Arrhythmias in children with congenital heart disease may be associated either with the underlying abnormality or result from surgical intervention.The treatment rationale bases on the rapid and accurate diagnosis.As a general rule,risk-benefit analysis of drug therapy should be considered when facing an arrhythmia in a child,due to the negative inotropy.In the last decades,non-pharmacological therapy is progressively used in pediatric population.

Pulmonary arterial hypertension is common in patients with congenital heart disease.An effective reduction in pulmonary vascular resistance could improve the survival rate in the future.Research in the pathophysiology of pulmonary arterial hypertension shows that phosphodiesterase 5 inhibitors result in pulmonary vasodilation,decrease vascular resistance.Sildenafil,a inhibitor of phosphodiesterase 5 has been permitted to treat pulmonary arterial hypertension oversea now.Phosphodiesterase 5 inhibitors are proved to reduce pulmonary vascular resistance significantly,improve the survival rate in the future and be well tolerated.This review contains the changes of hemodynamics,exercise tolerance,side effect and pharmacokinetics of phosphodiesterase 5 inhibitors.

Objective To compare the count of circulating endothelial cells (CECs) between patients with reversible pulmonary hypertension (RPH) and irreversible pulmonary hypertension (iRPH) by flow cytometry in order to find a new biomarker to distinguish RPH from iRPH.Methods Ninety pulmonary hypertension associated with congenital heart disease patients treated in our hospital between September 1,2013 to March 31,2014 were enrolled in this study.According to the mean pulmonary arterial pressure (mPAP) measured by echocardiography in six months after treatment,the patients were separated into 2 groups:iRPH group(mPAP≥50 mmHg),RPH group(mPAP ＜50 mmHg).Results Totally the clinical data of 87 patients were enrolled in the statistic analysis,86 patients finished the follow-up in 6 months after the surgery,1 patient died from pulmonary hypertensive crisis.The count of CECs was not correlated with the extent of pulmonary hypertension(P =0.925).The number of CECs was higher in iRPH group than that of RPH group(0.46‰ vs.0.09‰,Z =-5.021,P =0.000).And also the age of patients in iRPH group was elder than that of patients in RPH group [(43.1 ± 37.4) months vs.(9.3 ± 12.6) months,t =-5.079,P =0.000].Conclusion The count of CECs is significantly increased in iRPH patients.It could be used as one biomarker to distinguish RPH from iRPH.

Objective To evaluate accurately the cardiac output, an early post-operative indicator for the cardiac function, after cardiac surgery in the neonates with congenital heart diseases and assess the risk factors for surgery, with an aim at exploring the early strategy for decressing the mortality. Methods From January 2007 to November 2007, forty-seven consec-utive neonates with complex congenital heart diseases underwent surgical correction at Shanghai Children's medical center.There were 38 boys and 9 girls with age from 3 days to 29 days [mean age (21.98 +8.15) days] and weight from 2.6 kg to 4.2 kg [ mean (3.49±0.51 ) kg]. 15 patients had delayed sternal clousure. The operations were performed with hypothermia, deep hypothermia low flow, or deep hypothermia circulatory arrest techniques. Cardiac index (CI) was derived from pulse contour analysis and calculated with the PiCCO plus system. Meanwhile, serum cardiac troponin I ( cTnl), mixed venous oxygen saturation ( SvO_2 ) and cardiopulmonary bypass (CPB) time were measured. Finally, the risk factors for surgical treatment in survivors and that associated with an increased mortality were analyzed. The association between post-operative cardiac output and the death after surgery was examined. Results Four neonates died after surgery, with a surgical mortality of 8.5%. CIvalue in the neonates [ (2.0±0.3 ) liters per minute per square meter of body surface was less than normal 2.5±0. 3. CI was associated inversely with CPB time but had a positive correlation with SvO_2. No significant difference association between CI and cTnI was observed. The value of cTnl was associated with the type of surgical procedures. Cases for which DHCA and low flow cerebral pefusion technique were used were free from neurological complications, ischemia in the lower extremities and oli-guria. The duration of cardiopulmonary bypass, urgent state of the operation and the abnormal coronary artery were associated with high mortality in the neonates after cardiac surgery. Conclusion The cardiac output of the neonates in whom cardiac pro-cedures were performed is adequate for the the oxygen supply required by the whole body though it is slightly lower than nomad after cardiac surgery. Higher mortality in the neonates during early postoperative period may be due to the complexity of the primary diseases, prolonged cardiopulmonary bypass time, residual abnormalities and severe acidosis before operation. Various techniques for CPB and that for the myocardial protection are safe and can provide an appropriate operative field.

