半心室矫治术是理想的二次手术治疗策略选择.

Objective:To explore the anatomic repair strategy for congenital corrected transposition of great arteries (ccTGA).Methods:At the retrospective study, from August 2004 to May 2019, all 120 consecutive ccTGA were included and all accepted anatomic repair. There were 36 cases with with left ventricular outlet obstruction(LVOTO) and cardiac malpositon [ages(4.6±2.2) years, weight(17.7±5.9)kg] underwent the one and a half ventricle repair(hemi-Mustard and bidirectional Glenn procedures combined with the Rastelli), 49 cases[ages(3.4±2.7) years, weight(17.7±11.4)kg] underwent double switch operation(Great artery swtich with Senning operation), 24 cases [ages(5.7±4.3) years, weight(19.1±8.6)kg] with LVOTO and ventricular sept defect(VSD) accepted the Rastelli with Senning operation, and 14 cases with LVOTO and remote VSD [ages(6.9±4.8) years, weight(23.0±12.9)kg] accepted the Double root transposition(DRT) with Senning operation. Follow up data were collected by telephone interviews and echo. The median follow-up time were 49 months varied from 20 to 84 months, 46 months varied from 18 to 108 months, 35 months varied from 7 to 84 months and 98 months varied from 72 to 145 months. Statistical analysis was performed with SPSS 19.0.Results:There were 6 in-hospital deaths and 2 follow-up deaths. The survival probability were(84.0±6.0)% and(84.0±6.0)% at 5 and 10 years after operation. The probability of freedom from re-intervention were(95.0±11.8)% and(89.0±11.8)% at 5 and 10 years after operation. All 6 patients need implant pacemaker for Ⅲ A-V block. Seven patients had moderate or more than moderate tricuspid regurgitation. The left ventricular(systemic ventricle) EF were 0.61±0.09, 0.63±0.08, 0.59±0.01 and 0.65±0.07 in one and a half ventricle repair group, double switch(AS group), Rastelli with Senning(RS group) and DRT with Senning(DS group) patients. There were 1 heart failure in one and a half ventricle repair group, 1 in AS group and 1 in RS group. For 36 pure ccTGA patients, compared with direct double switch patients these patients accepting double switch after pulmonary banding(PAB) had more EF(0.54±0.09 vs. 0.65±0.08, P=0.00). There were significantly less patients need re-operation in one and a half ventricle repair group compared with RS group(0 vs. 13.6%, P=0.03). Conclusion:For ccTGA/LVOTO/cardiac malpositon, the one and a half ventricle repair was ideal strategy with significant less RV-PA conduit stenosis and re-operation. For pure ccTGA patients, second staged double switch after PAB had better long-term heart function. For ccTGA/ LVOTO/ remote VSD patients DRT with Senning was ideal strategy.

目的 探讨Ozaki自体心包主动脉瓣成形术行小儿主动脉瓣成形的近中期治疗效果.方法 回顾性分析我院2017年5月至2021年4月间15例行Ozaki手术患儿的临床资料,其中男9例,女6例,平均手术年龄(4.9±1.4)岁.主动脉瓣狭窄2例,主动脉瓣反流13例,均使用Ozaki方法重建主动脉瓣单个或多个瓣叶.术前及术后行超声分别评估主动脉瓣功能及左心室功能.结果 围手术期患儿无死亡,无因修复失败而术中即刻改行主动脉瓣置换病例.平均随访(17.7±15.0)个月,无血栓等不良并发症,无患儿死亡.结论 采用Ozaki手术重建小儿主动脉瓣叶的近中期手术疗效良好,瓣膜功能持久性可待长期随访明确.

