BACKGROUND:Infective endocarditis (IE) with irreversible structural damage has high mortality despite conventional surgery. Heart transplantation (HTx) remains underused in active IE owing to guideline limitations and historical contraindications. CASE SUMMARY:A 61-year-old man with recurrent prosthetic valve IE (aortic/tricuspid vegetations, perivalvular abscesses, and coronary embolism) developed cardiogenic shock. Multidisciplinary evaluation deemed conventional repair futile. Emergency HTx achieved survival, with no infection recurrence at 4-month follow-up under prolonged antibiotics and immunosuppression. DISCUSSION:Whereas guidelines reserve HTx for healed IE, recent evidence supports its role in active infection with controlled bacteremia. This case highlights feasibility of HTx in anatomically destructive IE, urging guideline updates to prioritize transplantation over high-risk valve-centric approaches. TAKE-HOME MESSAGES:HTx may represent a last-resort option for end-stage IE with irreparable damage, even in active infection. This exceptional case required both multidisciplinary consensus and timely organ availability. Multidisciplinary protocols integrating prolonged antibiotics and tailored immunosuppression are critical for success.

AbstractBackgroundVein grafts are commonly employed in revascularisation surgery for multivessel coronary artery disease, yet neointimal hyperplasia (NIH) remains a critical impediment to the long‐term patency of these grafts. Despite this, effective methods to precisely identify and target interventions for the neointima are still inadequate.MethodsIn this study, Sprague–Dawley (SD) rats were used to establish an external jugular vein transplantation model, and the NIH pathophysiological process was tracked across 11 time points (0–35 days) using various histological stains. Spatial transcriptomics was performed on normal veins and 19‐day grafts to explore gene expression in neointimal regions. Immunohistochemical analysis identified neointima‐specific markers, while NIH progression was assessed in SD rats with four and a half LIM domains protein 1 (Fhl1) knockout and in human saphenous veins (HSV) with adenovirus‐mediated Fhl1 overexpression.ResultsTypical neointimal formation commenced by day 11 postgrafting and peaked at day 19. Neointimal cells originated from newly generated α‐SMA(+) repair cells located outside the grafted vein, displaying a hybrid fibroblast‐smooth muscle cell phenotype. Spatial transcriptomics identified stable and sustained Fhl1 expression within the neointima throughout the entire NIH phase. Systemic knockout of Fhl1 in SD rats via the phosphoinositide 3‐kinase pathway exacerbated graft inflammation, heightened cell proliferation, and accelerated NIH. Conversely, FHL1 overexpression in cultured HSV suppressed NIH.ConclusionThese findings indicate that, following grafting into the arterial system, the newly formed repair cells external to the grafted vein play a pivotal role in NIH, with neointimal cells exhibiting stable and continuous Fhl1 expression. Fhl1 serves as a protective factor against NIH both in vivo and in HSV, likely due to its anti‐inflammatory and anti‐proliferative effects.Key points This study firstly used spatial transcriptomics technique to analyse the neointima and generated a specific neointimal transcriptomic atlas. Fhl1 exhibits specific and stable expression in the spatial region of the neointima. It has thus far the highest enrichment of expression in the neointima in NIH phases, suggesting that it is a prominent molecular biomarker of neointima. We generated rats with a Fhl1 deletion and found that insufficient Fhl1 expression caused an increase in the severity of vascular inflammation and proliferation during neointimal hyperplasia. Adenovirus‐mediated FHL1 overexpression in human saphenous vein have beneficial effects in preventing neointimal hyperplasia. These highlight its potential as a therapeutic target for mitigating vein graft failure associated with cardiovascular procedures. Spatial transcriptomics profiles and morphological observations demonstrated that a newly generated cell population outside the grafted vein with hybrid phenotype between SMCs and fibroblasts contributes to neointimal formation.

