Objective: To describe the superior approach for correction of supracardiac (type I) total anomalous pulmonary venous return. Methods: From June 1998 to August 2001, total 11 of the supracardiac type of TAPVC were corrected by the superior approach. There were 7 males and 4 females with mean age of (5。33?4。98) years(5 months to 15 years) and mean weight of (15。09? 8。78)kg (6。4 to 33 kg). The total correction was performed under CPB. The top of the left atrium and the common pulmonary venous trunk were exposed through the transverse sinus and a direct anastomosis between those was done. Results: There was no operative mortality. No late death and arrhythmia occurred during follow-up period (4 months to 3 years). Conclusion: This superior approach for correction supracardiac type of TAPVC can afford a better exposure and a bigger orifice between the left atrium and the common pulmonary venous trunk and less injury. Therefore, the postoperative morbidity of arrhythmia is low.

Objective To investigate the hemodynamic effects and mechanisms of aerosolized iloprost in children with pulmonary hypertension(PAH)after congenital heart surgery,in the setting of early ventilation and continuous nitric oxide(NO)inhalation were administered.To observe the outcomes of the patients after iloprnst therapy.Methods From April 2008 to April 2009,all postoperative children with PAH in ICU,Fuwai hospital had regularly been given ventilation and NO inhalation at a dose of 10 ppm for 2 hours since they were leaving the operation room,and then ultrasonic cardiography was used to evaluate the systolic pulmonary arterial pressure(sPAP=4×TIVmax+RAP).Thirty children were diagnosed as having postoperative PAH for sPAP/sBP≥0.5.They were divided into two groups(group T and group C)randomly,with ventilation and NO inhalation administered continuously,and were given inhaled iloprost at a dose of 100 ng·kg-1·min-1*10 min and inhaled 0.9% NaCl 4ml respectively,once every 4 hours for 48 hours.We used the 24-hour cardiac monitors and ultrasonic cardiography for hemodynamic monitoring in the patients of two groups at the six time points:baseline(t1),20 min later after the first inhalation(t2),120 min later after the first inhalation(t3),24 hours later after the treatment(t4),48 hours later after the treatment(t5) and 24 hours after ceasing the last inhalation(t6).We examine the blood cAMP and cGMP by ELISA assay before and after the first inhalation.We observed the blood coagulation,the liver and kidney function of these patients.The outcomes of these patients were also investigated.Results At t1,the sPAP and sPAP/sBP had no differences between the two groups.At t2,the sPAP(43.23±11.72)mmHg and sPAP/sBP(0.48±0.13)in group T were both lower than the sPAP(53.13±13.60)mmHg and sPAP/sBP(0.60±O.15)in group C(P<0.05).At t3,the sPAP/sBP of group T was also lower than that of group C(0.48±0.09 vs 0.59±0.14,P<0.05).At t4 and t5,the sPAP in group T were (39.84±12.87)and(34.99±12.98)mm Hg,with sPAP/sBP(0.42±0.15)and(0.36±0.14),were much lower than those in group C(P<0.01).From t1 to t2,the cAMP level increased sharply from(406.64±179.18)to(578.68±193.05)pg/dl in group T(P<0.01),and was also obviously higher than that in group C at t2(392.26±94.46)pg/dl(P<0.01).HR,BP and RAP showed no notable difference between two groups at every time point.So did the PIP.There were no differences in coagulation,liver and kidney function at t6.2 patients died from pulmonary hypertension crisis(PAH)in group C and no one in group T.One patient showed flush during the treatment in group T and recovered spontaneously after the inhalation.Nevertheless,none in group C.Conclusiou Inhaled iloprost significantly improved pulmonary hemodynamics in children with PAH after congenital heart operation even thongh they were ventilated and inhaling NO.Increased blood cAMP level was considered to be a contributing factor.Howeve,systemic BP remain unaffected after iloprost inhalation.Aerosolized iloprost may be associated with improvement in the survival and deczeased PHC.

Objective: To evaluate the clinical results of cryopreserved homografts for the treatment of complex congenital heart defects. Methods: In 420 patients with complex congenital heart diseases received surgical correction with right ventricular outflow tract (RVOT) reconstruction was done using cryopreserved homografts. Of the 420 homografts, 234 were aortic homografts and 186 were pulmonary homografts. The mean age of patients was 4.6 years (range, 5 months to 21 years). The mean body weight was 17.5 kg (range, 5.5～52 kg). The mean follow-up time was 27.8 months (range, 3 months to 12.9 years). Results: There were 19 operative deaths with a mortality rate of 4.5%. The postoperative complications occurred in 25 patients and they all recovered uneventfully. The postoperative mechanical ventilation time was 48.7±42.5 hours (range, 8～192 hours). The mean hospital stay was (12.2±9.0) days (range, 7～46 days). There were no late death and complication. Conclusion: The results of this study suggest that ROVT reconstruction with cryopreserved homografts as transannular patch can be achieved with excellent the clinical outcome.