目的 分析低龄重度主动脉瓣狭窄婴儿的病变特征及手术治疗结果.方法 选择2012年8月至2019年12月于我院行主动脉瓣交界切开术低龄婴儿(年龄≤90d)28例,其中男22例、女6例,年龄62.00(47.00,82.50)d.分析患者病变特征、手术及随访结果.结果 20例(71.43％)患者瓣叶病理为二叶瓣.术前5例(17.86％)患者心力衰竭,2例(7.14％)使用前列腺素.术后呼吸机使用时间25.00(17.00,62.75)h,住ICU时间3.50(2.00,8.50)d,术后住院时间10.00(7.00,16.50)d.延迟恢复(住ICU时间＞14 d)4例(14.29％),死亡1例(3.57％).术后随访55.00(43.25,82.25)个月,随访期间无死亡,4例(14.81％)患者再次入院行手术治疗,其中3例(11.11％)为主动脉瓣重度狭窄、1例(3.70％)为主动脉瓣重度关闭不全.结论 低龄重度主动脉瓣狭窄婴儿病情重,术后恢复时间长,需尽早手术,术后随访结果满意.

Objective:To summarize the surgical treatments of deep sternal wound infection (DSWI) in children aged under 7 years after pediatric cardiac surgery.Methods:From January 2010 to December 2019, a total of 31 866 children underwent cardiac surgery through a median sternal incision.There were 387 neonates.Among them, 0.15%(47/31 866) developed deep incision infection involving sternum.The incidence of DSWI in children of different ages was 2.33%(9/387) for neonates, 0.15%(6/4 018) for children aged 2-6 months, 0.09% for children aged 6-12 months (6/7 010), 0.13% (15/11 511) for children aged 1-3 and 0.12%(11/8 940) for children aged 3-7.Among 47 children, there are 30 boys and 17 girls; their age was (1.98±2.03) years; their weight (10.55±6.24) kg.Complex congenital heart disease accounted for 75% of all cases.There were 9 neonates (19.1%), 21 infants (44.7%) aged under 1 year, 14 infants (29.8%) with wound infection and debridement within 15 days after an initial operation, 11 infants (23.4%) with wound infection and debridement 15 to 30 days after an initial operation and 22 infants (46.8%) over 30 days.Results:The incidence rate of postoperative deep incision infection in newborns was significantly higher than that in children of other ages, and the difference had statistical significance ( P=0). All 47 children had a median sternotomy, and the original surgical incision was opened for drainage and dressing change immediately after the diagnosis of infection. After the wound was clean and granulation was fresh, they were sent to the operating room for debridement surgery. The time from debridement surgery to the last cardiac surgery was (126.89±310.53) d, ranging from 4 to 1997 days. Among them, the children with infection time >30 days who underwent debridement surgery had a high recurrence rate of infection, 50.0% (11/22) required multiple debridement, and only 59.1% (13/22) of the children were able to preserve the sternum. In this study, 29.8% (14/47) of the children required more than two debridement procedures in the hospital; 25.5% (12/47) of the children underwent pectoralis major muscle flap transfer surgery due to severe infection that could not preserve the sternum; and 14.9% (7/47) of the children used closed continuous vacuum aspiration therapy before debridement surgery due to severe exudation of the infection or inability to cooperate with bedside dressing change. Bacteriological culture was performed on the wound secretions of 31 children, of which 18 cultured Gram-positive cocci, mainly Staphylococcus epidermidis and Staphylococcus aureus, 9 did not detect pathogenic bacteria, and the remaining 4 cultured Gram-negative bacilli. There were no hospital deaths among the children in this study. One child required continuous dressing change after discharge due to sinus formation; one child had cardiac rupture and bleeding during debridement, and the chest was successfully closed after intraoperative compression hemostasis, and the child was recovered and discharged; no serious complications occurred in other children. Conclusions:Higher attention should be paid to treatment, early detection and diagnosis, thorough drainage, early debridement and avoiding other serious complications caused by the expansion of infection range are vital for a successful treatment.