Objective To analyze the safety and effect of non-invasive pressure support ventilation in 32 patients by using a helmet and to give the appropriate way of patients who need non-invasive ventilation ( NIV) support after congenital heart disease surgery. Methods Patients over one year old after congential heart disease surgery were admitted in our Department of Cardiovascular Thoracic Surgery from July 2015 to December 2015. Patients who get clinically improved within one hour were divided into the early improved group( Group-E) ,otherwise they were classified to non-early improved group( Group-NE) . The general infor-mation,diagnosis, indication of NIV, ICU and hospital stay, complications, and mortality were collected. Results Thirty-two patients were engaged in this study,including 18 patients(56. 25%) in Group-E and 14 patients(43. 75%) in Group-NE. Patients who got improved in the first hour might have a higher incidence of avoiding reintubation[83. 33%(15/18) vs. 42. 86%(6/14),P=0. 02]. The heart rate,respiratory rate, pH,PaO2/FiO2 and lactate were improved in Group-E compared with Group-NE after the first hour by using helmet. At the end of NIV,the oxygenation showed no difference but the PaCO2 was lower in Group-E. In Group-E,the values showed a trend of improvement,while the values in Group-NE showed not only no statis-tical significance in different time points but also seemed to have a tendency of hypercapnia and reduced com-fort behavior scale in the end of NIV. There were 6 cases in Group-E and 10 cases in Group-NE developed ventilation associated pneumonia with the incidence of 33. 33%(6/18) and 71. 43%(10/14),respectively, which was significant difference (χ2 =4. 571,P =0. 03). The total duration of mechanical ventilation of Group-E was shorter than that of Group-NE [ ( 136. 72 ± 151. 49 ) h vs. ( 252. 79 ± 155. 33 ) h, P <0. 05 ] . Conclusion NIV through a helmet in children could be well tolerated and avoid re-intubation. Patients who get improved earlier may have more clinical advantages,such as less time of mechanical ventilation and lower incidence of postoperative complications. Early improvement can be considered as a valuable indicator wheth-er the patient needs to use NIV continuously.

Objective To investigate the riskf actors associated with sepsis following surgery of con-genital heart disease in children, for early-recognizing and diagnosing, improving prognosis and decreasing mortality.Methods Retrospective study wa s employed,52 patients of sepsis and 104 patients of non-sepsis with similar age,sex,na d the same diseases were enrolled during Jan 2012 to Apr 2015 in cardiac intensive care unit.Variables such as age,sex,preoperative infection,delayed sternum closure,diaphragmatic paralysis, exploraot ry chest,cardiopulmonaryb ypass time, placed invasive catheter were included in the research fac-tors.The Logistic regression model was set up,OR and 95%CI were calculta ed.Results In sepsis group,the more shock and dysfunction organs,longre hospital stays,longer ICU stays and higher mortality were found compared to the non-sepsis group[25.32%vs.6.73%,3.5 ±1.1 vs.1.1 ±0.7,(35.1 ±11.2)d vs.(11.3 ±3.1)d,(21.3 ±7.1)d vs.(7.1 ±2.3)d,19.23%vs.4.81%,P<0.05].In the Logistic regres ion mod-e l,variables significantly associated with sepsisw ere preoperative infection,deal yed sternal closure,explorato-ry chest,and placed more invais ve duct,diahp ragmatic paralysis(P<0.05).OR value (95%CI) were 10.53 (1.73,64.2),26.66(2.69,263.83),19.47(1.87,203.02),4.99(1.36,18.31),8.32 (0.12,16.46 ), respectively.Conclusion Preoperative infection,delayed sternal closure,exploratory chest,placed more inva-sive duct,diaphragmatic paralysis are the risk factors of sepsis.Children with sepsis had poor clinical outcome compared to those without sepsis.

