Objective To summarize the surgical outcomes of neonates with critical and complex congenital heart diseases in the last 15 years to determine the optimal operation time and improve the prognosis.Method From January 2004 to December 2018,data of patients with complex congenital heart disease (CHD) who received surgery in our center were retrospectively analysed.All patients were diagnosed using echocardiography,some patients also received multidetector computed tomography (MDCT) examination.According to the operation date,patients were assigned into three groups:2004-2008,2009-2013 and 2014-2018 groups.Follow-up data were obtained from outpatient check-up and telephone interview.Result A total of 357 (261 males and 96 females) neonates were included.They received the operation at 2-28 days after birth and their body weights were 2 100 ～ 4 900 g.13.4％ (48/357) were diagnosed before birth.Percutaneous oxygen saturation (SpO2) ＜ 0.95 were detected in 315 patients (88.2％,315/357),among whom 60 had SpO2 ≤0.60(19.0％,60/315).Cardiac murmur were detected in 181 patients (50.7％,181/357).Among the 357 patients,221 neonates had transposition of the great arteries (TGA),55 had total abnormal pulmonary venous connection (TAPVC),43 had pulmonary atresia and intact ventricular septum (PA/IVS) and severe pulmonary stenosis and intact ventricular septum (SPS/IVS),20 had severe coarctation of the aorta (CoA)/interrupted aortic arch (IAA)/severe aortic stenosis (AS)/hypoplastic left heart syndrome (HLHS) and 18 had other types of complex CHD.The rate of onestage repair was 83.8％ (299/357) and 58 neonates underwent palliative surgery.28 patients died in hospital (7.8％,28/357).The rate of prenatal diagnosis in three groups increased [3.0％ (2/67),6.3％ (7/111),21.8％ (39/179)] and in-hospital mortality of all patients,especially patients with TGA,were remarkably decreased in the last 15 years [11.9％ (8/67),5.4％ (6/111),2.8％ (5/179)].329 patients survived after surgery and discharged.The follow-up rate was 97.9％ (322/329),7 patients were lost during follow-up,the median duration of follow-up was 63 months (3 ～ 183 months).5 died during follow-up,9 underwent second-stage radical operation,3 had re-operation due to the RVOT/LVOT stenosis.The majority of survivors were asymptomatic and with Ⅰ degree (NYHA classification) during follow-up.Conclusion Cyanosis is the most common manifestation in neonates with complex CHD.The rate of prenatal diagnosis increased gradually in the last 15 years,and in-hospital mortality of all patients,especially patients with TGA,was remarkably decreased.Most symptomatic neonates with complex CHD can survive radical operations with high success rate and good curative effect.Palliative procedures are also important for critical and complex CHD.

Objective To study the clinical manifestations,imaging features,treatment and outcome of umbilical venous catheters/peripherally inserted central venous catheters (UVC/PICC) associated pericardial effusion (PCE) and cardiac tamponade (CT) in neonates.Method Clinical data of cases with UVC/PICC associated PCE/CT that were found in neonatal intensive care unit of five hospitals in Shanxi province from January 2017 to December 2018 were extracted and retrospectively reviewed.Result In total,there were 632 cases received UVC/PICC insertions in 5 hospitals.7 cases of which were identified as PCE (1.0％) on echocardiography during the period of catheter indwelling,among which 5 cases suffered from PCE complicated CT (0.7％).7 cases with PCE included 6 premature infants and 1 full term infants with mean gestational age (32.6 ± 3.8) weeks and mean birth weight 1 550 (1 200,3 960) g.The mean age of PICC/UVC insertion was 14 h (1 ～ 19 h),the mean age of PCE/CT developed was 69 h (13 ～104 h) after insertion.The most common presentations included apnea/respiratory distress (7 cases),cyanosis/desaturation (7 cases),tachycardia (3 cases),bradycardia/asystole (5 cases).UVC/PICC tip ectopic was found in 6 cases after the symptoms of PCE emerged (4 cases at T6-T7 and 1 case at T12 vertebra level on chest X-ray,and 1 case at right atrium on echocardiogram).UVC/PICC tip was normal in 1 case (tip at T7-T8 vertebra level on chest X-ray).5 cases recovered after removal of catheter,pericardiocentesis and drainage of effusion;1 case recovered after removal of UVC without pericardiocentesis;1 case underwent pericardiocentesis,continued infusion for 5 h after extubated the UVC into inferior vena cava,then deceased.Conclusion UVC/PICC tip ectopic is the main cause of UVC/PICC associated PCE/CT.Immediate bedside echocardiography should be performed to any patient with UVC/PICC indwelling,who develops sudden unexplained apnea/respiratory distress,cyanosis,tachycardia/bradycardia/asystole.Timely removal of catheter and pericardiocentesis drainage may be life-saving.

