Objective Norwood StageⅠis the standard procedure to cope with hypoplastic left heart syndrome(HLHS), which continues to be the most challenging congenital heart disease .The aim of this study is to retrospectively analyse the perio-perative management of Norwood StageⅠ.Methods Between June 2010 and August 2014, totally 5 small infants with HLHS underwent the standard Norwood StageⅠprocedure.They were all boys.Age at surgeries ranged from 29 to 75 days, and weight from 2.57-3.50 kg with median of 3.13 kg.Case 1, 2 and 3 received standard medical regimen after accessing NICU, which included intravenous prostaglandin E1 and mechanical ventilation.The 3 infants underwent emergent operations because of unstable hemodynamics.Case 4 and 5 received no medical intervention before the urgent surgeries.All 5 cases underwent the standard Norwood StageⅠprocedure under deep hypothermic circulatory arrest, including 4 cases of modified Blalock-Taus-sig shunt( MBTS) and 1 case of Sano shunt.Results The case with Sano shunt died from severe hypoxemia and persistent aci-dosis 32 hours after the operation, another case died from low cardiac output syndrome after cardiopulmonary bypass.The first case underwent bidirectional Glenn procedure 12 months after Norwood Stage I, the postoperative heart function was NYHA I and the oxygen saturation was 0.90-0.95 in room air, but he died from accidental brain injury 3 months after stage Ⅱ.The second case was followed up 3 months after stage I procedure with NYHA I and oxygen saturation of 0.78-0.83 in room air, and lost the follow-up after.The fifth case was followed up 3 months after stageⅠprocedure with NYHAⅠ, confluent MBTS and oxygen saturation of 0.84, the patient is being followed up and waiting for further evaluation for stageⅡprocedures.Con-clusion The standard Norwood StageⅠprocedure is a complex procedure, which demands multidisplinary cooperation, to pal-liatively correct HLHS .Sharing expericences on perioperative managements of Norwood Stage I between heart centers in China will be helpful to decrease the mortality and morbidity in relatively short period .

Objective The aim of this study is to retrospectively analyze perioperative managements of very-low-birth-weight(VLBW) preterms with hemodynamic significant patent ductus arteriosus (hsPDA).Methods Between January 2006 and December 2011,totally 22 VLBW preterms with hsPDA underwent surgical ductal ligation.There were 12 boys and 10 girls.The median gestatianal age was 29 weeks (24 + 5-32 +6 weeks).The birth weight was (1103 ± 228) g(640-1440 g).The Apgar score was 6.1 ± 2.2 at 1 minute,8.6 ± 1.2 at 5 minutes.The average ductal size was (3.79 ± 1.01)mm (2.0-5.9 mm)、(2.69 ± 0.84) mm/kg(1.23-4.23 mm/kg),left atrial diameter to aortic root ratio(LA:AO) was 1.69 ± 0.41.The median weight at surgery was 1500 g(640-2100 g),average (1512 ±539) g.The median age at surgery was 24 days (11-167 days).Results 1 case death because of anesthetic accident.The average hospitalization days were (67.1 ± 36.1) days.The days of ventilation treatment after surgery were 2-44 days,15 cases (68.2％) weaned from mechanical ventilation within seven days after surgeries.The complications includes pulmonary hemorrhage (18.2％),necrotizing enterocolitis (13.6％),septicemia(22.7％),broncho-pulmonary dysplasia (63.7％),brain injury(18.1％),retinopathy of prematurity (31.8％),pneumonia (86.4％) and metabolic acidosis (45.5 ％).Conclusion For VLBW preterms with hsPDA,early diagnosis and early interfere are key points.Surgical PDA ligation is a promising option to avoid severe complications when medical treatments are ineffective.

AIM: To evaluate the pulmonary function in patients with type 2 diabetes mellitus in order to identify whether the lung is a target organ of chronic pathologic changes in diabetes mellitus. METHODS: Pulmonary ventilation function and diffusion capacity were studied in 107 patients with type 2 diabetes mellitus and 61 healthy subjects matched for age and sex. Glycosylated hemoglobin (HbA1c), urine albumin excretion rate (AER), fundus examination and nerve conduction velocity were included as parameters of glycemic control and diabetic microangiopathies. RESULTS: Pulmonary ventilation function was similar in type 2 diabetic group and the control. Compared with the control, carbon monoxide diffusion capacity (DLCO) and DLCO corrected by alveolar volume (DLCO/VA) were significantly lower in type 2 diabetic group (P<0.05). DLCO and DLCO/VA were inversely correlated with microangiopathy score (r: -0.291, -0.324, respectively, P<0.01). Furthermore, DLCO/VA was negatively correlated with age and duration of diabetes mellitus (r: -0.269, -0.236, respectively, P<0.05). CONCLUSIONS: Pulmonary ventilation function is normal in patients with type 2 diabetes mellitus, but their diffusion capacity is impaired. It suggests that the lung may also be the target organ of the chronic pathologic changes of diabetes mellitus.

