Objective:To review and analyze the clinical experience of children with aortic valve stenosis and/or insufficiency treated with autologous pulmonary valve for aortic valve replacement procedure(Ross operation) with ePTFE artificial valve for right ventricular outflow tract reconstruction.Methods:From 2015 to 2020, 8 cases of aortic stenosis and/or aortic insufficiency treated by Ross operation in our center were collected, with an age of 0.5-13.2 years old. 4 cases of aortic stenosis were diagnosed preoperatively, 3 cases of aortic stenosis with aortic insufficiency, and 1 case of infective endocarditis involving the aortic valve. The operation was carried out in three steps: Harvest autologous pulmonary valve; the diseased aortic valve was resected and autologous pulmonary valve was transplanted to the aorta by aortic root transplantation; the right ventricular outflow tract was reconstructed by a handmade ePTFE artificial flap blood vessel.Results:In 6 cases, the right ventricular outflow tract was reconstructed by hand-sewn ePTFE trileaflets, and artificial univalve in 2 cases, no death occurred during operation; all patients were cured and discharged. The patients were followed up for 1 to 36 months, with mean of(12.63±12.19) months. There was no long-term death or valvular complications. During follow-up echocardiography indicated 1 case of moderate aortic regurgitation, 1 case of mild-moderate regurgitation, and moderate regurgitation was found in 2 patients with artificial single pulmonary valve. For the remaining patients, they were mild aortic regurgitation, and a trivial or mild pulmonary artery regurgitation with hand-sewn three-leaflets ePTFE artificial vessel; All patients were followed up at the last time with a peak pressure of(6.63±3.46) mmHg(1 mmHg=0.133 kPa) across the aortic valve. The left ventricular outflow tract and aortic annulus shrank slightly after surgery(the diameter of one patient with Ross-Konno operation increased), but the annulus diameter increased with age. There was no need for further intervention.Conclusion:The Ross operation is safe for the treatment of aortic valve disease, it has good hemodynamic effect, and the autologous pulmonary artery has growth potential, especially suitable for children and young patients. Hand-sewn ePTFE with trileaflet vessels for reconstruction of right ventricular outflow tract performs well in anti-regurgitation function in the short term or may be used as a replacement material for the homograft/heterograft vessel, but longer follow-up and more cases are needed.

Objective:To discuss the effect of modified ultrafiltration combined with sequential infusion of blood products, such as platelets and cryoprecipitation, on perioperative coagulation function in neonates undergoing cardiovascular surgery under extracorporeal circulation.Methods:A retrospective analysis was performed on 83 neonates who underwent cardiovascular surgery from January 2017 to December 2019. They were divided into the control group (conventional ultrafiltration, 51 cases) and the treatment group (modified ultrafiltration + sequential infusion of blood products, 32 cases).Results:The age of treatment group was significantly younger than that of the control group ( P<0.05). The extracorporeal circulation time of the treatment group was significantly longer than that of the control group. Compared with the preoperative data, post-APTT, post-PT and post-INR were increased significantly in the control group, platelet count and FIB were decreased significantly ( P<0.01), while there was no significant difference in the treatment group. Compared with the treatment groups, post-APTT, post-PT and post-INR were significantly increased in the control group, and postoperative platelet count and FIB were significantly decreased ( P<0.05). The drainage of control group was significantly higher than that of the treatment group at 12 h and 24 h after surgery ( P<0.01). Conclusion:The application of modified ultrafiltration combined with sequential infusion of blood products can significantly improve perioperative coagulation and reduce mediastinal bleeding in neonatal after cardiovascular surgery.