China has the largest number of individuals with congenital heart disease (CHD) in the world and a heavy burden of CHD. Therefore, understanding current CHD treatment outcomes and patterns in China will contribute to global progress in CHD treatment and be a valuable experience. Generally, CHD treatment in China has satisfactory outcomes owing to the joint efforts by all relevant stakeholders across the country. However, efforts are needed to overcome the remaining challenges: management of mitral valve disease and paediatric end-stage heart failure needs to be improved; cohesive paediatric cardiology teams should be established and collaboration between hospitals enhanced; CHD-related medical resources need to be more accessible and equitable; and nationwide CHD databases should be enhanced. In the second paper of this Series, we aim to systematically summarise the current CHD treatment outcomes in China, discuss potential solutions, and provide future perspectives.

OBJECTIVES: Risk factors associated with adverse cardiac events (cardiac AEs) after pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot are incompletely understood. In this study, we aimed to determine the relationship between histological myocardial fibrosis and cardiac AEs after PVR in patients with rTOF.METHODS: We consecutively collected clinical, cardiac magnetic resonance, echocardiography and electrocardiogram data of 51 patients with rTOF who underwent surgical PVR. The right ventricular outflow tract tissue was collected during the PVR and the degree of histological myocardial fibrosis was determined by a tailor-made automated image analysis method of picrosirius red staining.RESULTS: The median follow-up time was 4.9 years, and 14 patients had cardiac AEs (a composite of heart failure admission and arrhythmia) during follow-up. The total analysis area of myocardial samples was 5782.18 mm(2), and the median percentage of myocardial fibrosis was 20.6% (interquartile range 16.7-27.0%), which were significantly elevated in patients with cardiac AEs compared with patients without cardiac AEs (24.1% vs 19.7%, P = 0.007). Right ventricular ejection fraction and left ventricular end-systolic volume index were significantly associated with myocardial fibrosis in multivariable stepwise linear regression analysis (R-2 = 0.238). Cox proportional hazards regression identified degree of myocardial fibrosis [hazard ratio 1.127; 95% confidence interval (CI) 1.047-1.213; P = 0.001] and age at PVR (hazard ratio 1.062; 95% CI 1.010-1.116; P = 0.019) were associated with increased risk of cardiac AEs. The incidence of adverse cardiac events was significantly increased when myocardial fibrosis >20.1% and age at PVR >18.2 years.CONCLUSIONS: Histological myocardial fibrosis was associated with biventricular systolic functions in rTOF. Higher myocardial fibrosis and older age at PVR are independent risk factors for the adverse cardiac events after PVR in patients with rTOF.

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.

Background: Atrial appendage tachycardia (AAT) originating from the atrial appendage (AA) is extremely difficult to eliminate using radiofrequency catheter ablation (RFCA). The optimal management strategy for AAT refractory to RFCA remains unclear. Objective: This study aims to investigate the long-term result of ablative therapy and the optimal alternative management for AAT refractory to RFCA. Methods: A total of 51 patients with AAT originating from the AA undergoing RFCA were recruited. Video-assisted atrial appendectomy and oral ivabradine were performed on those with AATs refractory to RFCA, and this study aimed to evaluate their safety and long-term efficacy. Results: We included 51 patients (51/586, 8.7%) with AATs confirmed by activation mapping and contrast venography. Among them, there were 28 (54.9%) AATs originating from the distal AA. In total, 14 (27.4%) AATs were refractory to RFCA, including 13 originating from the distal AA and one arising from the proximal AA. Ten of 11 (90.9%) AATs originating from the distal AA were eliminated after an atrial appendectomy, and the other three AATs were suppressed using oral ivabradine. Origins from the distal AA refractory to RFCA and early age of AAT onset ≤26.5 years indicated the need for atrial appendectomy. No major complications occurred, and nine patients with tachycardia-induced cardiomyopathy fully recovered. Long-term success was achieved in 98.0% of patients with multiple treatment managements. Conclusion: AATs originating from the distal AA were more refractory to RFCA. RFCA was the cornerstone of AAT catheter ablation. Video-assisted thoracoscopic atrial appendectomy was an effective strategy for those origins at the distal AA and the age of AAT onset ≤26.5 years. Ivabradine represents a promising treatment for AAT temporarily in pediatric and young adult patients.

