Objective To evaluate the risk factors,diagnostic index of the restrictive right ventricle physiology(rRV) and the impact of the mid-term outcome of the patients.Methods Eighty patients(30 in rRV group and 50 in non-rRV group) undergoing TOF repair admitted in our department from Oct 2011 to May 2012 were studied.Perioperative clinical data were collected and echo data were recorded after operation.Mixed linear regression for repeated measures was used to compare the variables and analyze the correlations.Results Patients in rRV group were younger with longer cardiopulmonary bypass(CPB) time,aortic cross clamp time,ventilation time,intensive care unit and hospital stay compared with those in non-rRV group(P<0.01,respectively).The younger patients with longer CPB time had high risk of rRV by logistic regression analysis.Within 7 days post operation,the increase of saturation of venous oxygenation and decrease of oxygen extraction ration were slower in rRV group than those in non-rRV group(P<0.05,respectively).Lactate decreased in both groups,but was higher in rRV group throughout the 7 days(P=0.03).NT-proBNP was higher in rRV group throughout the 7 days than that in non-rRV group.NT-proBNP≥4750pg/ml often indicated the patients in the state of rRV.CRP slightly increased in 1-2 days post operation,and decreased thereafter,and the decrease was slower in rRV group(P=0.08).With regard to the mid-term outcome,there was no significant differences in the incidences of the obstruction of the right ventricle outflow and main pulmonary artery,the stenosis of the branch of pulmonary artery and the degree of the pulmonary valve regurgitation.Conclusion rRV is associated with significantly higher levels of NT-proBNP and CRP.The incidence of rRV correlates with age on operation and positively correlates with CPB time.NT-proBNP would be regarded as an indicator of the incidence of rRV.The study indicated the rRV would have impact on the early outcome of the patients but there was no significant effect on mid-term outcome.

Objective:To compare the outcomes of combined lingual mucosal graft with buccal mucosal graft urethroplasty and combined lingual mucosal graft with ADM (acellular dermal matrix) urethroplasty for the treatment of repair failed hypospadias.Methods:From February 2017 to February 2019, 26 patients with failed hypospadias repairs were treated with combined lingual mucosal graft with buccal mucosal graft urethroplasty (14 cases in Group A), and combined lingual mucosal graft with ADM urethroplasty (12 cases in Group B). The mean age of Group A was (29.5±1.2) years (range 18.0-41.0 years), and (26.5±0.8) years (range 20.0-38.0 years) in Group B. The previous operation times was mean (3.6±0.7)(range 2-5 times) and (4.6±0.8)(range 3-5 times) in Group A and Group B respectively. Operation method: All patients were nasally intubated, the remaining curvature was corrected, the fibrous tissue or scar was removed, and the defected urethra was measured. In Group A, the lingual mucosa was spread and fixed to the corpora cavernosa over the midline as the urethral plate, the buccal mucosa was covered the lingual mucosa as ventral urethra, both the mucosa lateral edges was sutured. In Group B, the lingual mucosa was harvested and fixed to the corpora cavernosa the same as in Group A, the ADM was made appropriate length and width, covered and sutured with the lingual mucosa. The lingual mucosa was harvested mean (5.0±0.2)cm(range 4-6cm)long, mean (1.2±0.2)cm (range 1.0-1.5cm)wide and mean (5.0±0.2)cm(range 5-6cm)long, mean (1.2±0.2)cm (range 1.0-1.5cm)wide in Group A and Group B respectively( P<0.05). In Group A, the buccal mucosa was harvested mean (4.1±0.2)cm(range 3.5-5.5cm)long, mean (1.2±0.2)cm wide. Criteria for successful repair of hypospadias were set as: ①The appearance of the penis is nearly normal; ②The penis curvature was corrected; ③Urethra orifice in normal position; ④Urine flow line is normal. The outcomes of the two groups were analyzed and compared, statistical analysis was done using SPSS 18.0 software. Results:The mean follow-up time was (16.3±1.6)(8-24) months. The age, number of preoperative surgeries, number of previous oral mucous membranes, and length of urethral defects were no statistically significant differences between the two groups in A and B( P>0.05). The length of oral mucosa was harvested during the operation between group A and Group B were statistically significant differences( P<0.05). The incidence of oral complications in group A and B: Oral pain 7/14, 1/12; The feeling of tension in mouth 8/14, 1/12; The numbness in the oral 8/14, 1/12, A and Group B were statistically significant differences( P<0.05). The incidence of urethral complications in group A and Group B: the urethra fistula 1/14, 4/12; the urethral stricture 2/14, 6/12, there were statistically significant differences between the two groups ( P<0.05). Penile curvature 2/14, 1/12, ( P>0.05). The success rate was 12/14 and 5/12 in Group A and B respectively, with statistical difference( P<0.05). Conclusions:Combined lingual mucosal graft with buccal mucosal graft urethroplasty could be a good choice for repeated failed hypospadias repairs. Combined lingual mucosal graft with ADM urethroplasty has many complications and less success, should be performed in caution.