Objective To study the effects of maternal gestational diabetes mellitus (GDM) on neonatal cardiac development.Method From January to December in 2016,full-term neonates of GDM mothers admitted to the neonatal department of our hospital were retrospectively included as the GDM group,and full-term neonates with non-GDM mothers during the same period as the control group.Their birth weight,length,placenta weight,birth weight/placenta weight ratio,echocardiographic cardiac measurements within the first week,and the incidence of congenital heart disease were compared between the two groups.Multivariate linear regression analysis was used to analyse the possible factors causing neonatal ventricular septal hypertrophy in GDM group.Result A total of 104 cases in the GDM group and 107 cases in the control group were included.Significant differences existed in birth weight,length and placental weight between the two groups (P ＜ 0.05).No significant differences in gender,gestational age and birth weight/placental weight ratio between the two groups (P ＞ 0.05).The width of the aorta and main pulmonary artery,the size of the left atrium,the left ventricle and the right ventricle,and the thickness of the ventricular septum in the GDM group were greater than the control group,the differences were statistically significant (P ＜ 0.05).The ventricular septal thickness of the GDM group was greater than the control group [≥3500g:(3.6±0.5) mmvs.(3.3±0.3) mm,＜3500g:(3.5±1.0) mmvs.(3.1 ± 0.4) mm],the difference was statistically significant (P ＜ 0.05).The incidence of congenital heart disease was 4.8％ (5/104) in the GDM group and 0％ in the control group.The difference between the two groups was statistically significant (P ＜ 0.05).Multivariate linear regression analysis showed that the interventricular septal thickness of the GDM group was positively correlated with the levels of HbA1c,HbA1 c,insulin of their mothers during pregnancy and birth weight of the newborn (P ＜ 0.05).Conclusion GDM mother may pose adverse effects on neonatal myocardial development.Further research is needed on its mechanism and how to monitor the incidence.

Objective To study the clinical characteristics,cardiac magnetic resonance (CMR)features,and genetype of hypertrophic cardiomyopathy (HCM) in neonates.Method HCM newborns diagnosed at pediatric department of Anzhen Hospital Affiliated to Capital Medical University from January 2015 to December 2018 were retrospectively collected.The clinical manifestations,imaging features and genetype were analyzed.Result During the study period,there were 11 227 deliveries and 11 397 live births.52 of the parturient women were diagnosed as HCM before or during pregnancy (0.46％,52/11 227).54 neonates were delivered from HCM mothers.Among them,5 were diagnosed as HCM (5/11 397,0.04％).In addition,there was another infant patient who was admitted from another hospital.In total,6 HCM infants were included in this study,with 4 males and 2 females.The average gestational age of the infants was (36.6 ± 1.2) weeks,with 3 preterm infants,and the others were full-term infants.The median age of diagnosis was 1 day.The clinical manifestation was that 4 patients needed rest in the course of suckling,3 of them also had accompanied symptoms,with pale face,cyanosis while crying and sweating in each case.CMR showed that the ventricular septum was (6.80 ± 1.78)mm,ejection fraction was (60.0 ± 2.8)％.All six patients showed septal thickening on CMR,including a case with right ventricular myocardial hypertrophy,another with left ventricular outflow tract obstruction and late gadolinium-enhanced.The infants were all treated with metoprolol.They were followed up until 6 months to 2 years old.Among them,4 cases had no progress in myocardial thickening.Two cases had progressive myocardial thickening,one of them was diagnosed as end-stage HCM at 2 year-old.Three MYBPC3 mutations,one MYH7 gene mutation,one MYH7 gene mutation combined with TMPO gene mutation and one RYR2 gene mutation were detected.Conclusion The neonatal HCM is characterized by thickening of ventricular septum,outflow tract obstruction and delayed enhancement of CMR.The most common HCM mutations were MYBPC3 and MYH7,the most common clinical symptoms were breast feeding interruption,followed by cyanosis,pale and sweating.It is suggested that beta-blockers should be recommended for neonates with hypoxic episodes or outflow tract obstruction.

