Short-term and medium-term changes of the left ventricular ejection fraction in children with dilated cardiomyopathy complicated with complete left bundle branch block and the predictive value of relevant electrocardiogram indexes / 中华实用儿科临床杂志

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.

Effect of transcatheter occlusion of azygos/hemiazygos vein in patients with venous stealing after the bidirectional Glenn procedure-analysis of 24 cases / 中华胸心血管外科杂志

Objective:To observe and follow up the effect of transcatheter occlusion of the azygos/hemiazygos veins in patients with complex congenital heart diseases and venous stealing after bidirectional Glenn procedure, who had not meet the criteria of total cavopulmonary connection(TCPC) or corrective operation.Methods:This article analyzed retrospectively and followed up the effect of transcatheter occlusion of the azygos/hemiazygos vein in patients after bidirectional Glenn procedure hospitalized during February 2012 to September 2017.Results:Transcatheter occlusions of azygos veins were performed in 21 patients and left superior vena cava(LSVC) was occluded in one of patients meanwhile. Azygos veins and hemiazygos veins were both occluded in two patients. One patient had the hemiazygos vein occluded. Aortic-to-pulmonary collaterals were occluded in 6 patients in the meantime. Atrial septal defect occluders were applied in the transcatheter occlusions of azygos veins, hemiazygos veins and LSVC, except one patient with an azygos vein which was 5.5 mm in diameter. Saturation was increased from 0.78 to 0.85 through occlusion( P<0.05). The pressure of superior vena cava(SVC) did not elevate and the mean pressure of SVC was13.97mmHg and 14.22 mmHg before and after occlusion( P>0.05). No complications associated with interventional procedures occurred. There was no significant decrease in blood oxygen saturation. Facial and upper limb edema, varicose veins in the chest and abdominal wall, pleural effusion and peritoneal effusion did not happen during follow up of 2 years. Two patients underwent TCPC operation one year and five years after interventional occlusion separately. Conclusion:Transcatheter occlusion of azygos/hemiazygos vein or PLSVC was feasible in patients with complex congenital heart diseases and venous stealing after bidirectional Glenn procedure on the premise of choosing suitable indications. Suitable indications meant that the pressure of pulmonary artery and superior vena cava was less than 20 mmHg in the basal state, and the pressure of superior vena cava was also less than 20 mmHg before the release of the occluder. Occlusions may increase the oxygen saturation of patients, improve the quality of life, and even promote the development of pulmonary vascular bed, so as to achieve the indications of TCPC operation in some patients.

Clinical observation on six children of left bundle branch area pacing / 中华儿科杂志

Objective@#To explore the safety and effectiveness of left bundle branch area pacing (LBBAP) in children.@*Methods@#Clinical data, pacing electrocardiogram and parameters of 6 patients (5 females and 1 male) who underwent permanent pacemaker implantation and LBBAP from January to June 2019 in the Department of Pediatric Cardiology of Anzhen Hospital were retrospectively analyzed.@*Results@#The weight of the 6 patients aged between 9 and 14 years ranged from 26 to 48 kg. Five patients were diagnosed with third degree atrioventricular block, and 1 patient was diagnosed with cardiac dysfunction after right ventricular apical pacing. Cardiac function decreased in one patient and remained normal in the other five patients. Left ventricular end diastolic diameter (LVEDD) Z score was 1.85±0.65. V1 lead showed changes like right bundle branch block in pacing electrocardiogram. No significant difference was found regarding QRS wave duration ((95±13) ms vs. (111±20) ms, t=-1.610, P>0.05) between preoperation and postoperation. Pacing threshold was (0.85±0.26) V. The sensing threshold was (15.0±4.3) mV and the impedance was (717±72) Ω. P potential was recorded in 3 cases. The earliest left ventricular local activation time was (56±5) ms and remained stable at different output voltages. Postoperative echocardiography revealed that the electrodes were located near the endocardium of the left ventricular septum. No complications such as myocardial perforation and electrode dislocation occurred during follow-up. The pacing threshold, sensing threshold and impedance were (0.60±0.09)V, (16.1±3.9)mV, (662±78)Ω respectively at 3 months after operation. The patient with low left ventricular ejection fraction (LVEF) recovered to normal on the 3 rd day after LBBAP (45% vs. 57%). The LVEDD Z score decreased to (1.1±0.3) at 3 months after operation and was significantly lower than that before operation (t=2.38, P<0.05).@*Conclusions@#LBBAP in children can achieve narrow QRS pacing andphysiological pacing with stablepacing parameters. It can improve left ventricular enlargement caused by long-term bradycardia, and cardiac dysfunction and cardiac enlargement caused by long-term right ventricular apical pacing quickly and effectively.LBBAP is safe and feasible for older children in the near future. However, the long term potential risks of LBBAP need further observation and study.