PurposeSeveral adults with repaired tetralogy of Fallot (rToF) undergo pulmonary valve replacement (PVR) to reduce the right ventricular volume and retain right ventricular function. However, there is currently no consensus on the ideal time for PVR surgery in asymptomatic patients with rTOF with pulmonary regurgitation (PR). Clinical outcomes after PVR are also indeterminate. Recently, myocardial strain and strain rate derived from cardiac magnetic resonance (CMR) feature tracking were found to be more sensitive to right ventricular dysfunction than conventional parameters and therefore may add prognostic value in patients with rToF. We aimed to analyze whether pre-PVR left ventricular (LV) strain and strain rate detected by CMR feature tracking are associated with midterm outcomes after PVR in patients with rToF.MethodsSeventy-eight asymptomatic patients with rToF who required PVR due to moderate or severe PR were prospectively enrolled between January 2014 and June 2020. CMR cine sequences were obtained, and feature tracking parameters were measured preoperatively. Adverse events were documented during the follow-up. Receiver operating characteristic analysis was performed to determine the cutoff value. Kaplan–Meier curves were drawn with log-rank statistics; moreover, univariate and multivariate Cox proportional hazards regression analyses and Harrel C-indices were analyzed.ResultsDuring 3.6 ± 1.8 years of follow-up, 25 adverse events were recorded. Kaplan–Meier survival curves and univariate Cox analysis verified that patients with significantly reduced radial strain (RS), circumferential strain (CS), longitudinal strain (LS), RS rate at systole and diastole (RSRs and RSRe), and circumferential and LS rates at diastole (CSRe and LSRe) had worse event-free survival. After multivariate correction, only LS and LSRe remained significantly associated with adverse outcomes (hazard ratio = 1.243 [1.083–1.428] and 0.067 [0.017–0.258], respectively, all p < 0.05). The cutoff values of LS and LSRe were −12.30 (%) and 1.07 (s–1), respectively.ConclusionThe LV strain and strain rate prior to PVR are important prognostic factors for adverse events after PVR in rToF.

Background:Pulmonary regurgitation (PR), though well tolerated for short term in patients with repaired tetralogy of Fallot (rTOF), could lead to right ventricular (RV) dysfunction, arrhythmias, and sudden cardiac death. Pulmonary valve replacement (PVR), considered as the gold-standard treatment for PR, is performed to mitigate these late effects. In this study, we aimed to evaluate the midterm outcomes and predictors of adverse clinical outcomes (ACO).MethodsFrom May 2014 to December 2017, 42 patients with rTOF undergoing surgical or transcatheter PVR in our department were retrospectively included. Cardiovascular magnetic resonance was performed before PVR (pre-PVR), early after PVR (early post-PVR), and midterm after PVR (midterm post-PVR). Medical history and individual data were collected from medical records. ACO included all-cause death, new-onset arrhythmia, prosthetic valve failure, and repeat PVR.ResultsThe median follow-up duration was 4.7 years. PVR was performed at a median age of 21.6 years. There was no early or late death. Freedom from ACO at 3 and 5 years was 88.1 ± 5% and 58.2 ± 9%, respectively. RV end-diastolic volume index (RVEDVI) and end-systolic volume index (RVESVI) significantly reduced early after PVR and further decreased by midterm follow-up (pre-PVR vs. early post-PVR vs. midterm post-PVR: RVEDVI, 155.2 ± 34.7 vs. 103.8 ± 31.2 vs. 95.1 ± 28.6 ml/m2, p < 0.001; RVESVI, 102.9 ± 28.5 vs. 65.4 ± 28.2 vs. 57.7 ± 23.4 ml/m2, p < 0.001). Multivariable analysis revealed that the occurrence of ACO was significantly increased in patients with lower left ventricular end-systolic volume index.ConclusionsA significant reduction of RV volume occurred early after PVR, followed by a further improvement of biventricular function by midterm follow-up. The midterm freedom from ACO was favorable.