Objective To study the characteristics of urinary metabolites in the premature infants with or without bronchopulmonary dysplasia (BPD) within 36 hours after birth and to find new biomarkers for the early warning indicators for BPD.Method From January 2014 to October 2016,premature infants hospitalized in the Sixth Affiliated Hospital of Sun Yat-sen University with gestational age ＜ 32 weeks,hospitalization time ＞ 28 days and urine samples were collected within 36 hours after birth for metabolite detection were enrolled in the study.Preterm infants diagnosed as BPD were selected as the BPD group.Preterm infants with the same gestational age,days of age with the BPD group had no BPD were selected as the control group at a ratio of 1 ∶ 1.The gas chromatography-mass spectrometry was used to measure the metabolites.The data were analyzed using orthogonal partial least squares discriminant analysis and receiver operating characteristic (ROC) curve and the area under which were used to determine the performance of differential metabolites in the diagnosis of BPD.Result There were 20 patients in the BPD group and 20 patients in the control group.Within 36 hours after birth,in the BPD group,the level of fucose and tartrate in urine (nmol/mgCr) were significantly lower than that in the control group [0.00 (0.00,0.03)vs.0.07 (0.00,0.41);0.00 (0.00,0.01) vs.0.02 (0.00,0.06),respectively].The level of kynurenic acid and thymine (nmol/mgCr) were significantly higher than the level in the control group [0.04 (0.00,0.43) vs.0.00 (0.00,0.00);7.10 (0.00,14.47) vs.0.00 (0.00,0.22),respectively].All the differences were statistically significant (P ＜ 0.05).ROC curve analysis showed that the area under the curve for the diagnosis of BPD in combination with the four metabolites was 0.857 (95％ CI 0.732 ～0.982).Conclusion Changes in urinary metabolites such as fucose,thymine,kynurenine and tartaric acid in preterm infants may be related to the development of BPD.Early detection of these four metabolites has potential in the early diagnosis of BPD,and may warn against the occurrence of BPD.

Objective To study the video-electroencephalography (vEEG) background activities of very premature infants within the first 7 days after birth.Method The inclusion criteria were gestational age (GA) within 31 weeks and bedside vEEG monitoring within first 7 days of birth.Those with severe cranial ultrasound (CUS) findings,refused treatment,dead and with seizures on vEEG were excluded.The clinical information of included cases were reviewed and the continuity and sleep-wake cycle (SWC) on their EEG background activities were analysed.Result From June 2017 to April 2018,a total of 56 cases were included.Their GA were (29.4 ± 1.6) weeks,and birth weight were (1 306 ± 316) g.A total of 31 cases were diagnosed with asphyxia(1 min Apgar score less than 8),and no one with 5 min Apgar score below 5.Their umbilical artery blood pHs were 7.29 ± 0.07.Their first CUS and the CUS at corrected gestational age (cGA) full term were all within the normal range.The Gesell neurological assessment at cGA 6 months were all within the normal range.As the cGA increased,the continuity of EEG background activities became more continuous as the normal continuous/discontinuous pattern.The 95％ confidence interval of max interburst interval was 95 s.The SWC first occurred at GA 26 w,but was immature,then became mature as the cGA increased.Conclusion The EEG background activity analysis of relatively normal very premature infants within first week shows that interburst interval can be longer than the previous report,and the SWC first appear at GA 26 w.

