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.

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 .

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 In order to optimize the Fontan circulation,a technique for direct total cavopulmonary connection was devised.To evaluate its surgical feasibility as well as surgical outcomes,our clinical experience was retrospectively reviewed.Methods From August 2005 to March 2012,23 consecutive patients underwent modified extracardiac Fontan operation with direct total cavopulmonary connection.Clinical profile of the patients,and procedural variables were examined and analyzed.Results All patients had adequately developed main and branch pulmonary arteries.Inferior caval vein was contralateral to the pulmonary trunk main pulmonary artery in 7 cases,ipsilateral in 8,and others in 8.There was 1 hospital death.The other 22 patients remained hemodynamically stable postoperatively.Prolonged effusions (n =13,62％) was a challenging problem.No obvious stenosis was found at the direct cavopulmonary anastomosis.Conclusion we are convinced that a direct total cavopulmonary connection is feasible in select subset of patients.This modified Fontan procedure retains the advantage of extracardiac connections together with the avoidance of prosthetic materials.

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 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 Retrospectively analyze primary experences of surgical correction of complicated aortic arch anomaly with autologous palmonary artery.Methods Between July 2010 and December 2012,13 cases of complicated aortic arch anomaly underwent reconstruction of aortic arch with autologous pulmonary artery.Classifications of aortic arch anomaly were interrupted aortic arch with ventricular septal defect in 4 patients,ventricular septal defect associated with coarctation in nine patients.There were 7 males and 6 females.Their age at surgeries ranged from 1 month to 16 years,and the body weight were from 3.5 kg to 52.0 kg with median weight of 12.6 kg.Cardiopulmonary bapass was estabished with dual arterial cannulations in patients with interrupted aortic arch.During cooling to deep hypothermia(rectal temperature was 18 ℃),intracardiac defects were totally corrected.Arch anomaly was reconstructed under deep hypothermia,including deep hypothermic cardiac arrest(DHCA) in 9 patients,deep hypothermia with regional perfusion in 4 patients.Anterior wall of pulmonary artery was excised in all of 13 patients.In 4 cases,the excised wall of anterior wall of pulmonary artery was sutured to form a conduit with different diameters according to the patient's bady surface area.Two ends of the conduit were anastomosed the aortic arch and desceding aorta respectively.In another 9 patients,aortic arch was augmented with tailored pulmonary artery patch in oval shape.The defect of pulmonary artery was repaired with autologous pericardial patch.Results There was only 1 death due tomutiple organ failure postoperatively.Another 12 patients survived without neurologic complications.Differences of arterial pressures between upper and lower extremeties were not monitored in all cases.During follow-up,routine echocardiogram showed satisfactory results with unobstructive blood flow at the aortic arch.Conclusion Autologous pulmonary artery can be used to relieved complicated aortic arch anomaly completely without any tension of anastomosis site and compression of left main bronchea postoperatively.More patients with long-term follow-up are necessary to draw an accurate conclusion of this technique.

Objective To evaluate the role of transesophageal echocardiography (TEE) in perioperative period of pediatric patients with congenital heart disease (CHD).Methods From July 2011 to December 2012,TEE was used in 393 pediatric patients(≤ 14 years) with congenital heart disease in perioperative period.We make a retrospective review with the clinical data of these cases.Results Operative schemes or therapeutic schedules of 60 patients(15.3％) were altered according to TEE.By preoperative TEE,the diagnosis of transthoracic echocardiography (TTE) of 4 cases(1.0％) were amended,4 cases (1.0％) were complemented,and 4 cases (1.0％) got the auxiliary diagnosis,among which operative schemes of 11 cases (2.8％)were altered.During the operate,29 cases(7.4％) found residual problems,11 cases(2.8％) got the auxiliary diagnosis.By postoperative TEE,7 cases(1.8％) found residual problems,1 case(0.3％) got the auxiliary diagnosis.Complications occurred in 9 cases(2.3％) of the 393 patients.Oral and pharyngeal mucous membrane bleeding occurred in 7 cases (1.8％),inadvertent tracheal extubation in 2 cases(0.5％).Conclusion TEE plays an important role in confirming preoperative diagnoses,formulating surgical plans,evaluating immediate operative results,identifying patients with residual defects and guiding the therapeutic schedule in perioperative period of pediatric patients with congenital heart disease.

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.