BACKGROUND Surgical outcomes for primary pulmonary vein stenosis (PPVS) remain unfavorable, and risk factors are still poorly understood. This study evaluated outcomes and risk factors after PPVS repair. METHODS This retrospective study included 40 patients with PPVS who underwent surgical repair in Fuwai Hospital from 2010 to 2020. Adverse outcomes included overall death, pulmonary vein (PV) restenosis, and reintervention. A univariate and multivariate risk analysis was performed to determine risk factors. RESULTS The mean follow-up duration was 37.5 +/- 31.5 months. Sutureless technique was performed in 7 patients (17.5%), endovenectomy in 9 (22.5%), and patch venoplasty in 24 (60%). Bilateral PV involvement was documented in 12 patients (30%). Overall death, PV reintervention, and restenosis occurred in 15%, 12.5%, and 25% of patients, respectively. Freedom from overall death, PV reintervention, and restenosis at 5 years was 85% +/- 6.3%, 88.9% +/- 5.2%, and 65.1% +/- 13.2%, respectively. Multivariate analysis revealed that bilateral PV involvement was an independent risk factor for death or PV reintervention (hazard ratio, 10.4; 95% confidence interval, 1.9-56; P = .006) and that involvement of the left inferior PV was an independent risk factor for postoperative restenosis of the left inferior PV (hazard ratio, 13.1; 95% confidence interval, 2.2-76.8; P = .004). CONCLUSIONS Surgical treatment for PPVS remains a challenging issue with imperfect prognosis. Therefore, it is right and appropriate to take close surveillance on mild or moderate stenosis on a single PV. Bilateral and left inferior PV involvement are independent risk factors for adverse outcomes. (c) 2022 by The Society of Thoracic Surgeons

OBJECTIVES:The optimal timing for atrioventricular valve (AVV) repair in patients with a Fontan circulation remains controversial. Few studies have reported the long-term outcomes of AVV repair concomitant with a Fontan operation.METHODS:From January 2006 to December 2018, a total of 89 patients who developed moderate or severe AVV regurgitation before a Fontan operation were divided into 2 groups: group 1, including 37 patients who did not undergo concomitant AVV repair; and group 2, including 52 patients who received AVV repair concomitant with a Fontan operation.RESULTS:The mean age at the time of the Fontan operation was 6.74 years for group 1 and 8.96 years for group 2, respectively. Early death occurred in 3 patients [2 patients (5.4%) in group 2, patient 1 (1.9%) in group 1]. Freedom from long-term death, cardiac function reduction and protein-losing enteropathy were similar among the 2 groups. Common AVV function was apparently poorer than mitral valve function after repair [hazard ratio (HR) 3.83, 95% confidence interval (CI) 1.31-11.17; P = 0.014]. The occurrence of AVV valve failure in group 1 was lower than that in group 2 (HR 0.44, 95% CI 0.22-0.91; P = 0.026). AVV function became worse during the follow-up period than that at discharge in both groups (P = 0.03 in group 1 and P = 0.001 in group 2).CONCLUSIONS:The long-term results of AVV repair concomitant with a Fontan operation are favourable.

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 提供一种基于儿童先天性二尖瓣反流(mitral regurgitation,MR)解剖异常特点的,相对简单化、可重复性高的标准化二尖瓣成形(mitral valvuloplasty,MVP)策略,并报道单中心的近期结果.方法 回顾性分析2016～2018年在本中心接受标准化MVP策略手术74例患者的临床资料,其中男30例、女44例,中位年龄18.5(6～146)个月,中位体重15.4(7～51)kg.结果 二尖瓣畸形包括:(1)瓣下水平:72例(97.3％)腱索异常连接,31例(41.9％)腱索缺失,14例(18.9％)乳头肌融合或发育不全;(2)瓣叶水平:10例(13.5％)前叶裂,61例(82.4％)瓣叶脱垂,其中56例(91.8％)为前叶脱垂;(3)瓣环水平:71例(95.9％)瓣环扩张.61例(82.4％)瓣叶脱垂均进行了瓣叶折叠.所有患者均顺利出院,4例(5.4％)患者为中量MR.中位随访时间22.0(9.1～41.8)个月,随访期间无患者死亡,4例(5.4％)患者发生中量或以上MR,均为术后即中量MR患者,其中1例患者由中量MR进展为大量MR,并行MVP再手术.患者整体心功能良好,平均左室射血分数66.0％±6.1％.平均左室舒张末期直径(31.8±6.0)mm,较术前明显减小(t=6.090,P＜0.000 1).结论 基于瓣叶折叠理念并辅以异常腱索处理和后瓣环环缩的MVP标准化术式近期预后良好,是一种安全可行的治疗先天性MR的MVP策略.