Objective To study the difference of clinical outcome between late preterm twins (LPT) and late preterm singletons (LPS),and the influence of assisted reproduction technology (ART) or natural conception on the clinical outcomes of LPT.Method The data of late preterm infants born in the Beijing Obstetrics and Gynecology Hospital between January 2016 and January 2018 were retrospectively collected.Infants were assigned to LPT group or LPS group according to twins or singleton status.The differences of the demographic data,morbidity,oxygen therapy rate,respiratory support rate,mortality,hospitalization rate and hospitalization time between LPT and LPS were compared.In order to analyze the effects of different ways of conception on LPT,LPT was further divided into assisted reproduction technology (ART) subgroup and natural pregnancy subgroup.Result A total of 1 824 late preterm infants were included in the study,including 582 cases of LPT and 1 242 cases of LPS.The birth weight and body length of LPT were lower than those ofLPS [(2572±395)gvs.(2614±413)g,P＜0.05;(46.5±1.1)cm vs.(47.5 ± 1.0)cm,P ＜0.01],while the cesarean delivery rate of LPT was higher than that of LPS [90.7％ (528/582) vs.39.9％ (496/1 242),P ＜ 0.01].The incidence of respiratory distress syndrome (RDS) in LPT was higher than that of LPS [5.2％ (30/582) vs.3.6％ (45/1 242),P ＜ 0.05],and the length of hospitalization of LPT was also longer than that of LPS[(8.9 ±3.1)d vs.(7.2 ±2.9)d,P ＜0.01],the differences were statistically significant.There were no significant differences between groups in neonatal pneumonia,apnea,hypoglycemia,sepsis,neonatal necrotizing enterocolitis (NEC),grade Ⅲ ～ Ⅳ intracranial hemorrhage,severe asphyxia,bronchopulmonary dysplasia (BPD),oxygen therapy rate,respiratory support rate and mortality rate.There was also no significant difference between ART subgroup and natural pregnancy subgroup in gestational age,gender,cesarean section rate,birth weight,and birth length (P ＞ 0.05).There was no significant difference in RDS,pneumonia,apnea,hypoglycemia,sepsis,NEC,Ⅲ ～ Ⅳ intracranial hemorrhage,severe asphyxia,BPD,oxygen uptake rate,respiratory support rate,mortality rate and hospitalization time between the two subgroups (P ＞ 0.05).Conclusion The birth weight and birth length of LPT are lower than those of LPS,and the incidence of RDS is higher.The hospitalization time of LPT is also longer than that of LPS.It is necessary to strengthen the high-risk management of twin pregnant women and post-natal management of LPT.

Objective To study the value of lung ultrasonography (LUS) in the diagnosis of pneumothorax in critically ill neonates.Method The neonates admitted to our NICU and suspected to have pneumothorax were prospectively enrolled from June 2017 to December 2018.All eligible infants received both LUS examination and chest X-ray.The characteristics of LUS imaging was analyzed based on the chest X-ray which was used as the golden standard for the diagnosis of pneumothorax.The sensitivity,specificity,positive predictive value and negative predictive value of LUS is computed.The duration of LUS and chest X-ray were compared.The outcome and complications were also observed.Result Fifty neonates with suspected pneumothorax were collected.Among them,pneumothorax was confirmed with chest X-ray in 31 neonates (62.0％).Ultrasound signs of pneumothorax included absence of lung sliding (100％),absence of B lines (100％),stratosphere sign (100％) were observed in all of the 31 neonates.Presence of lung point was also observed in 90.3％ of the patients.The sensitivity,specificity,positive predictive value,negative predictive value and X-ray coincidence rate of LUS in the diagnosis of pneumothorax were 100％.LUS and chest X-ray examination took (5.6 ±5.1) min and (20.1 ± 12.2) min,respectively,the difference was statistically significant (P ＜ 0.05).All 31 infants with pneumothorax survived.15 infants underwent closed thoracic drainage after emergency thoracic puncture or aspiration assisted by LUS.No postoperative complications occurred.Conclusion LUS showed high accuracy,sensitivity and specificity in detecting pneumothorax in critically ill neonates.It is simple to operate and can guide clinical rescue more promptly and quickly.

