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Objective:To investigate the short-term and medium-term changes of the left ventricular ejection fraction (LVEF) and the predictive value of relevant electrocardiogram (ECG) indexes in children with dilated cardiomyopathy (DCM) complicated with complete left bundle branch block (CLBBB).Methods:Children clinically diagnosed with DCM in the Department of Heart Center, Women and Children′s Hospital, Qingdao University and Beijing Anzhen Hospital, Capital Medical University between November 2011 and August 2020 were retrospectively recruited.According to the combination of CLBBB, they were divided into CLBBB group and non-CLBBB group.Echocardiogram and ECG were regularly performed.Short-term and medium-term changes of LVEF based on the 1-5-year follow-up data were compared between groups.COX proportional hazards model and Kaplan-Meier multiplicative limit method were used to analyze the predictive value of ECG indexes of LVEF changes in children with DCM combined with CLBBB.Results:Ninety-four children with DCM were enrolled, including 35 cases in CLBBB group and 59 cases in non-CLBBB group.There was no difference in baseline LVEF between groups.However, significant differences were found in QRS duration, corre-cted QT interval(QTc), R peak time in lead V 5 (T V5R) and QRS notching or slurring between groups ( P<0.05). LVEF of all children showed an upward trend within one year after onset, while the Z value of eft ventricular end diastolic diameter(LVEDd) showed a downward trend, and the two indexes tended to be stable within 1 - 5 years.The Z value of LVEDd in CLBBB group was significantly higher than that of non-CLBBB group, while LVEF was significantly lower (all P<0.05). The mean LVEF of CLBBB group slightly fluctuated around 50%, that of LVEF in non-CLBBB group was 60%.The multivariate COX regression analysis showed that QRS duration ( HR=0.979; 95% CI: 0.960-0.999, P<0.05) and QTc ( HR=0.988; 95% CI: 0.979-0.998, P<0.05) were independent predictors of LVEF recovery in children with DCM.Kaplan-Meier method showed a significant difference of LVEF normalization between DCM children with different QRS durations ( P<0.05), which was also detected in those with QTc interval ( P<0.05). Conclusions:LVEF of children with DCM combined with CLBBB increases in the short term after standard treatment, and then being stable.CLBBB can affect the recovery of left ventricular systolic function in children with DCM.Moreover, QRS duration and QTc interval are independent predictors of LVEF recovery in DCM children.
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Objective:To investigate the correlation between specific expression of serum micro ribonucleic acid (miRNA) and dilated cardiomyopathy (DCM) in children.Methods:Sixteen children diagnosed with DCM in Pediatric Heart Center of Beijing Anzhen Hospital from November 2013 to March 2016 were enrolled in the DCM group.Meanwhile, 12 age- and gender-matched healthy children who underwent medical examinations at the same time in the same hospital were selected as the healthy control group.Their serum was collected and miRNA sequencing was performed.The sample size was expanded at the later stage (the DCM group included 30 cases, and the healthy control group included 16 cases). The real-time fluorescence quantitative polymerase chain reaction (qRT-PCR) verification experiment was conducted on 11 miRNAs with statistically significant sequencing results.Results:Serum miRNA sequencing showed that 172 miRNAs were up-regulated but no miRNAs were down-regulated in the DCM group, compared with the healthy control group (fold change>2, P<0.001). Top 11 significantly up-regulated miRNAs were verified by qRT-PCR, and it was found that 8 of the 11 miRNAs (let-7f, let-7g, miR142-5p, miR143-3p, miR26a, miR27a-3p, miR27b-3p, and miR126-3p) in the DCM group were significantly up-regulated (all P<0.05). In the receiver operating characteristic (ROC) curves of DCM patients, the area under the curves of serum miR142-5p, miR143-3p, miR27b-3p, and miR126-3p were 0.983, 0.992, 0.915 and 0.950, respectively, which were statistically significantly different from those of the healthy control group (all P<0.05). Conclusions:Four serum miRNAs (miR-142-5p, miR-126-3p, miR-143-3p and miR-27b-3p) can distinguish children with DCM from healthy children.Circulating miRNAs are effective in screening DCM children.
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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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Objective@#To analyze the gene mutations and clinical features of patients with Noonan syndrome and hypertrophic cardiomyopathy.@*Method@#Determined the mutation domain in five cases diagnosed with Noonan syndrome and hypertrophic cardiomyopathy and identified the relationship between the mutant domain and hypertrophic cardiomyopathy by searching relevant articles in pubmed database.@*Result@#Three mutant genes (PTPN11 gene in chromosome 12, RIT1 gene in chromosome 1 and RAF1 gene in chromosome 3) in five cases all had been reported to be related to hypertrophic cardiomyopathy. The reported hypertrophic cardiomyopathy relevant genes MYPN, MYH6 and MYBP3 had also been found in case 1 and 2. Patients with same gene mutation had different clinical manifestations. Both case 4 and 5 had RAF1 mutation (c.770C>T). However, case 4 had special face, low IQ, mild pulmonary artery stenosis, and only mild ventricular hypertrophy.@*Conclusion@#Noonan syndrome is a genetic heterogeneity disease. Our study identified specific gene mutations that could result in Noonan syndrome with hypertrophic cardiomyopathy through molecular biology methods. The results emphasize the importance of gene detection in the management of Noonan syndrome.