OBJECTIVESSurgical outcomes of supracardiac total anomalous pulmonary venous connection (TAPVC) repair by the posterior technique (PT) remain unsatisfactory. This study aimed to compare the outcomes of the modified L-shaped incision technique with the PT for supracardiac TAPVC repair.METHODSFrom January 2009 to December 2019, 121 consecutive patients with supracardiac TAPVC undergoing surgical repair in our institution were included (L-group, n = 53; PT group, n = 68). A propensity score-matched analysis was performed. Patients with single-ventricle physiology or atrial isomerism were excluded. All clinical data were retrospectively analysed.RESULTSIn the unmatched cohort, the median follow-up duration was 33 months (interquartile range 26-65 months). There were 5 operative mortalities (4.1%) and 12 late mortalities (9.9%). Postoperative pulmonary venous obstruction (PVO) was documented in 21 patients. After matching (52 pairs), the overall survival rate in the L-group was 88.2% at both 3 and 5 years. For the propensity score-matched patients with preoperative PVO (n = 20), statistically significant differences (P = 0.002) were found by Kaplan-Meier curves with freedom from death and postoperative PVO at 1 and 3 years of 100% and 85.7% [standard deviation (SD): 13.2%] in the L-group and 90% (SD: 9.5%) and 22.9% (SD: 14.1%) in the PT group, respectively. Multivariable analysis revealed that the use of the PT was an independent risk factor for death and postoperative PVO (hazard ratio 4.12, 95% confidence interval 1.12-15.16; P = 0.03).CONCLUSIONSThe modified L-shaped incision technique provided an acceptable outcome for supracardiac TAPVC repair. Compared with PT, the modified L-shaped incision technique was significantly associated with decreased death and postoperative PVO in patients with obstructed supracardiac TAPVC.

Background: Isolated congenital tricuspid regurgitation other than Ebstein's anomaly was rare especially for children. The objective of this study was to investigate the clinical characteristics and to assess the results of tricuspid valvuloplasty for children with isolated tricuspid regurgitation. Methods: From January 2010 to June 2019, 10 consecutive patients with isolated tricuspid regurgitation who were unresponsive to drug therapy underwent tricuspid valvuloplasty in our hospital. Patients' clinical data were analysed retrospectively. Results: Mean age at operation was 48.5 +/- 31.0 (range: 9-106) months and mean weight at operation was 16.1 +/- 6.9 (range: 8.6-33.0) kg. All patients presented severe isolated tricuspid regurgitation. According to pathological lesions, the main causes accounted for chordae tendinea rupture (3/10), leaflet cleft (2/10), mal-connected chordal tendinea to leaflets (2/10), elongated chordae (1/10) and chordae absent (1/10), and severe anterior leaflet dysplasia (1/10). Individualised tricuspid valvuloplasty was adapted to all of them successfully. Post-operative echocardiography showed no tricuspid regurgitation in two patients and mild regurgitation in eight patients. The cardiothoracic ratios on their chest roentgenograms decreased from 0.59 +/- 0.05 to 0.54 +/- 0.05. At the latest follow-up (50.4 +/- 47.2 months), echocardiography showed that mild to moderate tricuspid regurgitation in seven patients, moderate tricuspid regurgitation in three patients, and no patient with severe tricuspid regurgitation. All patients were in NYHA functional class I. Conclusions: For patients with isolated tricuspid regurgitation who were not well responsive to drug therapy, individualised tricuspid valve repair can achieve an excellent result.