Analysis of clinical and electrophysiological characteristics of children with Wolff-Parkinson-White syndrome or ventricular pre-excitation / 中华实用儿科临床杂志

Objective@#To summarize the clinical and electrophysiological characteristics of children with Wolff-Parkinson-White syndrome or ventricular pre-excitation.@*Methods@#One hundred and seventy-nine consecutive children with Wolff-Parkinson-White syndrome or ventricular pre-excitation who were hospitalized at Beijing Anzhen Hospital Affiliated to Capital Medical University and planned to accept radiofrequency ablations were selected.Electrocardiogram and echocardiography were completed and detailed clinical data were collected before ablations.Anterograde refractory period of accessory pathways (ERPAPA) and retrograde ventriculoatrial conduction were measured during electrophysiology studies.The clinical and electrophysiological characteristics of these children were analyzed.@*Results@#The ratio of left-sided to right-sided accessory pathways was 1.001.93(61 cases vs.118 cases). Six out of 61 patients with left-sided accessory pathways were verified by electrophysiology study.There was no statistical difference of P-R interval and QRS duration between patients with right-sided and left-sided accessory pathways(all P>0.05). Twelve patients were combined with congenital heart diseases.Fourteen patients with right-sided accessory pathways were of ventricular pre-excitation induced dilated cardiomyopathy.ERPAPA was measured successfully in 110 patients, ERPAPA less than 250 ms accounted for 32.7%(36/110 cases). Six children with two pathways were recognized.Antidromic atrioventricular reentrant tachycardia was induced in 3 children.One child had slow conduction pathway localized in right-sided septum.@*Conclusions@#Right-sided overt accessory pathways are more common in children with Wolff-Parkinson-White syndrome or ventricular pre-excitation.Organic heart diseases are not uncommon.Besides congenital heart disease, a small proportion of patients with right overt accessory pathways may develop ventricular pre-excitatory induced dilated cardiomyopathy resulting from ventricular wall dyskinesia.Most of the decremental retrograde ventriculoatrial conduction is the characteristic of ventricular muscles rather than the accessory pathways in nature.Two pathways are not uncommon in children with Wolff-Parkinson-White syndrome or ventricular pre-excitation.Before finishing radiofrequency ablation, the anterograde and retrograde function of the accessory pathway should be evaluated in detail again because it is easy to neglect the existence of another accessory pathway.

Accessory pathway induced dilated cardiomyopathy / 中华实用儿科临床杂志

Accessory pathway-induced dilated cardiomyopathy is a new diagnosis proposed recently.Pre-excitation of part of the myocardium may mediate electrical and mechanical dyssynchrony,which will result in decreased left ventricular systolic function and increased diameter of left ventricle in patients with right-sided overt accessory pathways (type B ventricular preexcitation).Its features are listed as follows:(1) Incessant and prolonged tachyarrhythmia never happened.Tachycardiomyopathy and other causes of dilated cardiomyopathy are excluded.(2) Right-sided overt accessory pathways are shown by electrocardiogram.(3)Echocardiography indicates dyssynchronous left ventricle contraction,decreased left ventricular systolic function and increased diameter of left ventricle.Normal interventricular septal motion,recovered left ventricular and reversed left ventricular remodeling can be realized by radiofrequency ablation with a good prognosis.To infant patients,taking anti arrhythmia drugs to inhibit the conduction of the accessory pathway was recommended.

Clinical characteristics of ventricular preexcitation dilated cardiomyopathy induced by accessory pathway / 中华实用儿科临床杂志

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.

Adverse effects of type B ventricular pre-excitation on ventricular wall motion and left ventricular function: clinical analysis of 9 cases / 中华儿科杂志

<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>

Expression of hypoxia-inducible factor-1α and vascular endothelial growth factor in synovium of collagen-induced arthritis and its correlation with angiogenesis / 中华风湿病学杂志

Objective To investigate the effect of hypoxia-inducible factor (HIF)-1α and vascular en-dothelial growth factor (VEGF) on the angioge-nesis of rheumatoid arthritis (RA). Methods The collagen-induced arthritis (CIA) model was set up in male Wistar rats. The pathological angiogenesis and expression of HIF-1α and VEGF in the synovia different time points were observed by H&E and immunohistochemistry staining. Results The expressions of HIF-1α and VEGF on CIA synovium were significantly elevated and their expression increased gradually with the prolonged disease course. Both synovial HIF-1α expression and VEGF expression were correlated significantly with the pathological angiogenesis score. Synovial lining and sublining HIF-1α expression were correlated significantly with VEGF expression respectively. Conclusion H1F-1α may play an important role in the pathogenesis of RA by upregulating the expression of VEGF and then promoting angiogenesis.

Propythiouracil-induced antineutrophil cytoplasmic antibody-associated vasculitis:analysis of 6 cases / 中国免疫学杂志

Objective:To improve the knowledge on propythiouracil(PTU)-induced antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis.Methods:6 cases of PTU-induced ANCA-associated vasculitis were analyzed on clinical manifestation,laboratory and biopsy findings,therapy and prognosis.Results:All patients with Grave's disease had been treated with PTU from 2 months to 7 years suffered from ANCA-associated vasculitis, which can also appear after withdrawal of PTU. Clinical manifestations are different with only skin, joint, muscle involvements on mild cases or lung, renal and hematological involvement on severe cases. Sera from all patients were anti MPO-ANCA positive. All patients responded to withdrawal of PTU and administration of prednisone and immuno-suppressants well with ANCA declined or turned to negative.Conclusion:PTU can induce ANCA-associated vasculitis in the part of which lung and kidney can be seriously involved. Early withdrawal of PTU and administration of immunosuppressive agents might improve the prognosis.