22q11.2 deletion syndrome (22q11.2DS) is the second leading chromosomal cause of congenital heart disease (CHD) after Down syndrome.In the past few decades, great progress has been made in describing the anatomical types, potential heredity, and pathogenesis of CHD complicated with 22q11.2DS in terms of improving the diagnosis and treatment of these diseases.However, cardiac malformation is still the leading cause of death in 22q11.2 DS.Further studies are needed to elucidate the genetic mechanism behind high phenotypic variability of CHD and to explore the multidisciplinary treatment for the improvement of the prognosis.22q11.2 gene detection in patients suffering from CHD may provide useful insights into early diagnosis and multidisciplinary treatment.

Objective Approach to safe and effective lung protection strategies on ventricular septal defect infants during perioperation.Methods There were 4 groups as blank control group,ambroxol group,methylprednisolone group and ulinastatin group.We tried to find a safe and effective stratege on lung protection by comparing each other on the respiratory function indexes and inflammation biomarkers.Results Postoperation of VSD repair,respiratory function indexes such as Ppeak,oxygenation index inspiratory resistance and mechanical ventilation time were significantly different (P ＜ 0.05) among the 4 groups.The inflammation biomarkers such as WBC,CRP and PCT had no differences the first day(P ＞0.05),and were statistically significant the third day (P ＜ 0.05).Conclusions Ambroxol,methylprednisolone and ulinastatin all are effective drugs for lung protection.Considering about the clinical curative effect and side effects,ambroxol is more safe and effective relatively.

Objective To explore the clinical diagnosis of tetralogy of Fallot( TOF) children with concurrent DiGeorge syndrome ( DGS ) . Methods Retrospective analyses were conducted for the clinical characteristics of 715 TOF children with concurrent DGS at Henan Provincial People′s Hospital and the Third Affiliated Hospital of Zheng-zhou University from January of 2008 to October of 2014. Among them,there were 78 definite cases of thymic aplasia (DGS group),including 45 boys and 33 girls with an age range of(9. 12±4. 35) months and a body mass range of (7. 28±2. 34) kg. And the remainder was designated as non-DGS group(NDGS group),including 387 boys and 250 girls with an age range of(8. 21±5. 61) months and a body mass range of(8. 19±3. 47) kg. In DGS group,genetic screening uncovered 10 cases of chromosome 22q11. 2 gene deletion. And based upon this result,DGS group was fur-ther divided into genetic and clinical diagnosis subgroups. The genetic diagnosis group had 10 cases,including 6 boys and 4 girls with an age range of(8. 12±4. 15) months and a body mass range of(6. 28±2. 74) kg,the clinical diagnosis group had 68 cases,including 39 boys and 29 girls with an age range of(8. 19±4. 37) months and a body mass range of (7. 05±2. 39) kg. Results No statistical difference existed in age,body mass or preoperative developmental status of pulmonary vasculature between DGS group and NDGS group(P>0. 05). And the preoperative incidence of recurrent pneumonia was obviously higher in DGS group than that in NDGS group(P<0. 001). The probability of concurrent hy-pocalcemia and facial malformation was higher in DGS group than that in NDGS group. However,there was no statistical difference(P>0. 05). And an inter-group comparison of T lymphocyte immunity defect had no statistical difference(P>0. 05). The duration of on-machine and intensive care unit stay was markedly longer in DGS group than that in NDGS group(P<0. 001). And the probability of concurrent hypocalcemia and facial malformation was higher in genetic diag-nosis subgroup than that in clinical diagnosis subgroup. However,there was no statistical difference(P>0. 05). And an inter-group comparison of T lymphocyte immunity defect had no statistical difference ( P>0. 05 ) . Conclusions With diverse clinical manifestations,DGS patients may have non-specific findings of cellular immunity defect,hypocalcemia or facial malformation. TOF children with concurrent thymic aplasia should raise an alert so that effective interventions may be adopted to boost their long-term quality of life.

