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OBJECTIVES@#To develop a prediction model for postoperative prognosis in patients with cholangiocarcinoma (CCA) based on the expression of silence information regulator 2 (SIRT2).@*METHODS@#The differential expression of SIRT2 between CCA and normal tissues was analyzed using TCGA and GEO databases. Gene set enrichment analysis (GSEA) was used to explore potential mechanisms of SIRT2 in CCA. The expression of SIRT2 protein in CCA tissues and normal tissues (including 44 pairs of specimens) was also detected by immunohistochemistry (IHC) staining in 89 resectable CCA patients who underwent surgical treatment in The First Affiliated Hospital of Bengbu Medical College between January 2016 and December 2021. The relationship between SIRT2 expression and clinicopathological characteristics and prognosis of CCA patients was analyzed. A survival prediction model for patients with resectable CCA was constructed with COX regression results, the calibration curve and the time-dependent receiver operating characteristic curve (ROC) were used to evaluate the performance of the constructed model, and the predictive power between this model and the AJCC/TNM staging system (8th Edition) was compared.@*RESULTS@#SIRT2 mRNA was overexpressed in CCA tissues as shown in TCGA and GEO databases. IHC staining showed that SIRT2 protein expression in CCA tissues was significantly higher than that in adjacent non-tumor tissues. GSEA results showed that elevated SIRT2 expression may be involved in multiple metabolism-related signaling pathway, such as fatty acid metabolism, oxidative phosphorylation, amino acid metabolism, etc. SIRT2 expression level was related to serum triglycerides level, tumor size and lymph node metastasis (all P<0.05). The survival analysis results showed that the patients with higher SIRT2 expression had a significant lower overall survival (OS) than patients with lower SIRT2 expression (P<0.05). Univariate COX regression analysis suggested that pathological differentiation, clinical stage, postoperative treatment and SIRT2 expression level were associated with the prognosis of CCA patients (all P<0.05). Multivariate regression analysis confirmed that clinical stage and SIRT2 expression level were independent predictors of OS in postoperative CCA patients (both P<0.05). A nomogram based on SIRT2 for prediction of survival in postoperative CCA patients was constructed. The C-index of the model was 0.675, and the area under the time-dependent ROC curve (AUC) for predicting survival in the first, second, and third years was 0.879, 0.778, and 0.953, respectively, which were superior to those of AJCC/TNM staging system (8th Edition).@*CONCLUSIONS@#SIRT2 is highly expressed in CCA tissues, which is associated with poor prognosis in patients with resectable CCA. The nomogram developed based on SIRT2 may have better predictive power than the AJCC/TNM staging system (8th Edition) in prediction of survival of postoperative CCA patients.
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Objective:Pulmonary annulus index is used to predict the application value of transannular patch in children with tetralogy of Fallot.Methods:A retrospective analysis of 130 patients with pediatric heart disease diagnosis of TOF and undergoing TOF repair in Beijing Anzhen Hospital affiliated to Capital Medical University from December 2018 to December 2019, 112 cases were included in this study, 18 cases were excluded. They were divided into TAP group and no TAP group, related values of pulmonary annulus and aortic annulus were measured. The pulmonary annulus index, the pulmonary annulus Z-score and main pulmonary artery Z-score were calculated to do statistically analyze.Results:A total of 112 patients, average age(22.87±12.21) months; 66 males and 46 females; weight( 9.94±4.08)kg; 3 cases died, 1 case died of sepsis caused by pulmonary infection, 1 case died of low cardiac output syndrome, and 1 case died of multiple organ failure. 62 cases(55.8%) did not transannular patch, 50 cases(44.2%) transannular patch. The pulmonary annulus Z-score, main pulmonary artery Z-score and PAI in TAP group were smaller than those in no TAP group( P<0.05). Receiver operator curves( ROC) analysis showed that the cut-off value of PAI at 0.53 AUC was 0.85, the sensitivity was 75%, the specificity was 80%; the cutoff value of pulmonary annulus at -1.98 AUC was 0.88, the sensitivity was 80%, the specificity was 71%; the cutoff value of main pulmonary artery at -2.12 AUC was 0.87, the sensitivity was 77%, and the specificity was 87%. When the critical value of PAI(>0.53) and main pulmonary artery Z-score(>2.12), 88.2% of the children could avoid TAP, and 94% of the children could avoid TAP when the critical value of pulmonary annulus Z-score(> -1.91) and main pulmonary artery Z-score(>-2.12) were combined. Conclusion:The predictive effect of pulmonary annulus index as a simple and effective predictor of TAP in TOF radical operation is the same as that of pulmonary annulus Z-score. Combining either with main pulmonary artery Z-score was the most accurate method of prediction.
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Objective@#To analyze the ratio of pulmonary valve ring to aortic valve ring (GA ratio), and to explore the application value of GA ratio in predicting the need of transannular patch in the radical operation of children with tetralogy of Fallot (TOF).@*Methods@#A retrospective analysis was performed in 355 children (182 males and 173 females) with TOF and underwent radical operation in the Pediatric Cardiac Center of Beijing Anzhen Hospital Affiliated to Capital Medical University from January 2016 to December 2017.They were divided into transannular patch group and non-transannular patch group.The values of pulmonary valve ring and aortic valve ring in two groups were collected, and the Z-score of pulmonary artery and GA ratio were calculated respectively for statistical analysis.@*Results@#Among the 355 patients, 156 children (43.9%) required a transannular patch, and 199 patients (56.1%) did not receive transannular patch.The GA ratio and the Z-score of of pulmonary artery in the transannular patch group were lower than those in the non-transannular patch group [0.45±0.12 vs. 0.54±0.15, (-1.75±0.98) scores vs.(-0.86±1.39) scores], and the differences were statistically significant (t=5.29, 6.32, all P<0.01). Receiver operating characteristic curve analysis showed that in the case of the Z-score of pulmonary artery of -2 for the children received transannular patch, the area under the curve (AUC) was 0.702 (95%CI: 0.64-0.76), sensitivity was 69%, and specificity was 83%; in the case of the GA ratio of 0.58 for the children received transannular patch, the AUC was 0.712 (95%CI: 0.66-0.77), sensitivity was 70%, and specificity was 87%.@*Conclusions@#The GA ratio is a simple and effective predictor of transannular patch in the radical operation of TOF and can be applied in clinical practice.
