刘锐

中国医学科学院阜外医院 心脏外科

Fate of transposition of the great arteries with pulmonary stenosis after double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire.

OBJECTIVES:For transposition of the great arteries with unrestricted ventricular septal defect and pulmonary stenosis, double-root translocation is reported to reconstruct ideal double artery roots with growth potential. However, prospective long-term studies describing the long-term outcomes are still scarce. Therefore, the aim was to assess development of double artery roots, hemodynamics, and freedom from death and heart failure 17 years after double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire procedures.METHODS:In this prospective population-based study, 266 patients with transposition of the great arteries/ventricular septal defect/pulmonary stenosis (from July 2004 to August 2021) were consecutively included before surgery. All patients were divided into 3 groups based on the type of operation: double-root translocation (174), Rastelli (68), and Réparation à l'Etage Ventriculaire (24), who accepted postoperative evaluations annually. Generalized linear mixed model analysis was performed to determine growth potential of artery roots.RESULTS:Longitudinal repeated computed tomography measurements show the pulmonary root has significantly increased diameter (0.62 [0.03] mm/y, P < .001) over time and an adequate Z-score (-0.18) at the last follow-up only in the double-root translocation group. The pressure gradients of double outflow tracts in the double-root translocation group were the least among 3 groups. The probabilities of freedom from death/heart failure at the 15th year were 73.1%, 59.3%, and 60.9% in the double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire groups, respectively (double-root translocation vs Rastelli, P = .026; double-root translocation vs Réparation à l'Etage Ventriculaire, P = .009; Rastelli vs Réparation à l'Etage Ventriculaire, P = .449).CONCLUSIONS:By reconstructing ideal double artery roots, double-root translocation can provide postoperative long-term excellent hemodynamics and minimal death and heart failure for patients with transposition of the great arteries/ventricular septal defect/pulmonary stenosis.

6.0
1区
第一作者

The Journal of thoracic and cardiovascular surgery 2023

Can an operation provide superior outcomes for corrected transposition of the great arteries with left ventricular outflow tract obstruction? A multi-institutional study.

OBJECTIVES:Our goal was to evaluate the risk-adjusted effects of operative and non-operative repair on long-term outcomes in patients with congenitally corrected transposition of the great arteries and left ventricular outflow tract obstruction (CCTGA/LVOTO).METHODS:We retrospectively reviewed 391 patients with CCTGA/LVOTO from 2001 to 2020 (operative group, 282; non-operative group, 109) in 3 centres in China. The operative group included 73 patients who underwent anatomical repair and 209 patients who underwent non-anatomical repair. The median follow-up time was 8.5 years. The inverse probability of treatment weighted-adjusted Cox regression and a Kaplan-Meier analysis were used to evaluate long-term outcomes.RESULTS:Operative repair did not reduce the hazard ratio (HR) of death, tricuspid regurgitation or New York Heart Association functional class III/IV, but a significantly increased HR was observed for pulmonary valve regurgitation [HR, 2.84; 95% confidence interval (CI), 1.10-7.33; P = 0.031]. Compared with that in the non-operative group, anatomical repair resulted in significantly increased HRs for death (HR, 2.94; 95% CI, 1.10-7.87; P = 0.032) and pulmonary valve regurgitation (HR, 9.71; 95% CI, 3.66-25.77; P < 0.001). Subgroup analysis showed that in patients with CCTGA/LVOTO with moderate or worse tricuspid regurgitation (≥ moderate), anatomical repair significantly reduced the HR of death. An inverse probability of treatment weighting-adjusted Kaplan-Meier analysis showed that the survival rates at 5 and 10 days postoperatively were 88.2 ± 4.0% and 79.0 ± 7.9%, respectively, in the anatomical repair group; these rates were significantly lower than the rates in the non-operative group (95.4 ± 2.0% and 91.8 ± 2.8%; P = 0.032).CONCLUSIONS:For patients with CCTGA/LVOTO, operative repair does not provide superior long-term outcomes, and anatomical repair results in a higher incidence of death. However, in patients with CCTGA/LVOTO with tricuspid regurgitation ≥ moderate, anatomical repair can reduce the risk of death in the long term.