目的:总结分析室间隔缺损介入封堵术后并发症的发生情况及外科治疗的效果.方法:选择2016年5月至2019年12月因室间隔缺损介入封堵术后并发症就诊于我院并行外科手术治疗的患者14例,分析室间隔缺损介入封堵术后并发症的发生情况、手术方法的选择、手术结果及随访结果.结果:患者室间隔缺损介入封堵术后并发症的发生包括:瓣膜损伤9例(三尖瓣、主动脉瓣损伤分别有5例和6例,其中三尖瓣和主动脉瓣同时损伤2例),左心室流出道梗阻1例,心律失常6例(三度房室阻滞2例、完全性左束支阻滞4例),溶血1例.所有患者均于体外循环下行室间隔缺损封堵器取出术和室间隔缺损修补术;另外,5例患者行主动脉瓣成形术,1例行主动脉瓣置换术,9例行三尖瓣成形术,2例植入永久起搏器.术后重症监护室住院时间为19.00(13.00,46.75)h,呼吸机使用时间为6.00(3.00,9.25)h,总住院时间为7.00(6.00,9.50)d.术后中位随访18.00(7.75,38.00)个月,所有患者室间隔缺损无残余分流,无瓣膜功能不良,植入起搏器的患者起搏器功能良好.结论:室间隔缺损封堵术后需外科手术治疗的并发症较多样,应以预防为主,一旦出现需早期外科干预;外科干预效果良好.

Background. The outcomes, therapeutic strategies, and risk factors of congenital corrected transposition of great arteries (ccTGA) unoperated before adulthood are unclear. Methods. From October 2009 to January 2018, 117 adult ccTGA patients, classified into ccTGA with intact ventricular septum, ventricular septum defect, and pulmonary valve or subpulmonary outflow tract stenosis (PS) groups, were reviewed. Statistical analysis was performed with SPSS 19.0 (IBM, Armonk, NY). Results. At the first visit, no patients suffered operation. The PS group had the least systemic atrioventricular valve regurgitation and the greatest systemic ventricular ejection fraction. All 49 patients underwent surgery. From the first visit to last follow-up, systemic ventricular ejection fraction of unoperated ccTGA decreased significantly. In the intact ventricular septum group, patients receiving systemic atrioventricular valve replacement/valvuloplasty had a significantly increased systemic ventricular ejection fraction and statistically more freedom from death and transplant than unoperated. In the ventricular septum defect group the late systemic ventricular ejection fraction of operated patients was not statistically different from their basic data at first visit. In the PS group patients receiving physiologic repair had significantly decreased systemic ventricular ejection fractions. Severe systemic atrioventricular valve regurgitation, physiologic repair, and systemic ventricular dysfunction (ejection fraction <40%) were risk factors for mortality, transplant, and congestive heart failure. Conclusions. PS protects against systemic atrioventricular valve regurgitation and ventricular dysfunction. Systemic atrioventricular valve replacement/valvuloplasty improved systemic ventricular function for ccTGA with an intact ventricular septum. Physiologic repair was not ideal for ccTGA with PS. Severe systemic atrioventricular valve regurgitation and systemic ventricular dysfunction were associated with suboptimal outcomes. (C) 2021 by The Society of Thoracic Surgeons

目的 分析经右心室流出道肺动脉瓣球囊成形术(surgical balloon valvuloplasty,SBV)进行右心室减压联合个体化分流手术治疗室间隔完整型肺动脉闭锁(pulmonary atresia with intact ventricular septum,PA/IVS)的中远期疗效.方法 回顾性分析2005年1月至2017年12月在本中心接受SBV治疗的91例PA/IVS患者的临床资料,其中男52例(57.1％)、女39例(42.9％),中位年龄3个月(1d,24个月),中位体重4.1 (2.5,12.0) kg.结果 所有患者均行SBV,其中62例患者同期行其他外科手术,包括动脉导管未闭(patent ductus arteriosus,PDA)结扎术33例;PDA结扎术+改良Blalock-Taussig分流术23例;PDA结扎术+双向格林手术6例.没有早期死亡.中位随访时间8.8 (2.5,13.4)年,4例(4.4％)失访.随访患者中,7例(8.0％)患者死亡,1例(1.1％)患者因肺动脉瓣狭窄再次行SBV.在随访中,5例(5.7％)行一个半心室矫治术,2例(2.3％)行Fontan手术;平均三尖瓣Z值为-1.7±1.5,较术前明显增加(t=5.587,P＜0.001).结论 通过SBV联合个体化分流手术策略治疗PA/IVS是安全、有效的,大多数患儿可达到双心室矫治,避免单心室姑息治疗.