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Objective To analyze the ratio of pulmonary valve ring to aortic valve ring (GA ratio),and to explore the application value of GA ratio in predicting the need of transannular patch in the radical operation of children with tetralogy of Fallot (TOF).Methods A retrospective analysis was performed in 355 children (182 males and 173 females) with TOF and underwent radical operation in the Pediatric Cardiac Center of Beijing Anzhen Hospital Affiliated to Capital Medical University from January 2016 to December 2017.They were divided into transannular patch group and non-transannular patch group.The values of pulmonary valve ring and aortic valve ring in two groups were collected,and the Z-score of pulmonary artery and GA ratio were calculated respectively for statistical analysis.Results Among the 355 patients,156 children (43.9％) required a transannular patch,and 199 patients (56.1％)did not receive transannular patch.The GA ratio and the Z-score of of pulmonary artery in the transannular patch group were lower than those in the non-transannular patch group [0.45 ±0.12 vs.0.54 ±0.15,(-1.75 ±0.98) scores vs.(-0.86 ± 1.39) scores],and the differences were statistically significant (t =5.29,6.32,all P ＜ 0.01).Receiver operating characteristic curve analysis showed that in the case of the Z-score of pulmonary artery of-2 for the children received transannular patch,the area under the curve (AUC) was 0.702 (95％ CI:0.64-0.76),sensitivity was 69％,and specificity was 83％;in the case of the GA ratio of 0.58 for the children received transannular patch,the AUC was 0.712 (95％ CI:0.66-0.77),sensitivity was 70％,and specificity was 87％.Conclusions The GA ratio is a simple and effective predictor of transannular patch in the radical operation of TOF and can be applied in clinical practice.
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<p><b>BACKGROUND</b>The anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) and congenital left main coronary artery atresia (CLMCA-A) are two kinds of very rare coronary heart diseases which affect heart function profoundly. This study aimed to retrospectively illustrate the clinical features and therapy experience of ALCAPA and CLMCA-A patients.</p><p><b>METHODS</b>From April 1984 to July 2012, in Beijing Anzhen Hospital, 23 patients were diagnosed with ALCAPA and 4 patients with CLMCA-A. We summarized the clinical data of the 27 cases and retrospectively analyzed the clinical manifestation, diagnosis, and treatments of these two kinds of congenital coronary abnormalities.</p><p><b>RESULTS</b>The 23 patients (13 males and 10 females, aged ranging from 2.5 months to 65 years) identified with ALCAPA were classified into infantile type (age of onset younger than 12 months, 16 cases) and adult type (age of onset older than 12 months, 7 cases). Four patients were diagnosed with CLMCA-A (three males and one female, aged ranging from 3 months to 2 years). The main clinical manifestations of infantile-type ALCAPA and CLMCA-A include repeated respiratory tract infection, heart failure, dyspnea, feeding intolerance, diaphoresis, and failure to thrive. And these two congenital coronary abnormalities might be misdiagnosed as endocardial fibroelastosis, dilated cardiomyopathy, and acute myocardial infarction. As for the adult-type ALCAPA, cardiac murmurs and discomfort of the precordial area are the most common presentations and might be misdiagnosed as coronary heart disease, myocarditis, or patent ductus arteriosus. In ECG examination: Infantile-type ALCAPA and CLMCA-A showed abnormal Q waves with T wave inversion in leads I, avL, and V4-V6, especially in lead avL. However, ECG of adult-type ALCAPA lacked distinct features. In chest radiography: pulmonary congestion and cardiomegaly were the most common findings in infantile-type ALCAPA and CLMCA-A, while pulmonary artery segment dilation was more common in adult type. In echocardiography, the common features of infantile-type ALCAPA and CLMCA-A included left ventricular enlargement, left ventricular systolic function normal or mildly reduced in CLMCA-A or significantly reduced in ALCAPA, and moderate to large mitral valve. It was performed in 9 of 23 cases of ALCAPA and showed the origin of the dilated right coronary artery (RCA) from the right sinus of the aortic root and absence of LCA origin in angiography. After opacification of RCA, reverse flow in the LCA and pulmonary artery was visualized through coronary artery collateral circulation. Angio was performed in three of the four cases of CLMCA-A and showed left main coronary artery was a blind end, with diameter of only 1.1-2.0 mm. Treatment and prognosis: 21 patients with ALCAPA had cardiac surgery and 6 of them died postoperatively. Fifteen postoperative patients survived without overt symptoms within the follow-up period of 6-166 months (median 17 months). As for treatment of CLMCA-A, four patients took digoxin and diuretics without undergoing cardiac surgery. Their clinical symptoms improved during the close follow-ups.</p><p><b>CONCLUSIONS</b>ALCAPA and CLMCA-A are two rare coronary artery abnormalities that affect cardiac function in infants and children. In younger patients with cardiomegaly and heart dysfunction these two congenital coronary diseases should be noticed.</p>