Objective    To investigate somatic growth of children undergoing stageⅡ Fontan procedure. Methods    Clinical data of 152 children who underwent stageⅡ Fontan procedure in Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine between January 2012 and December 2015 were reviewed. There were 84 males and 68 females at age of 4-16 years. The weight and height of those children in each operative stage were assessed, and WHO Z-score was obtained to describe the pattern of growth of the children. Results    Mean weight for age Z-score (WAZ) and height for age Z-score (HAZ) were –0.73 and 0.06 at birth, –1.27±1.37 and –1.27±1.72 before Glenn procedure, –0.47±1.08 and –0.69±1.17 before Fontan procedure, –0.42±1.18 and –0.39±1.48 at follow-up, respectively. A significant decline (P<0.05) in WAZ and HAZ was found before Glenn procedure. After the Glenn procedure, there was significant catch-up growth (P<0.05). The WAZ was stabilized after Fontan procedure, while the HAZ was reversed (P<0.05) continually. Conclusion    The somatic growth is impaired in children with stageⅡ Fontan procedure. There is a trough of growth before Glenn procedure. Although catch-up growth occurs after Glenn procedure, low body weight and growth retardation remain at follow-up. Effective interventions such as more intensive nutritional strategies before Glenn procedure should be targeted at those children in the future, so as to improve their growth.

Objective    To explore the effect of pulmonary arterial hypertension on the children with functional single ventricle in the early period after Fontan operation. Methods    Forty-three children with pulmonary arterial hypertension after Fontan operation were enrolled in our department between January 2015 and December 2016. There were 24 males and 19 females at a median age of 4.3 years ranging from 2.5 to 4.8 years. The pulmonary arterial pressure was evaluated by cardiac catheterization. There were 23 children diagnosed without pulmonary hypertension (a non-PAH group) including 16 males and 7 females, while 20 patients were diagnosed with pulmonary hypertension (a PAH group) including 8 males and 12 females. Postoperative parameters related to outcomes were compared between the two groups. Results    There was no death in the non-PAH group, but the mortality of children in the PAH group was 20.0% (4/20， χ2=5.34, P=0.02). The central venous pressure (t=–2.50, P=0.02), N-terminal prohormone of brain natriuretic peptide (NT-proBNP, Z=–3.50, P<0.01), peritoneal dialysis rate (χ2=5.40, P=0.02), incidence of arrhythmia (χ2=4.40, P=0.03) in the PAH group were significantly higher than those of the non-PAH group. The early postoperative utilization rate of pulmonary vascular targeting agents in the PAH group was significantly higher than that in the non-PAH group (χ2=6.30, P=0.04). Conclusion    Pulmonary arterial hypertension is one of the most important factors which influence the early postoperative prognosis of children with functional single ventricle after Fontan operation.