Objective To establish a standardized scoring system after tetralogy of Fallot (TOF) correction and explore its clinical application values.Methods During the period of Jan.2006 and Apr.2014 at the Third Affiliated Hospital,Zhengzhou University and Henan Provincial People's Hospital,a total of 624 infants undergoing stage Ⅰ anatomic correction were selected and their common clinical parameters such as liver size,systolic blood pressure,urinary volume,oxygenation index and occurrences of complications were recorded and scored.The cases were divided into low-risk,medium-risk and high-risk groups according to scores.The relevant endpoint parameters,including mortality or survival,on-pump time,intensive care unit(ICU) duration,hospitalization and medical expenses were recorded to analyze their relationship with postoperative scores.Results The data indicated that the short-term mortality after repair of tetralogy of Fallot was 5 ％.Moreover,there was no statistical difference (P =0.309) in the mortality between the lowrisk group (total score ＜ 3 and mortality of 0) and medium-risk group,and the mortality of the medium-risk group was statistically different(P ＜ 0.001) from that of the high-risk group (total score ＞ 7 and mortality of ＞ 80％).The postoperative standby time,ICU stay,total hospital stays and cost increased with the rise of the score(P ＜ 0.001),and there were no statistical differences in hospital stays(P =0.469) and cost(P =0.365) between the medium-risk group and the high-risk group.Conclusions Based upon common clinical parameters,a standardized scoring system has been successfully established for patients after TOF correction,which plays an important role in gauging the disease progression and predicating the immediate postoperative outcomes of TOF.

Objective:To investigate the changes and their clinical values of renal oxygen saturation(RrSO 2) and abdominal local oxygen saturation(A-rSO 2) in infants who underwent cardiac surgery. Methods:Thirty children with atrial septal defect or ventricular septal defect underwent cardiopulmonary bypass (CPB) in Henan People′s Hospital from April to August 2019 were randomly selected.There were 15 males and 15 females, aged 2-13 months, weighted 4.5-10.0 kg and American Society of Anesthesiologists(ASA) grade Ⅰ-Ⅲ.The probe of near-infrared spectroscopy(NIRS)was fixed at the body surface of the right kidney and 1 cm below the umbilicus.RrSO 2 and A-rSO 2 were continuously monitored during operation.The changes of parameters, including RrSO 2, A-rSO 2, mean arte-rial pressure(MAP), and nose temperature were recorded after anesthesia induction (T 0), cardiopulmonary bypass (T 1), 5 minutes after aortic blockade (T 2), the lowest temperature (T 3), 5 minutes after aortic opening (T 4), and 5 minutes after stopping cardiopulmonary bypass (T 5). CPB time, ascending aorta occlusion time and operation time were recorded as well.Meanwhile, perioperative complications such as acute renal injury (AKI) and gastrointestinal dysfunction were recorded.Relevant information, including the time of first eating after operation was recorded. Results:Totally, 30 children were enrolled in this study.The basic values of RrSO 2 and A-rSO 2 were (70.00±7.50)% and (70.70±11.29)%, respectively.Compared with T 0, the RrSO 2 and A-rSO 2 of patients decreased at T 1, gradually increased at T 2, T 3 and T 4, and returned to T 0 at T 5.There was no significant difference in RrSO 2 and A-rSO 2 at each observation point. Pearson correlation analysis displayed that there was a positive correlation between A-rSO 2 and RrSO 2 ( r=0.806, P<0.01). RrSO 2 and A-rSO 2 were positively correlated with MAP ( r=0.565, 0.605, all P<0.05), and negatively correlated with the nasopharynx temperature ( r=-0.365, -0.331; all P<0.05). Among them, 3 children(10%) suffered from AKI after operation.Compared with T 0, RrSO 2 values at T 1, T 2, T 3 and T 4 decreased significantly ( P<0.05). Postoperative gastrointestinal hysteresis occurred in 6 children(20%). The A-rSO 2 value in T 0-T 5 of children with gastrointestinal hysteresis was significantly lower than that of children without gastrointestinal hysteresis ( P<0.05). Conclusions:As a new noninvasive monitoring method of renal and intestinal function, NIRS has certain clinical guiding value in perioperative period of infantile congenital heart disease.

Objective:To summarize the clinical experience of changing the membranous pulmonary system during extracorporeal membrane oxygenation(ECMO) in infants after congenital heart disease opration with cardiopulmonary bypass.Methods:From January to September in 2019, 6 cases of congenital heart disease with cardio-pulmonary bypass in our hospital were analyzed retrospectively, whose membrane obstruction occurred during ECMO treatment and replaced successfully.The hemodynamics and blood gas before and after replacement of ECMO system were observed, and the experience was summarized.Results:Six patients(3 males and 3 females), aging from 1 to 3 months and weighing from 3.0 to 4.9 kg, were received VA-ECMO adjuvant therapy.The ECMO system replacement process was smooth and took 175-209 s. The hemodynamic of the children was stable.The ECMO support time was 134-249 h. After the improvement of cardiac systolic function, all children were successfully withdrawn and survived.Conclusion:The improved method of liquid replacement in ECMO system can make full use of the blood components in the original system and avoid the loss of blood tangible components.According to the plan of rapid replacement, the risk of replacement will not be increased.