Objective: To analyze the neurodevelopmental outcome and its risk factors in infants with CHD at 18 months of age. Methods: Eighteen-month-old infants with CHD at the follow-up clinic of our hospital were selected. The Bayley scales of infant development(BSID) were used to evaluate the levels of mental development(MDI) and psychomotor development(PDI). The clinical features during hospitalization were reviewed, and the risk factors of MDI and PDI were analyzed. Results: A total of 116 children with CHD underwent BSID evaluation at 18 months of age. Both the MDI(95.38±22.98) and PDI(87.84±22.57) of the cohort were significantly lower than the average value of the normal population(P<0.05). In infants with cyanotic CHD, the MDI was higher(β=17.218). The longer the length was of the hospital stay, the lower the PDI(β=-0.577). In patients undergoing cardiopulmonary bypass surgery, the PDI was higher(β=11.956). Compared to infants with relatively normal behavior, the PDI of infants with mild behavioral problems was lower(β=-10.605). Conclusion Children with CHD who underwent cardiac surgery have delayed neurodevelopmental outcomes compared with those of healthy children. The outcomes of infants with cyanotic CHD or undergoing cardiopulmonary bypass surgery was better than others. Long hospital stays and mild behavioral problems were risk factors for poor neurodevelopmental outcomes.

Objective To review the clinical experience with the Norwood stage Ⅰprocedure.Methods Between June 2016 to October 2018, totally 5 neonates underwent Norwood stage Ⅰ procedure.There were 3 boys and 2 girls, weighing(2. 98 ±0.60)kg with median of 2.95 kg.Age at surgeries ranged from 1 to 8 days.All 5 cases underwent the Norwood stage Ⅰprocedure under deep hypothermic circulatory arrest, including 3 cases of modified Blalock-Taussig shunts (MBTS) and 2 ca-ses of RV-PA shunt.Results The third case was successfully closed the chest on postoperative day 2 and extubated, but died from DIC due to severe infection on the postoperative day 6.The fifth case died from pericardiol tamponade at 10 hours after the operation.The first,second and fourth cases were followed up 1 month after discharge with NYHA Ⅰ, but the first and second cases dropped out of follow-up due to some personal resaons.The fourth case underwent the bidirectional Glenn procedure 9 monthes after the stage Ⅰ procedure and recovered smoothly.Conclusion The application of these modified methods and te-chiques based on the classical Norwood procedure help to accumulate experience in sugical treatment of HLHS in China .

Objectlve To explore the correlation between amplitude-integrated electroencephalographic(aEEG) findings and clinical features and to investigate the status of perioperative brain function in infants with critical congenital heart disease (CCHD) and its influencing factors.Methods Newborns and infants with critical CHD who were admitted to the NICU at our hospital were included.Postoperative aEEG was continuously monitored and analyzed,and its correlation with clinical conditions was compared.Results A total of 226 patients were enrolled.Of the 226 patients who underwent postoperative aEEG monitoring,approximately 5.8％ showed mild abnormal background patterns,0.9％ showed severe abnormalities,27.4％ demonstrated an immature SWC,and 3.5％ lacked SWC.The patients who had a history of hypoxia at birth exhibited delayed sternal closure or showed severe postoperative neurological symptoms and had higher probabilities of postoperative SWC abnormalities.Several infants,all with complex CHD,had postoperative seizures.Conclusion Gestational age and oxygen deficiency at birth were the risk factors of brain injury.Delayed sternal closure,severe postoperative infection,and postoperative neurological symptoms were risk factors for postoperative brain injury.Postoperative nervous system monitoring and prevention postoperative severe infection may obviously improve the brain function of neonates and small infants with critical congenital heart disease.

Objective To determine the long term outcomes of laryngomalacia infants with anomalies and to determine the clinical practice guideline for these infants.Methods The charts of infants with moderate to severe laryngomalacia,who were admitted to our hospital between January 2013 and December 2015,were retrospectively reviewed.These infants were divided into two groups,anomaly(A) group(n=37) and non-anomaly (NA) group(n=19).Results Fifty-six cases were enrolled.Infants in A group were older at symptom relief than those in NA group[(10.00±3.56) months vs.(7.89±3.03) months,P＜0.05],and the weight percentiles of infants in A group were lower at 3,6 and 12 months than those in NA group(P＜0.05).There was no statistically significant difference between the two groups on the weights percentiles in infants at 24 months after diagnosis.Five of 37 cases in A group and 3 of 19 cases in NA group had supraglottoplasty.One infant in A group had tracheotomy.Conclusion Both breathing difficulty and development retardations of infants with moderate or severe laryngomalacia could gradually improved with age.There is not enough evidence to support the aggressive supraglottoplasty for infants with anomalies and laryngomalacia.