3.4
2区
第一作者

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2023

Sox9 Promotes Cardiomyocyte Apoptosis After Acute Myocardial Infarction by Promoting miR-223-3p and Inhibiting MEF2C.

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.

2.6
4区

Molecular biotechnology 2022

Handmade tri-leaflet ePTFE conduits versus homografts for right ventricular outflow tract reconstruction.

BACKGROUND:This study aimed to investigate the performance of handmade tri-leaflet expanded polytetrafluoroethylene (ePTFE) conduits in the absence of a suitable homograft.METHODS:Patients who underwent right ventricular outflow tract reconstruction with tri-leaflet ePTFE conduits or homografts between December 2016 and August 2020 were included. The primary endpoint was the incidence of moderate or severe conduit stenosis (≥ 36 mmHg) and/or moderate or severe insufficiency. The secondary endpoint was the incidence of severe conduit stenosis (≥ 64 mmHg) and/or severe insufficiency.RESULTS:There were 102 patients in the ePTFE group and 52 patients in the homograft group. The median age was younger [34.5 (interquartile range: 20.8-62.8) vs. 60.0 (interquartile range: 39.3-81.0) months, P = 0.001] and the median weight was lower [13.5 (10.0-19.0) vs. 17.8 (13.6-25.8) kg, P = 0.003] in the ePTFE group. The conduit size was smaller (17.9 ± 2.2 vs. 20.5 ± 3.0 mm, P < 0.001) and the conduit Z score was lower (1.48 ± 1.04 vs. 1.83 ± 1.05, P = 0.048) in the ePTFE group. There was no significant difference in the primary endpoints (log rank, P = 0.33) and secondary endpoints (log rank, P = 0.35). Multivariate analysis identified lower weight at surgery [P = 0.01; hazard ratio: 0.75; 95% confidence interval (CI) 0.59-0.94] and homograft conduit use (P = 0.04; hazard ratio: 8.43; 95% CI 1.14-62.29) to be risk factors for moderate or severe conduit insufficiency. No risk factors were found for moderate or severe conduit stenosis or conduit dysfunction on multivariate analysis.CONCLUSION:Handmade tri-leaflet ePTFE conduits showed acceptable early and midterm outcomes in the absence of a suitable homograft, but a longer follow-up is needed.

8.7
2区

World journal of pediatrics : WJP 2022

Comparing mini-sternotomy to full median sternotomy for aortic valve replacement with propensity-matching methods.

Objective:This study aims to compare clinical outcomes between mini-sternotomy and full median sternotomy for aortic valve replacement using propensity-matching methods.Methods:From August 2014 to July 2021, a total of 1,445 patients underwent isolated aortic valve surgery, 1,247 via full median sternotomy and 198 via mini-sternotomy. To reduce the impact of potential confounding factors, a propensity score based on 18 variables is used to obtain 198 well-matched case pairs, which include 231 aortic valve regurgitations and 165 aortic stenosis cases.Result:Occurrences of in-hospital mortality (P = 0.499), stroke (P > 0.999), renal failure (P = 0.760), and paravalvular leakage (P = 0.224) are similar between the two groups. No significant difference in operation, cardiopulmonary bypass, and aortic cross-clamp times are found between the two groups. However, compared with the full sternotomy group, the mini-sternotomy group has less postoperative 24-hour drainage (131.7 ± 82.8 ml, P < 0.001) and total drainage (459.3 ± 306.3 ml, P < 0.001). The median mechanical ventilation times are 9.4 [interquartile range (IQR) 5.4-15.6] and 9.8 (IQR 6.1-14.4) in mini-sternotomy and full sternotomy groups (P = 0.284), respectively. There are no significant differences in intensive care unit stay and postoperative stay between the two groups. For either aortic valve regurgitations or aortic stenosis patients, significantly less postoperative 24-h and total drainage are still found in the mini-sternotomy group compared with the full sternotomy group.Conclusions:Mini-sternotomy for aortic valve replacement is a safe procedure, with not only cosmetic advantages but less postoperative drainage compared with full sternotomy. Mini-sternotomy should be considered for most aortic valve operations.