Objective To summarize the preliminary experience and early -stage follow -up results of simple transesophageal echocardiography(TEE)to guide right jugular internal vein for closure of atrial septal defect (ASD), and to investigate its feasibility.Methods A total of 24 ASD patients(12 male,12 female)treated by transcatheter ASD closure under solely guidance of TEE were summarized in Children Heart Center,People′s Hospital of Zhengzhou University from February 2015 to February 2016.Mean age,body weight and average diameter of the 24 cases of ASD were 5 -18 (12.4 ±5.6)years old,16 -60 (34.1 ±9.6)kg,5 -20 (11.0 ±2.5)mm,respectively.All patients were treated by right jugular internal vein transcatheter closure under solely guidance of TEE.The efficiency of imme-diate post -operation under TEE was estimated,and follow -ups were done at 24 hours,1 month,3,6 and 12 months after the procedures by transthoracic echocardiography,X -ray examination and electrocardiogram.Results A total of 23 patients were treated successfully under solely guidance of TEE.The diameter of devices was (14.5 ±3.6)mm.The procedural time was(20.5 ±7.2)min.One patient who had 2 ASD was treated by traditional thoracic surgery,because the small ASD had 5 mm residual shunt after the procedures.There were 3 cases with trivial residual shunt after the pro-cedures immediately.All patients could move at once after operation.The average follow -up time was(5.6 ±1.2) months.No patients suffered from residual shunt,occluder malposition,peripheral vascular injury,pericardial effusion or cardiac perforation during the follow -up.Conclusions For some selected patients,simple TEE guiding right jugular in-ternal vein for closure of ASD is applicable.The method not only prevents injury from radiation,but also shortens the in -bed time and avoid the lower limbs braking,with good early -stage follow -up results are desirable.

Objective: To summarize the clinical experience and short-term outcome of minimally invasive occlusion in patients with peri-membranous ventricular septal defect (PmVSD) via right subaxillary route under trans-esophageal echocardiography (TEE) guidance.
Methods: A total of 122 PmVSD patients treated in our hospital from 2014-01 to 2015-07 were summarized. There were 54 male and 68 female with the mean age of (2.7±2.2, 0.5-9.7) years, mean body weight of (13.9±6.0, 6.1-38.0) kg and mean PmVSD diameter of (3.8±0.8, 2.5-7.0) mm. The patients were taken left lateral position, a (2-3) cm incision was performed along right mid-axillary line between the 3rd rib and 4th rib, the thoracic entrance was at 4th inter-costal space. A purse-string suture was conducted on right atrial surface, a special hollow probe was inserted into right atrium and crossed tricuspid into right ventricle under TEE guidance; the probe was adjusted to the point or crossed VSD into left ventricle followed by guide wire insertion to establish a deliver pathway, and ifnally, occlusion device was regularly deployed to close the defect. Post-operative ECG, TEE and chest X-ray were conducted for followed-up study.
Results: There were 119/122 (98.4%) patients occluded successfully and 3 failed patients were converted to cardiopulmonary bypass surgery at the original incision. The average size of occluder was (4.9±1.1, 4-10) mm and all devices were concentric. The patients were followed up at the mean of (8.3±5.0, 1.0-19.8) months, during that period, 12/119 (10.1%) had new mild tricuspid regurgitation, 16 (13.4%) suffered from incomplete right bundle branch block, 4 (3.4%) had small residual shunt and 2 of them were self-closed at 1 and 3 months after operation respectively. There were no complete atrio-ventricular block, no new aortic valve regurgitation and no device dislocation.
Conclusion: Minimally invasive occlusion of PmVSD via right subaxillary route under TEE guidance was a safe, effective, feasible and better cosmetic method for treating relevant patients; while its long-term outcome should be further observed.