Objective To determine the effectiveness of systolic blood pressure gradient between the right arm and the right leg(SBPG) tests in the diagnosis of neonatal aorta anomaly(AoA).Methods The SBPG of infants suspected of having critical congenital heart diseases were collected prospectively,who were admitted to Guangdong General Hospital from January 2013 to December 2015.The results of SBPG test were compared with those of echocardiography or cardiac computed tomography (golden standard).The rates of true positive,true negative,false positive,false negative were calculated under cutoff values of 5 mmHg(1 mmHg =0.133 kPa),10 mmHg,15 mmHg and 20 mmHg,respectively.Receiver operating characteristic (ROC) curves was used to compare tests of different cutoff and the areas under the ROC curve were also calculated.Results Among 664 enrolled infants,67 cases were confirmed by golden standard test.The systolic blood pressure in the right arm,the legs and SBPG in AoA group and non-AoA group were (88.0±20.4) mmHgvs.(73.4±9.3) mmHg (P＜0.01),(66.1 ±10.1) mmHg vs.(69.0 ±9.7) mmHg(P＞0.05) and (22.6±17.8) mmHgvs.(2.3 ±4.8) mmHg(P ＜0.01),respectively.In these patients,31 cases(46.3％),31 cases(46.3％),27 cases(40.3％) and 21 cases(31.3％) were diagnosed of AoA,and 36 cases(53.7％),36 cases (53.7％),40 cases(59.7％),and 46 cases (68.7％) were missed by SBPG tests of 5 mmHg,10 mmHg,15 mmHg and 20 mmHg,respectively (P ＜ 0.01).The rates of true negative among those groups were 94.1％,99.5％,99.7％ and 100.0％,and the areas under ROC curve were 0.656,0.722,0.695 and 0.657,respectively (P ＜ 0.01).Conclusions Almost half of AoA infants could be screened out by SBPG test.The cutoff of 10 mmHg could probably be used to screen potential AoA infants,with higher true positive rate and lower false positive rate.

Objective To investigate the correlation between the indications,findings,interventions of fibrobronchoscopy(FB) in neonates and their correlative diseases with neonatal FB results and clinical data.Methods Retrospective case series of 243 consecutive patients of 28 days old or younger were investigated underwent FB for the first time from January 2010 to December 2014,at a tertiary care hospital.The common indications for FB and detection rate of respiratory tract diseases were collected.If the findings of FB had significant associations with premature birth and other diseases were analyzed.Associations between interventions and basic illnesses were also analyzed.Results Of the 243 patients undergoing 275 procedures of FB,201 cases were boys(73.1％).The age of FB was (13.34 ± 9.76) days and the weight was (3.08 ± 0.68) kg.Forty-five cases were premature infants (16.4％).A total of 254 procedures were found to have congenital diseases (92.4％),and 177 cases of them had congenital heart diseases (CHD) (64.4％).Common indications for FB were dyspnea(140 cases,50.9％),tachypnea(82 cases,29.8％),and stridor(71 cases,25.8％).A total of 188 upper airway lesions were found and the most common findings were laryngomalacia(56 cases,20.4％) and vocal cord paralysis(bilateral/unilateral,50 cases,18.2％).A total of 315 lower airway lesions were found and the most common findings were airway mucosal inflammation (98 cases,35.6％),trachea and main bronchial stenosis (73 cases,26.5 ％).A total of 21 cases (7.6％) underwent supraglottoplasty during or after FB,while 17 cases (6.2％) underwent tracheal dilation and 10 cases (3.6％) underwent tracheotomy.Compared with non-CHD neonates,neonates with CHD were statistically significantly less likely to have congenital lesions statistically,such as laryngomalacia(15.8％ vs.28.6％,P =0.012),bilateral vocal cord lesions(6.2％ vs.21.4％,P =0.000) and congenital laryngeal dysplasia(0 vs.7.1％,P =0.001).The tracheotomy(0 vs.10.2％,P =0.000) and supraglottoplasty(2.3％ vs.17.3％,P =0.000) were more rare.Nevertheless,they were more likely to have secondary lesions such as the left main bronchial stenosis caused by extrinsic compression (23.7％ vs.1.0％,P =0.000),abnormal bronchial anatomy(9.6％ vs.2.0％,P =0.018),left vocal cord paralysis(9.0％ vs.1.0％,P =0.008) and airway mucosal inflammation(41.8％ vs.24.5％,P =0.004).The tracheostenosis and main broncial stenosis (37.3％ vs.7.1％,P =0.000) with long-term intubation(78.5％ vs.58.2％,P =0.000) were more common.There was no significant difference between term neonates and premature infants in the detection rate of respiratory tract diseases (P ＞ 0.05),tracheotomy (0 vs.4.3 ％,P =0.322),supraglottoplasty (13.3 ％ vs.6.5 ％,P =0.205) or long-term i ntubation (80.0％ vs.69.6％,P =0.157).Complications caused by procedure were rare and mild.Conclusions FB can detect whether the neonates with dyspnea,tachypnea and stridor have laryngomalacia,vocal cord paralysis,airway mucous edema,tracheal and main bronchial stenosis and other signs,and FB may play an important role in diagnosis,treatment and prognosis evaluation of neonatal respiratory diseases.