Objective To evaluate the risk factors,diagnostic index of the restrictive right ventricle physiology(rRV) and the impact of the mid-term outcome of the patients.Methods Eighty patients(30 in rRV group and 50 in non-rRV group) undergoing TOF repair admitted in our department from Oct 2011 to May 2012 were studied.Perioperative clinical data were collected and echo data were recorded after operation.Mixed linear regression for repeated measures was used to compare the variables and analyze the correlations.Results Patients in rRV group were younger with longer cardiopulmonary bypass(CPB) time,aortic cross clamp time,ventilation time,intensive care unit and hospital stay compared with those in non-rRV group(P<0.01,respectively).The younger patients with longer CPB time had high risk of rRV by logistic regression analysis.Within 7 days post operation,the increase of saturation of venous oxygenation and decrease of oxygen extraction ration were slower in rRV group than those in non-rRV group(P<0.05,respectively).Lactate decreased in both groups,but was higher in rRV group throughout the 7 days(P=0.03).NT-proBNP was higher in rRV group throughout the 7 days than that in non-rRV group.NT-proBNP≥4750pg/ml often indicated the patients in the state of rRV.CRP slightly increased in 1-2 days post operation,and decreased thereafter,and the decrease was slower in rRV group(P=0.08).With regard to the mid-term outcome,there was no significant differences in the incidences of the obstruction of the right ventricle outflow and main pulmonary artery,the stenosis of the branch of pulmonary artery and the degree of the pulmonary valve regurgitation.Conclusion rRV is associated with significantly higher levels of NT-proBNP and CRP.The incidence of rRV correlates with age on operation and positively correlates with CPB time.NT-proBNP would be regarded as an indicator of the incidence of rRV.The study indicated the rRV would have impact on the early outcome of the patients but there was no significant effect on mid-term outcome.

Training quality postgraduates in clinical neurology differs from in neuroscience. Combined with the features of clinical neurology, this article discussed the training processes for quality postgraduates in clinical neurology as following: individual training plan; clinical training in multiple academic directions and subjects; keeping the update ability for clinical neurology; improving the English skill in neurology and the ability to develop clinical researches.

Objective To study the risk factors of prolonged postoperative recovery after the total cavopulmonary connection(TCPC) in the current era.Methods Data on all patients admitted to the cardiac intensive care unit (CICU) after a TCPC between January 2013 and March 2014 were retrospectively analyzed.We excluded all patients who died and required TCPC takedown.The study cohort was further divided into a prolonged recovery group that included patients with 75％ ile for duration of mechanical ventilation or pleural drainage,and a standard recovery group which included all other patients.A multivariable logistic regression model was used to compare demographic,anatomic,and physiological variables between the prolonged and standard recovery groups.Then,the cohort was separated into a high volume resuscitation group and a low volume resuscitation based on the 75％ ile for volume resuscitation(ml/kg) administered on the first three days after the TCPC.Results Totally 118 TCPC operations were performed.Of the study population (n =118),the median age was 3.8 years (3.1 to 4.8 years) and median weight was 14.8 kg(13.3 to 17.1 kg).The most common diagnosis was double outlet of right ventricle (n =47,39.8％).The extracardiac conduit fenestrated TCPC was the most common surgery(n =79,66.9％).Within the study population,43 (39.8％) patients met criteria for prolonged recovery.Univariate risk factors for prolonged recovery included higher preoperative mPAP(P =0.022),atrioventricular valve regurgitation (P =0.000),longer total bypass time (P =0.044),higher postoperative central venous pressure (P =0.000),AST (P =0.001),ALT (P =0.010),NT-proBNP (P =0.000),SaO2 (P =0.012),I n-otropic score (P =0.001),higher incidence of arrhythmia (P =0.000),low cardiac output syndrome (P =0.000),need for peritoneal dialysis (P =0.000),and requirement for greater volume resuscitation during the 72 postoperative hours(75％ for the entire group,P =0.000).In a multivariable Logistic model,need for greater volume resuscitation (OR 10.860,95 ％ CI 2.681,43.987) and the higher postoperative central venous pressure (OR 1.446,95 ％ C I 1.113,1.879) were the only two independent risk factors for prolonged outcome after the TCPC.Conclusion The need for high volume expansion and higher central venous pressure were the risk factors of mediate prolonged recovery.

Objective To analyze the influencing factors of surgical management for neonatal complicated congenital heart disease(CCHD),sumarize the clinical experience and improve the surgical effect.Methods The clinical data of 154 neonates with complicated congenital heart disease who were undergone surgical management from August 2008 to August 2013 were retrospectively analysed.Results There were 14 cases of death with a mortality rate of 9.1％ (14/154),including 4 cases of preoperative death.The operative motality is 6.7％ (10/150),including 3 cases of single malformations(D-TGA/VSD,PA/VSD,IAA),and 7 cases of multiple malformation(HRHS/PS in 2,D-TGA/COA in 1,D-TAG/PS in 1,COA/SV in 1,SV/PA/IVS in 1 and D-TGA/COA/SV in 1).140 cases accepted regular follow-up after hospital discharge from 3 months to 5 years,8 of them underwent secondary surgery and all survived.Conclusion Timely diagnosis,actively improvement for preoperative internal environment,fine operative technique,individual cardiopulmonary bypass and intensive postoperative care are all important influencing factors of surgical effect for neonatal severe complicated congenital heart disease.