Objective The aim of this study is to retrospectively analyze surgical management of absent pulmonary valve syndrome(APVS).Methods Between January 2005 and January 2012,totally 11 children with APVS underwent primary surgical correction.There were 7 boys and 4 girls.Age at surgeries range from 1 to 5 years,and wcight from 10.2-17.5 kg,with average (12.3 ± 3.4) kg.Surgical procedures include VSD repair,pulmonary arteries reconstruction and RVOT reconstruction with monocusp valve.5 cases chose Lecompte maneuver as an option to release compression to bronchus,5 cases underwent fibroscopy inspection and airway secretion suction,and 4 cases adapted deep hypothermic circulatory arrest (DHCA)during correction procedures.All survivors are routinely followed-up with echocardiogram.Results All 11 cases survived,2 of them suffered from frequent lungs infections during the first year post surgery.No case exists bronchus or pulmonary arteries compression.Conclusion APVS is a rare congenital heart defect,which may challenge perioperative managements and operations.Ideal surgical correctiou includes RVOT reconstruction,decompression of bilateral bronchus,and airway inspection with fibroscopy.However,compression of intrapulmonary bronchi by abnormally branching pulmonary arteries may expose patients to a relative long time of medication therapy after surgical correction.A large number of patients with long-term follow-up are needed to draw definitive conclusions on this strategy' s effectiveness.

Objective Konno-Rastan procedure is one option to cope with complex multilevel left ventricular outflow tract obstruction (LVOTO),which continues to pose a serious challenge to cardiac surgeons.The aim of this study is to retrospectively analyse indications for Konno-Rastan procedure,and to review the safeguards and pitfalls.Methods Between January 1996 and August 2012,totally 13 children with multilevel LVOTO underwent Konno-Rastan procedure.There were 8 boys and 5 girls.Age at surgeries ranged from 5 to 13 years,and weight from 12 to 51 kg with median of 21 kg.The pathology of this cohort includes:8 cases of congenital aortic valvular stenosis,3 cases of congenital aortic valvular stenosis combined with supravalvular stenosis,1 case of congenital aortic stenosis combined with VSD,coarctation and RVOT obstruction,1 case of aortic stenosis s/p percutaneous balloon aortic valvuloplasty.All patients have secondary diffuse tunnel LVOTO.Diameter of aortic ring ranges from 12.0 to 16.4 mm,and pressure gradient across the stenotic region ranges from 90-151 mm Hg.8 cases were implanted with St.Jude AG19 while 5 cases implanted with St.Jude AG17.Results All 13 cases survived.The 4th patient was implanted permanent epicardial pacemaker for transient Ⅲ AVB.The 4th and 5th patients were found residual ventricular septal repture at the nadir of ventricular incision,one underwent redo procedure while another is being followed up.All cases take cumadine to sustain INR at 1.8-2.5.No death emerges during follow-up period.The motality is 0％,the incidence rate of B is 7.7％,residual VSD 15.4％ and endocarditis 7.7％.Conclusion Konno-Rastan procedure is a promising techi.to relieve LVOTO.However,this complex procedure may lead to several fetal complications.Success of the surgery demands perfect operations.

Objective To retrospectively analyse the anaesthetic management of Norwood Stage Ⅰ.Methods Between June 2010 and August 2014, totally 5 small infants with HLHS underwent the standard Norwood Stage I procedure .They were all boys.Age at surgeries ranged from 29 to 75 days with median 36 days, and weight from 2.57 -3.50 kg with median of 3.13kg.The first three cases were received intravenous prostaglandin E1 before they were sent to the operation theatre and were under mechanical ventilation .They were received emergent operations because of unstable hemodynamic situation .The other two cases were relatively stable without mechanical ventilation and were received restrict surgery .All 5 cases received the stand-ard Norwood Stage Ⅰprocedure under intravenous-inhalation balance-general anaesthesia with cardiopulmonary bypass.The technique of deep hypothermia and circulatory arrest were used in all five cases .Results The fourth case died from low cardi-ac output syndrome after cardiopulmonary bypass .The other 4 cases were transferred to the paediatric intensive care unit after withdrawal from bypass.One of the four cases died after 32 h after surgery.Conclusion The standard Norwood Stage Ⅰ pro-cedure is aquite complex procedure, which demands multidisplinary cooperation, to palliatively correct HLHS.We retrospect the experiences of the anesthetic management in our centre and hope it will be helpful to decrease the mortality and morbidity in relatively short period.