Objective: To explore the blood-saving effect of autologous platelet-rich plasma (PRP) back-transfusion in patients with Stanford type A aortic dissection surgery. Methods: A total of 59 consecutive patients who received Stanford type A aortic dissection surgery in our hospital from 2013-01 to 2015-10 were studied. The patients were at the age of (50±6) years with mean body weighting at (80±12) kg and were randomly divided into 2 groups: Traditional (T) group,n=31 and Autologous PRP back-transfusion (P) group,n=28. Blood levels of Hb, platelet counts, PT, APTT were measured at pre-induction of anesthesia (T1), before CPB (T2), prior ifnishing of CPB (T3) and at 1 h (T4), 24 h (T5), 48 h (T6) after the operation. The in-operative, 48 h post-operative volumes of allogeneic blood transfusion and the volume of chest tube drainage at 48h after operation were recorded; the complication occurrence at peri-operative period was recorded. Results: In P group, whole blood processing volume was (1269±197) ml, PRP volume was (753±78) ml, PRP separation time was (35±9) min and the separated platelets were about (22±3)% of total platelet counts. Compared with T group, P group had decreased Hb at T2 (131.0±15.0) g/L vs (101.0±10.0) g/L, decreased platelet counts at T3 (115.0±51.0)×109 /L vs (83.0±23.0)×109/L, while increased platelet counts at T4 (103.0±25.0)×109/L vs (151.0±27.0)×109/L, T5 (105.0±25.0)×109 /L vs (147.0±39.0)×109/L and T6 (101.0±26.0) ×109/L vs (149.0±35.0)×109/L, allP<0.05; P group presented reduced PT at T4 (17.6±2.1) s vs (14.1±1.1) s and T5 (17.6±2.7) s vs (13.5±0.8) s, allP<0.05. The in-operative transfusions of platelet, plasma, cold precipitation and post-operative transfusions of red blood cells, platelets, plasma, cold precipitation and the volume of chest tube drainage at 48h after operation were less in P group,P<0.05. Compared with T group, P group had the lower rates of acute post-operative lung injury (32.1% vs 19.4%), shorter mechanical ventilation time (69.1±5.9) h vs (43.1±1.5) h and ICU staying time (8.1±2.8) d vs (5.3±1.1) d, allP<0.05. Conclusion: Autologous PRP back-transfusion could reduce the post-operative bleeding and allogeneic blood transfusion for Stanford A aortic dissection surgery, it has obvious blood-saving effect.

Objective To investigate the feasibility and safety of surgical device closure of doubly committed sub-arterial ventricular septal defect via left sub-axillary.Methods A total of 45 patients diagnosed as doubly committed sub-arterial ventricular septal defect (dcVSD) with transthoracic echocardiography (TTE) and transesophageal echocardiography(TEE) were enrolled from June 2014 to August 2016 in Henan Children Heart Center,Henan Provincial People's Hospital.There were 39 males and 6 females,with the mean age of (2.2 ±2.1) years old(0.5-8.0 years),the body weight (13.8 ± 7.1) kg(7.0-34.1 kg),the defect size (4.5 ± 1.0) mm (3.0-8.0 mm).After general anesthesia,the patients were in supine and evaluated by TEE which indicated whether they were fit to closure.Then,they were turned to the right lateral position while this technique was determined.A vertical incision of 2-3 cm was made between the third and the fifth intercostal space and invasion in thoracic space via fourth intercostal space.Puncture was done at the anterior surface of right ventricular outlet tract to build a delivery tract.The occluder was released and the VSD was occluded under transesophageal echocardiography guidance.Results Forty-one patients had a successful surgical dcVSD closure with asymmetric occluders sized (6.0 ± 1.5) mm(4-10 mm).Among 4 failure cases,2 cases (4.4％) were switched to open-heart surgical repair,1 case (2.2％) due to device related aortic regurgitation,the rest 1 case (2.2％) experienced a dislocation of occluder into pulmonary artery and was converted to surgical repair after retrieve of occluder.Trivial residual shunt was detected in 2 cases (4.4％) postoperatively,a spontaneous closure was observed by 1 month follow-up and 3 months follow-ups,respectively.All the patients were discharged 5 to 8 days after the operation.With a follow-up of (10.4 ±5.0) months [3-24 months],there were no complications such as pericardial effusion,displacement of device,atrioventricular block or new valvular dysfunction.Conclusions Minimally invasive device closure of doubly committed sub-arterial ventricular septal defect via left sub-axillary is a feasible and safe treatment for closure of dcVSD.This technique has advantages of minor wound,less exudation,covert incision,however,long term follow-up is necessary.