1.8
4区
第一作者

Frontiers in surgery 2022

Congenitally corrected transposition with left ventricular outflow obstruction and cardiac malposition: One-and-a-half ventricular repair vs. Fontan pathway?

Objectives:This study was to assess the mid-term results of the one-and-a-half ventricular repair (hemi-Mustard and bidirectional Glenn procedures combined with the Rastelli procedure) and Fontan pathway for correcting congenitally corrected transposition of great artery (ccTGA) patients with left ventricular outflow tract obstruction (LVOTO) and cardiac malposition.Methods:In this retrospective study, 74 consecutive ccTGA with LVOTO and cardiac malposition underwent the one-and-a-half ventricular repair (group A; 33 cases) and Fontan operation (group B; 41 cases) between October 2011 and March 2018. The Median follow-up time was 49 (20-84) and 42 (7-85) months in groups A and B, respectively. To estimate excise tolerance the 6-min walk test (MWT) was performed.Results:No in-hospital death. Compared with group A, group B have significantly less CPB, mechanical ventilation time, and intensive care unit stay, but prolonged pleural effusions developed more frequently in Group B. The survival probability was 90.2% (95% CI, 80.2-100%) and 97.2% (95% CI, 92-100%) at 7 years (p = 0.300) in group A and B. The probability of freedom from re-intervention were 80.6% (95% CI, 66.5-97.6%) and 97.2% (95% CI, 92-100%) at 7 years (p = 0.110). Longitudinal repeated measured echo data at every follow-up time shows that group A has more systemic ventricular EF% (p < 0.001) and less moderate systemic ventricular valve regurgitation (p < 0.001) compared with group B. Estimated by 6 MWT, group A has better outcomes for 6-min walk distance.Conclusions:For correction of ccTGA with LVOTO and cardiac malposition, the one-and-a-half ventricular repair had superior midterm heart function and excise tolerance.

3.6
3区
第一作者

Frontiers in cardiovascular medicine 2022

Cardiovascular Phenotypes Profiling for L-Transposition of the Great Arteries and Prognosis Analysis.

OBJECTIVES:Congenitally corrected transposition of the great arteries (ccTGA) is a rare and complex congenital heart disease with the characteristics of double discordance. Enormous co-existed anomalies are the culprit of prognosis evaluation and clinical decision. We aim at delineating a novel ccTGA clustering modality under human phenotype ontology (HPO) instruction and elucidating the relationship between phenotypes and prognosis in patients with ccTGA.METHODS:A retrospective review of 270 patients diagnosed with ccTGA in Fuwai hospital from 2009 to 2020 and cross-sectional follow-up were performed. HPO-instructed clustering method was administered in ccTGA risk stratification. Kaplan-Meier survival, Landmark analysis, and cox regression analysis were used to investigate the difference of outcomes among clusters.RESULTS:The median follow-up time was 4.29 (2.07-7.37) years. A total of three distinct phenotypic clusters were obtained after HPO-instructed clustering with 21 in cluster 1, 136 in cluster 2, and 113 in cluster 3. Landmark analysis revealed significantly worse mid-term outcomes in all-cause mortality (p = 0.021) and composite endpoints (p = 0.004) of cluster 3 in comparison with cluster 1 and cluster 2. Multivariate analysis indicated that pulmonary arterial hypertension (PAH), atrioventricular septal defect (AVSD), and arrhythmia were risk factors for composite endpoints. Moreover, the surgical treatment was significantly different among the three groups (p < 0.001) and surgical strategies had different effects on the prognosis of the different phenotypic clusters.CONCLUSIONS:Human phenotype ontology-instructed clustering can be a potentially powerful tool for phenotypic risk stratification in patients with complex congenital heart diseases, which may improve prognosis prediction and clinical decision.

3.6
3区

Frontiers in cardiovascular medicine 2021

The Fate of Congenitally Corrected Transposition of the Great Arteries Unoperated Before Adulthood.

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.

4.6
2区
第一作者

The Annals of thoracic surgery 2021

Mid-term outcome of surgical treatment in patients with aorto-left ventricular tunnel.