Objective To evaluate the diagnostic value of multi-slice spiral CT (MSCT) in classifying coronary arteries of complete transposition of great arteries (D-TGA). Methods The clinical and imaging data of 367 patients with D-TGA who had undergone MSCT examination from March 2005 to June 2015 were retrospectively analyzed. The origin and course of the coronary arteries of the patients were classified according to the Marie Lannelongue classification. There were four patterns of courses: normal, looping, intramural and miscellaneous. And the four patterns were subdivided into eleven subgroups. The anatomic classification of coronary arteries in D?TGA were recorded, and the ratio of descriptive statistics was used according to categorical variable data. Results All the origin and course of the coronary arteries could be clearly displayed on MSCT. Of 367 patients with D-TGA, 209 cases (56.95%) were normal course (typeⅠ), 138 cases (37.60%) were looping course (typeⅡ), 16 cases (4.36%) were intramural course (typeⅢ), and 4 cases (1.09%) were miscellaneous course (typeⅣ). In looping course, the posterior looping (typeⅡA), anterior looping (typeⅡB) and double looping (typeⅡC) were found in 63 cases (17.17%), 30 cases (8.17%) and 45 cases (12.26%), respectively. The ratios of the anatomic classification of looping courses wereⅡA-1 44(11.99%),ⅡA-2 19(5.18%),ⅡB-1 12(3.27%),ⅡB-2 8(2.18%),ⅡB-3 10(2.72%),ⅡC-1 25 (6.81%),ⅡC-2 17(4.63%),ⅡC-3 3(0.82%). Conclusions MSCT is an effective technique to visualize and classify the coronary arteries in patients with D-TGA. And it is helpful for successful transfer of the coronary arteries and reducing the rate of coronary events after operation.

Objective To investigate the relationship between nutritional status and quality of life ( QOL) in patients with nasopharyngeal carcinoma undergoing concurrent chemoradiotherapy, and to identify the optimal indices of body composition for evaluation of malnutrition. Methods Based on the European Society Parenteral and Enteral Nutrition ( ESPEN) consensus on the diagnosis of malnutrition and patient?generated subjective global assessment (PG?SGA), a prospective study was performed in 48 patients with nasopharyngeal carcinoma who received concurrent chemoradiotherapy in our hospital from 2014 to 2015. Changes in body composition and nutritional status were evaluated in those patients. The relationship between nutritional status and QOL was studied. The predictive factors for nutritional status in patients with nasopharyngeal carcinoma were explored. The correlation between datasets was evaluated by the Pearson correlation coefficient. The influencing factors for nutritional status were analyzed by Logistic regression. Results During concurrent chemoradiotherapy, some indices of body composition, including weight, body mass index ( BMI) , fat mass index ( FMI) , fat?free mass index ( FFMI) , body cell mass ( BCM) , skeletal muscle mass ( SM) , and phase angle ( PA) had different degrees of reduction ( P=0?00) , while the PG?SGA score gradually increased. The incidence of malnutrition in patients with nasopharyngeal carcinoma during radiotherapy was 2?1%?39?6% based on 2015 ESPEN consensus on the diagnosis of malnutrition and 12?5%?41?7% based on PG?SGA. These two methods showed good agreement at the fourth and sixth weeks of radiotherapy ( Kappa=0?911;Kappa=0?957) . The changes in QOL score were correlated with changes in FFMI and weight during radiotherapy ( r= 0?805, P= 0?00;r= 0?777, P= 0?00 ) . Logistic regression indicated that age, FMI, and FFMI were influencing factors for nutritional status ( P= 0?035, 0?013, 0?043) . Conclusions Patients with nasopharyngeal carcinoma have substantial nutritional deterioration during chemoradiotherapy. The nutritional status is closely associated with QOL. A prediction model of nutritional status can provide a comprehensive and accurate judgment of nutritional status in patients.

Objective The results of repair for TOF with anomalous coronary artery(ACA) were studied to determine the incidence of coronary anomalies and evaluate surgical strategy choicesas well as postoperative outcomes.Methods From January 2008 to August 2014,1142 consecutive patients underwent repair of TOF including 44 patients with TOF and ACA:single coronary artery in 15,dual anterior descending coronary in 15,single left anterior descending coronary arising from the rightcoronary artery in 3 and the other ACA in 5.The median age was 5.7 years (range,1 month-27 years),and the median weight was 16.0 kg(range,4.5-51.0 kg).Surgical procedure was selected according to the extent of right ventricular outflow tract (RVOT) obstruction and distribution of the ACA.Results There was one operative death.No deaths during the follow-up period in the other 37 patients.Single patch techniquewasperformed in 15.RVOT residual obstruction detected in 7 who without transannular patch,and one need reoperation;Two patch technique was performed in 6,and 3 of them required an additional RV-PA(pulmonary artery) tube because of RVOT residual obstruction during the operation;Double oullet technique was in 6.No tube stenosis occurred in follow-up period time;PA translocation technique was in 11.The right PA stenosis was detected in 4;ACA was ligated and divided in 3,then RVOT reconstruction was performed.Conclusion The combination of ACA is not a contraindication to primary repair of TOF.But there are many anatomiacal variations of ACA,and the accuracy of preoperative diagnosis is low.So proper selection of surgical approach should be individualized based on the careful intraoperative identification of the distribution of the ACA as well as the location and degree of the RVOT obstruction.