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Objective To investigate the spectrum,diagnosis,time of therapy and management of the congeni-tal heart disease(CHD)in patients with Down′s syndrome(DS).Methods A retrospective report was undertaken of 96 cases in children with DS accompanied by CHD in Department of Pediatric Cardiology,Beijing Anzhen Hospital Af-filiated to Capital Medical University.Data were collected and analyzed about their clinical characteristics,and types of cardiovascular abnormalities,and the important laboratory examinations such as echocardiography and catheterization as well as the procedures of diagnosis and treatments were summarized.Then the interventions,complications and prognosis of different patients were estimated.Results (1)Single congenital heart disease was found in 33 cases (34.38%),a-mong which ventricular septal defect was the most common (14 cases,14.58%),followed by atrioventricular septal de-fect and atrial septal defect (equally,7 cases,7.29%).Multi -cardiovascular abnormalities were discovered in 63 ca-ses,and patent ductus arteriosus turned out to be the most common (42 cases,66.67%).(2)Cat-heterization was car-ried out in 18 cases of serious pulmonary arterial hypertension,and 8 cases were proved resistant pulmonary arterial hy-pertension without operation opportunity.The other 8 cases were estimated as high pulmonary arterial hypertension and medical therapy was suggested before reassessment to reduce surgical risks.(3)Operations were undertaken in 61 ca-ses,among which percutaneous interventional occlusion was performed in 7 cases and surgical interventions were per-formed in 54 patients,in which perioperation complications and death were found in 5 cases and 4 cases,respectively. Conclusions Operation interventions are practicable and most cases recovered well with systematic examinations and assessment in patients with DS and cardiovascular malformations.Early diagnosis and timely interventions are highly suggested.Also close attentions should be paid to follow -up and re -estimation after medical therapy.
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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>
Subject(s)
Adult , Aged , Child , Child, Preschool , Female , Humans , Infant , Male , Middle Aged , Young Adult , Bland White Garland Syndrome , Diagnosis , Pathology , General Surgery , Cardiac Surgical Procedures , Coronary Vessel Anomalies , Pathology , General Surgery , Coronary Vessels , Pathology , General Surgery , Echocardiography , Electrocardiography , Pulmonary Artery , Pathology , General SurgeryABSTRACT
<p><b>OBJECTIVE</b>To analyze the adverse effects of type B ventricular pre-excitation on ventricular wall motion and left ventricular function and its clinical characteristic.</p><p><b>METHOD</b>The clinical, electrophysiological and echocardiographic characteristics of the 9 cases with type B ventricular pre-excitation before and after ablation seen between March 2011 and March 2013 were analyzed. The patients aged from 3 to 16 years. Five of them were female.</p><p><b>RESULT</b>Dyschronous left ventricular contraction was demonstrated by M-Mode echocardiography in all of the cases. The basal segments of the interventricular septum turned thin and moved in a manner similar to that of an aneurysm, with typical bulging during end-systole, which was observed in six cases. All patients received successful RFCAs. The locations of the accessory pathways (APs) were the right-sided anteroseptum (n = 2) and the free wall (n = 7). Their physical activities and growth improved greatly in the four cases with coexisting dilated cardiomyopathy (DCM). The echocardiographic data demonstrated that their LV contraction recovered to synchrony shortly after the ablation, LVEF recovered to normal and LVED decreased to almost normal gradually during the follow-up.</p><p><b>CONCLUSION</b>Overt right-sided APs may have adverse effects on ventricular wall motion and left ventricular function. They can even result in DCM. Dyssynchronous ventricular contraction induced by right-sided overt accessory pathway may be the vital mechanism. Such kinds of cases are indication for ablation with good prognosis.</p>
Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Male , Cardiomyopathy, Dilated , Diagnostic Imaging , Catheter Ablation , Echocardiography , Heart Ventricles , Myocardial Contraction , Ventricular Dysfunction, Left , Diagnostic Imaging , Wolff-Parkinson-White SyndromeABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinical manifestations and treatment of congenital atresia of the left main coronary artery (CLMCA-A).</p><p><b>METHOD</b>Four patients were diagnosed to have CLMCA-A from June 2010 to June 2012 in Beijing Anzhen Hospital. Clinical manifestations, ultrasound, ECG and angiographic characteristics were analyzed and summarized.