目的:总结应用可吸收聚左旋乳酸(PLLA)微骨板外固定术治疗先天性心脏病(先心病)合并支气管软化患儿的经验.方法:回顾性分析2017年12月至2019年1月,在我中心接受可吸收PLLA微骨板外固定术治疗先心病合并支气管软化的患儿8例,其中男性2例,女性6例.平均年龄(8.4±4.1)个月,平均体重(6.5±1.2)kg.观察术后机械通气时间、重症监护病房(ICU)时间、呼吸系统并发症等评估治疗效果.并于术后1、3、6、12个月复查超声心动图和胸部CT.结果:8例患儿中7例成功接受同期心脏畸形矫治和支气管可吸收PLLA微骨板外固定术,1例成功接受单纯支气管可吸收PLLA微骨板外固定手术.全组无围术期死亡,术后呼吸机辅助时间(24.4±23.7)小时,ICU时间(3.8±3.8)天.平均随访时间(6.5±6.2)个月,超声心动图显示所有患者心脏畸形矫治满意,CT显示支气管通畅.结论:采用可吸收PLLA微骨板外固定术治疗先心病合并支气管软化简便易行,治疗效果明显,可显著缩短机械通气时间及ICU时间,降低费用.近期效果满意.

目的 探索黏着斑激酶(FAK)及其介导下游信号通路AKT/S6K在心房颤动(房颤)小鼠模型心房重构中分子改变.方法 利用为心脏特异性表达人源肾素原受体(pRR)的转基因小鼠作为房颤小鼠模型,选取野生型C57BL/6J小鼠为对照组,两组各选取16例8月龄小鼠作为研究对象,通过超声心动图检测观察心房心室改变;获取心房组织,分别进行Masson病理染色和蛋白免疫印迹,观察两组纤维化程度和FAK及其下游通路在两组小鼠心房组织中的变化.结果 房颤模型小鼠在8月龄已经出现明显的持续性房颤,超声心动图提示房颤组小鼠心房和心室都较对照组小鼠扩大,有心房重构.病理提示房颤组小鼠心房纤维化程度较高,同时FAK及下游AKT/S6K通路蛋白的磷酸化表达均发生上调.结论 心房重构是房颤引起的心肌组织结构的变化,为房颤持续和血栓形成提供基质.房颤可引起心房扩大和纤维化形成,其中FAK介导的AKT/SK通路可能是参与重构的重要分子.

To introduce a standardized strategy and reproducible procedures of mitral repair for mitral regurgitation in the pediatric population with leaflet plication as a principal technique. Consecutive patients who had undergone mitral repair by our standardized repair-oriented strategy in our institution from January 2016 to December 2019 were included retrospectively. The standardized repair strategy included 3-step inspections and repair from the subvalvular to leaflet, and then to the annular level. The main surgical techniques included chordae detachment, papillary muscle splitting, leaflet plication, and posterior annuloplasty. The indication for leaflet plication was that the distance between 2 adjacent chordae tendineae was greater than 4 mm. A total of 113 patients were enrolled. During 22.6-month (range, 2-50 months) follow-up period, primary endpoint was documented in 15 (13.3%) patients, including 1 (0.9%) death, 0 transplantation, and 14 (12.4%) functional mitral failure. Freedom form primary endpoints at 6 months, 1 year, and 3 years was 94.7%, 94.7%, and 82.3%, respectively. Significant independent predictors of functional mitral valve failure were younger age (hazard ratio [HR], 0.28; 95% confidence interval [CI], 0.04-0.72; P = 0.037) and ischemic mitral regurgitation (MR) (HR, 24.34; 95% CI, 4.52-47.33; P < 0.001). Leaflet plication was significantly associated with well-functioned mitral valve (HR, 7.42; 95% CI, 2.35-30.54; P = 0.004). Compared with nonischemic MR group, ischemic MR group was noted with higher occurrence of primary endpoint events (11/28 vs 4/85, P < 0.001). The short- to mid-term outcomes of standardized mitral repair technique with leaflet plication were favorable, among which, however, repair for mitral regurgitation with ischemic lesions is comparatively challenging.