Background The outcomes of anatomical repair for patients with congenitally corrected transposed great arteries remain unclear and the indications for different procedures are poorly understood. Methods From January 2005 to February 2016, consecutive corrected transposition patients who underwent anatomical repair at the current institution were enrolled in this retrospective study. Varied types of anatomical repair were individually customised. Results A total of 85 patients were included. Fifty-one (51) and 35 patients presented with left ventricular outflow tract obstruction and cardiac malposition, respectively. Thirty-nine (39) patients presented with moderate-to-severe tricuspid regurgitation. Thirty-four (34), 19, and 32 patients underwent Senning arterial switch operations, Senning-Rastelli, and hemi-Mustard-Rastelli-bidirectional Glenn, respectively. Early after repair, there were five in-hospital deaths and nine re-operations. During 4.6 years (range, 0.5-10.3) of follow-up, seven late deaths were documented. Estimated overall survival rate after anatomical repair was 89.3%, 85.0%, and 85.0% at 1 year, 3 years, and 5 years, respectively. Instead of Senning-Rastelli, most (75.0%) early left ventricular dysfunctions were noted in patients who underwent Senning arterial switch procedures. However, all the late left ventricular dysfunctions were found in patients who underwent previous left ventricular retraining. In patients with left ventricular outflow tract obstruction, the hemi-Mustard-Rastelli-bidirectional Glenn shunt provided a lower early mortality (0% vs 15.8%, p = 0.047). Conclusions Favourable outcomes can be achieved for anatomical repair of corrected transposition. Left ventricular dysfunction was a significant postoperative issue. Hemi-Mustard-bidirectional Glenn-Rastelli procedure may provide benefits for patients with associated left ventricular outflow tract obstruction and cardiac malposition. Each procedure has its own advantages in varied anatomy.

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.

There were controversies about the priority of modified single patch (MSP) technique compared with two-patch (TP) technique for patients with complete atrioventricular septal defect (CAVSD). From 2002 to 2013, patients who diagnosed as balanced CAVSD in our institution were retrospectively included. Patients with unbalanced ventricles or associated anomalies were excluded. The primary endpoint was all-cause mortality and the secondary endpoint consist of left atrioventricular dysfunction or left atrioventricular outflow tract obstruction (LVOTO). A total of 74 patients underwent MSP repair and 102 patients underwent TP repair. After 1:1 propensity matching, there were 46 Rastelli type A CAVSD patients in each group. Patients in MSP group had shorter cardiopulmonary bypass time [median (interquartile range) 73.5 (65.5–95.0) versus 105.0 (88.8–130.0) min, P < 0.001] and aortic cross-clamp time [105.5 (90.0–128.0) versus 143.0 (122.0–184.0) min, P < 0.001]. In total, the primary endpoint occurred in 12 patients, including 2/46 (4.3%) in MSP group and 10/46 (21.7%) in TP group. That 1-year, 3-year, 5-year survival rate can be calculated as 95.6%, 95.6%, 95.6% in MSP group and 92.6%, 80.4%, 78.2% in TP group, P = 0.015. No significant statistical difference found for the secondary endpoints that 7/46 (15.2%) patients occurred moderate or severe LAVVR in MSP group versus 11/46 (23.9%) patients in TP group, P = 0.293. No LVOTO occurred in both groups. Besides the simplicity of technique, MSP technique may be safer.

BACKGROUNDCommon atrioventricular valve (CAVV) repair in patients with a single ventricle remains a great challenge and a refractory issue for pediatric cardiac surgeons. Methods and Results: From January 2007 to April 2018, 37 consecutive patients with a single ventricle who underwent CAVV repair were included in the study group. Patients were divided into 2 groups based on the repair technique: patients in Group A were treated using the bivalvation technique, and patients in Group B underwent conventional repair techniques; baseline data were similar between groups. The inhospital and follow-up mortality were 5.4% (2/37) and 11.4% (4/35), respectively. After a follow-up of 65.5±29.3 months, the estimated 1-, 5-, and 10-year overall survival rates were 94.6%, 83.4%, and 77.0%, respectively. The rates of freedom from CAVV failure were 94.3%, 72.7%, and 62.9% after 1, 5, and 10 years, respectively. In the multivariate analysis, the independent factors for CAVV repair failure were repair technique (P=0.004) and heterotaxy syndrome (P=0.003). A total of 30 patients (81.1%) completed total cavopulmonary connection (TCPC); 3 patients required re-intervention; 24 of 31 patients (77.4%) were in New York Heart Association classes II and I at the latest follow-up.CONCLUSIONSOutcomes of CAVV repair in patients palliated by single-ventricular surgery are acceptable. The bivalvation technique is a simple and effective technique.