Objective To study efficacy and safety of continuous renal replacement therapy (CRRT) in the treatment of neonatal sepsis-related acute kidney injury (AKI).Method From June 2011 to June 2018,neonates with sepsis-related AKI hospitalized in the neonatal intensive care unit of our hospital and treated with CRRT were enrolled.Before CRRT,12 h,24 h,48 h after CRRT and by the end of CRRT,their clinical data including renal function,acid-base balance,electrolytes,blood pressure (BP)and the change of hemodynamic indexes were retrospectively analysed.The efficacy and safety of CRRT was evaluated.Kruskal-wallis H test was used for statistical analysis.Result A total of 9 cases of sepsis-related AKI neonates were enrolled in the study,all treated with continuous veno-venous hemofiltration dialysis.5 cases had oliguria,2 cases fluid overload and 2 cases shock.The duration of CRRT was 49 ～ 110 h (76.2 ±23.5) h.12 h after CRRT,BP were maintained at 40 ～60 mmHg and stable during the treatment,the blood pH value increased to 7.35 ～ 7.45 and the oxygenation index reached 200 mmHg.24 h after CRRT,the oxygenation index rose to more than 300 mmHg.Serum potassium,urea nitrogen and creatinine levels decreased significantly after 12 h of CRRT,and reached the normal range after 24 h of CRRT.After 24 h of CRRT,the urine volume significantly increased.Venous catheterization was performed successfully in 9 cases.2 cases had thrombocytopenia,1 case catheterization obstruction and 1 case hypotension during CRRT.No complications such as hypothermia,hemorrhage,thrombosis or infection occurred.All 9 patients were cured and discharged.Conclusion CRRT is safe and effective for the treatment of neonatal sepsis-related AKI.

Objective To study the clinical efficacy and safety of extracorporeal membrane oxygenation (ECMO) in critically ill neonates.Method From November 2016 to September 2018,the clinical data of 5 cases who received ECMO treatment in NICU of our hospital were retrospectively analyzed.The indication of ECMO was reversible respiratory failure irresponsive to conventional therapy.The treatment mode was V-A ECMO.Oxygenation index (OI),vasoactive-inotropic score,blood lactate before and 24 h after ECMO were recorded.Complications of ECMO were also studied.Paired t-test was used to compare the pre and post treatment parameters.Result Among the 5 cases,4 cases were male and 1 case was female.3 cases were diagnosed with meconium aspiration syndrome,2 cases pulmonary hypertension.OI[(9.5 ± 1.8) vs.(60.6 ± 19.4)],vasoactive-inotropic score[(19.5 ± 12.0) points vs.(204.0 ± 143.8) points]and blood lactate [(2.8 ± 1.5) mmol/L vs.(9.6 ± 3.6) mmol/L]) were all significantly decreased at 24 h after ECMO treatment (P ＜ 0.05).During follow-up,3 cases survived,2 cases died.All the 5 cases showed thrombocytopenia,3 cases developed renal failure and received continuous renal replacement therapy,1 case got intracranial hemorrhage.2 of the 3 survived cases developed neurological impairment and need long term follow-up and rehabilitation therapy.Conclusion ECMO treatment has remarkable effects on critically ill neonates and may actually save lives,but the risk of complications are quite high.