OBJECTIVES:The purpose of this study was to review the outcomes of surgical treatment in patients with aorto-left ventricular tunnel and to investigate what kind of patient cohort is more likely to have adverse events.METHODS:Twenty-one patients with a median age of 6.58 [interquartile range (IQR) 4.17-24.50] years who received surgical treatment of aorto-left ventricular tunnel from March 2002 to December 2019 were reviewed. The median follow-up time was 64.50 (IQR 25.15-120.50) months. Clinical characteristics, surgical methods and follow-up outcomes were summarized in separate groups of patients with or without preoperative aortic valve (AoV) issues. Composite adverse events were defined as death or requirement of reoperation. Time-related analysis of freedom from death and requirement of reoperation was performed with the Kaplan-Meier method.RESULTS:The average tunnel size was 8.68 (standard deviation: 3.62) mm. The most common and the most important associated lesions were AoV lesions. Tunnels in 20 patients were closed with direct sutures or a patch. For 1 patient with an irreparable AoV, the tunnel was cut open simultaneously with aortic valve replacement and aortic root plasty. In the group of patients with preoperative AoV issues, 4 patients received aortic valve replacement with mechanical prosthetic valves and 6 patients received aortic valvuloplasty repair. The follow-up outcomes differed significantly between groups (the incidence of death was 15.38% and the incidence of requiring a reoperation was 46.15% in patients with preoperative AoV issues). In the group without preoperative AoV issues, there were no deaths and no reoperations (Fisher's exact test; P = 0.018). The probability of freedom from death and of the requirement for reoperation between the 2 groups was not significantly different (log-rank, P = 0.09). Overall, the estimated probability of freedom from death and requirement of reoperation was 77.30% (standard error: 10.20%) [95% confidence interval (CI): 49.53-91.00] at 5 years, 67.64% (standard error: 12.70%) (95% CI: 36.71-85.84) at 10 years.CONCLUSIONS:Patients with aorto-left ventricular tunnel with preoperative AoV issues are more prone to die or to require a reoperation. In contrast, patients without preoperative AoV issues can be free from death or reoperation for a longer period of time. Patients with preoperative AoV issues need much stricter postoperative long-term echocardiographic follow-up.

3.4
2区

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2021

Impact of electrophysiological features acquired after anatomical repair of congenital corrected transposition of the great arteries on late mortality and ventricular dysfunction.

OBJECTIVES:In patients with anatomically repaired congenitally corrected transposition of the great arteries, the impact of electrophysiological features on postoperative ventricular dysfunction remains less well known. Our goal was to investigate the role of fragmented QRS and QRS duration in mortality and systemic ventricular dysfunction after anatomical repair of corrected transposed great arteries.METHODS:Consecutive patients who underwent anatomical repair in our institution from January 2005 to December 2017 were enrolled in this retrospective analysis. Fragmented QRS was defined as ≥1 discontinuous deflections in narrow QRS complexes, and ≥2 in wide QRS complexes, in 2 contiguous electrocardiogram leads. The primary end point was a composite of all-cause mortality and systemic ventricular dysfunction.RESULTS:A total of 74 patients were included. Among them, 30, 15 and 29 underwent the Senning arterial switch, the Senning Rastelli and the hemi-Mustard/bidirectional Glenn/Rastelli procedures, respectively. The primary end point occurred in 9 (12.2%) patients and included 7 late deaths and 2 cases of late-onset systemic ventricular dysfunction. Fragmented QRS and QRS prolongation were noted in 19 (25.7%) and 21 (28.4%) patients, respectively. In patients with the primary end point, QRS fragmentation (6/9 vs 10/65; P < 0.001) and QRS prolongation (6/9 vs 15/65; P = 0.013) were noted more frequently than in patients without the primary end point. No statistical differences in these electrocardiogram findings were found among patients treated with 3 surgical strategies.CONCLUSIONS:Appearance of QRS fragmentation or QRS prolongation is associated with death or ventricular dysfunction in anatomically repaired corrected transposition of the great arteries. Although there is a trend that QRS fragmentation and QRS prolongation appear more frequently in patients who had the Senning-arterial switch operation, there is no statistically significant difference associated with these electrocardiogram features among varied procedures.

3.4
2区

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2021