</p><p><b>RESULT</b>Of the 4 cases, age of onset was 3 months to 2 yrs. Three cases were diagnosed by angiography, and 1 case by CTA . All 4 cases had chronic heart failure symptoms and signs, such as sweating, shortness of breath, easily choked by milk, predispose to pneumonia, activity intolerance. ECG showed abnormal Q wave and other ischemic signs such as ST-T segment depression. Ultrasonography showed left ventricular enlargement, left ventricular systolic function was normal or slightly reduced, and there was moderate to large amount of mitral valve regurgitation. Left ventricular trabeculations increased. Coronary collateral circulation increased. Left coronary artery appeared to be slender and disconnected with left coronary artery sinus. Aortic root angiography was the golden diagnostic standard. Angiography was performed in 3 patients and showed that left main coronary artery had a blind end, diameter 1.1-2.0 mm. The right coronary artery was found rising from the right coronary sinus and visible on coronary collateral circulation. Contrast agent developing sequence: right coronary artery-collateral vessels-left coronary artery distal branches-left main coronary artery. CTA exam was performed in 2 cases and in 1 case the diagnoses was confirmed. All the 4 patients are currently in the close follow-up, digoxin and diuretics were taken everyday and clinical symptoms were improved.</p><p><b>CONCLUSION</b>CLMCA-A is not rare, its clinical manifestations should be differentiated from those of cardiomyopathy, endocardial fibroelastosis, congenital valvular disease and abnormal left coronary artery originating from pulmonary artery etc. For pediatric patients with cardiac enlargement, abnormal heart function, mitral valve regurgitation etc, attention must be paid to consider the developmental abnormality of coronary artery, particularly the CLMCA-A diagnosis.</p>
Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Male , Coronary Angiography , Methods , Coronary Vessel Anomalies , Diagnosis , Pathology , Coronary Vessels , Diagnostic Imaging , Pathology , Diagnosis, Differential , Echocardiography , Electrocardiography , Endocardial Fibroelastosis , Diagnosis , Pathology , Heart Defects, Congenital , Diagnosis , Pathology , Mitral Valve Insufficiency , Diagnosis , Pathology , Pulmonary Artery , Congenital Abnormalities , Diagnostic Imaging , Tomography, X-Ray Computed , MethodsABSTRACT
Objective To discuss the characteristics,diagnosis,mechanism and the prognosis of dilated cardiomyopathy (DCM) induced by accessory pathway (AP).Methods The clinical,electrophysiological and echocardiographic characteristics in four cases,who were diagnosed as AP-induced DCM in the Department of Pediatric Cardiology of Beijing Anzhen Hospital,Capital Medical University from Mar.2011 to Aug.2012,were analyzed before and after ablation.Results The electrocardiograms of the 4 patients all indicated type B ventricular preexcitation.The locations of the APs were the right-sided anteroseptum and the free wall.Dyschronous contractions between posterior wall of left ventricle and interventricular septum were demonstrated by M-Mode echo.The basal segments of the interventricular septum turned thin and moved similar to an aneurysm,with typical bulging during end-systole,which was observed in all cases by two-dimension echo.Dyschronous left ventricular contraction was shown by speckle tracing technique.All patients received successful radio frequency current ablations.Their physical activities and growth improved greatly in the 4 cases.The echocardiographic data demonstrated that their left ventricular contraction recovered to synchrony shortly after the ablation,left ventricular ejection fraction recovered to normal and left ventricular end diastolic diameter decreased to almost normal gradually during the follow-up.Conclusions Overt right-sided APs localized in anteroseptum or free wall may have adverse effects on ventricular wall motion and left ventricular function.They can even result in DCM.Dyssynchronous ventricular contraction induced by right-sided overt accessory pathway may be the vital mechanism.AP-induced DCM is an indication for ablation with good prognosis.
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Aurora-B as an important kinase to adjust the cell normal mitosis is a potent target for cancer treatment. Aurora-B is overexpressed in a broad range of tumor and tumor cells are more sensitive while Aurora-B is inhibited. Due to the key role of the Aurora-B in cell mitosis, the development of its inhibitors is becoming more and more important. Several small molecules inhibit with a similar efficacy both Aurora-A and Aurora-B, however, in most cases the effects resemble Aurora-B disruption by genetic methods, indicating that Aurora-B represents an effective therapeutic target. There were several Aurora-B kinase inhibitors which had entered the clinics and displayed good antitumor activity. In this review, we will outline the functions of Aurora kinase B in normal cell division and in malignancy. We will focus on recent preclinical and clinical studies that have explored the mechanism of action and clinical effect of Aurora-B inhibitors in cancer treatment.