Bronchopulmonary dysplasia (BPD)characterized as the abnormal development of premature lung and trachea influenced by perinatal factors is the most common respiratory disease in very premature infants,and is also one of the diseases confirmedly related to poor quality of life.With the advancement of neonatal research and the im-provement in clinical care,more very premature infants with BPD survived to discharge.BPD is becoming the disease which both neonatologists and pediatricians should pay much attention to.The definition of BPD in recent years is in controversy.In this paper,the physiologic definition of BPD was comprehensively reviewed as a recently more accepted definition.

Objective To investigate the time for spontaneous closure of the ductus arteriosus and the trends of cardiac index(CI),peripheral vascular resistance index(SVRI)and blood pressure of neonates with different gesta-tional ages(GA). Methods A prospective observational study about 95 newborns,including 20 full - term and 75 pre-term infants were divided into 4 groups according to their GA as GA ﹤ 32 weeks(n = 27),32 weeks≤GA ﹤ 34 weeks (n = 25),34 weeks≤GA ﹤ 37 weeks(n = 23),GA≥37 weeks(n = 20). Patent ductus arteriosus was diagnosed by echocardiography(ECHO)at the time of 24 h,48 h,72 h,7 d and 14 d. CI,SVRI and blood pressure were measured with ultrasonic cardiac output monitor(USCOM). Results The cumulative spontaneous closure rate of arterious ductus of 32 weeks≤GA ﹤34 weeks,34 weeks≤GA ﹤37 weeks group and GA≥37 weeks group at 24 h after birth was 44. 0% , 61. 0% and 90. 0% respectively. However the rate was only 59. 1% at 72 h with the GA less than 32 weeks,of which 9 cases needed ibuprofen and 1 patient was conducted ductus arteriosus ligation. The differences in cumulative closure rate among 4 groups based on GA at 5 time points were statistically significant(χ2 =6. 756,4. 735,18. 890,11. 366,28. 159,all P ﹤0. 05). There was significant increase of systolic blood pressure,diastolic blood pressure and SVRI with ductus arte-riosus closure(t = -32. 561,-19. 913,-31. 791,4. 760,all P ﹤0. 000 1;t = - 25. 091,- 23. 400,- 21. 147,2. 240,all P ﹤0. 05;t = -10. 931,-9. 975,-9. 629,2. 350,all P ﹤ 0. 05). Whereas CI significantly decreased in groups of GA ﹤32 weeks,32 weeks ≤GA ﹤34 weeks,34 weeks ≤GA ﹤37 weeks(t =9. 224,6. 515,5. 996,all P ﹤ 0. 001)and no sig-nificant changes in GA≥37 weeks(t =0. 940,P ﹥0. 05). Conclusions With the increase of gestational age,the rate of naturally early arterial catheter closure is higher. Whereas there still are nearly one - third infants requiring medical or surgical intervention in the group of GA ﹤32 weeks. Ductus arteriosus has great influence on hemodynamics. For preterm, especially very low birth weight preterm,the use of ECHO and USCOM can early detect and treat patent ductus arteriosus.