Objective To study the therapeutic effects of inhaled iloprost on pediatric pulmonary hypertension after congenital heart disease operation and to investigate the mechanisms of iloprost.Methods Thirteen 10 min every time,every 4 hours for 48 h after the surgery.We used the 24 h cardiac monitoring and ultrasonic cardiography for hemodynamical monitoring.We examined the blood cAMP and cGMP by ELISA assay.Results The patients were(17.88±12.56) months old with average weight of(9.29±3.59) kg.The right atrial pressure,systolic pulmonary arterial pressure,systolic pulmonary arterial pressure/systolic blood pressure were(11.5±1.41)mm Hg,(61.64±13.6) mm Hg and 0.66±0.16 before the treatment and they were(9.88±1.88) mm Hg,(47.67±12.18) mm Hg and 0.52±0.15 at the time point of 20 min after the treatment.For all the three parameters,there were significant differences before and after treatment.The effect of iloprost continues to 120 min later.The cAMP level was(335.75±127.31) μg/L before iloprost inhalation and increased to(519.68±148.54)μg/L at the time point of 20 min after inhaling iloprost,showing a significant difference(P＜0.01).Blood pressure,cGMP level and ventilator parameters showed no notable difference before and after the treatment.Conclusion Inhaled iloprost significantly improves pulmonary hemodynamics in children with pulmonary hypertension after congenital heart disease operation,which might be achieved by increasing the blood cAMP.However,systemic blood pressure and respiratory function keep unaffected after iloprost inhalation.

60 minutes. There were no significant differences in the three groups in age, sex ratio, C/T ratio, or left ventricular function. Blood samples for analysis were collected before skin incision and at time intervals up to 6 days postoperatively. Analysis of creatine kinase MB, LDH and cardiac-specific troponin I was used for the detection of myocardial damage. Meantime, the ECG was checked for myocardial infarction. After the reperfusion, myocardial tissue was obtained from the free wall of right ventricle myocardial structure studies. Results: The level of cTnI was increased significantly when the time of myocardial ischemia was prolonged. The changes of CK-MB and LDH were not significant in these three groups. Electron microscopy demonstrated the mitochondria of myocardial cell swelled, the myofilament shortened and the sarcoplasmic reticulum vacuolated in group III. The ECG was almost normal in all groups. Conclusion: The cTnI was an early and highly sensitive biochemical marker of ischemic and reperfusion injury during correction of cardiac defects in children. The concentration of cTnI was correlated ischemia with the degree of so evaluation of the release of cTnI could be used to assess myocardial protection during cardiac operation.

Objective: The aim of this study is to review the experience of using bidirectional Glenn shunt without cardiopulmonary bypass(CPB). Methods: Fifty-eight patients underwent bidirectional Glenn shunt without CPB between May, 2000 and September, 2001. The age was (3.65?1.59) years and the weight was (13.6?4.0) kg. The procedure consists of establishing temporary bypass with cannulae placed in the SVC and right auricular appendage for venous drainage and transection of right SVC. The cardiac end of the SVC is overseen. The cranial end is anastomosed to a longitudinal incision in the superior margin of the right pulmonary artery with absorbable running suture. The anterior wall of the anastomosis is widened with pericardium patch. Results: There was no operative mortality. Five cases had postoperative complications including coma in 1 and chylothorax in 4. The mean SVC crossclamp time was (48?15) mins. The preoperative oxygen saturation and CVP were 0.75?0.09 and (12.8?2.3) mmHg, respectively. While the postoperative oxygen saturation and CVP were 0.93?0.05 and (16.5?2.9) mmHg, respectively. The drainage was (145?103)ml. The average mechanical ventilation time was (13?7) hrs. The mean postoperative hospital stay was (10?5) days. Conclusion: Bidirectional Glenn shunt without CPB is a safe and reliable method, for complicated congenital heart diseases children with inadequate pulmonary blood flow when anatomic and primary physiological correction are not suitable.

Objective: We assessed whether inhalation of low dose of nitric oxide comparing with administering 100% oxygen during cardiac catheterization would be as a method to screen patients with congenital heart disease and pulmonary hypertension for operation. Methods: We chose 67 cases of congenital heart disease with pulmonary hypertension undergoing cardiac catheterization. While spontaneously breathing room air (FiO 2, 0 21), the pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) were recorded as baseline. Patients continued to breathing 40 ppm nitric oxide with FiO 2 0.30 and 100% oxygen, for 30 minutes. Effects of inhalation of nitric oxide on pulmonary vasculature were studied. Results: Comparing with breathing room air, after inhalation of 100% oxygen or 40 ppm nitric oxide, PAP, PVR and PRV/SVR were decreased significantly. Conclusion: Comparing with administer 100% oxygen, inhalation of nitric oxide is more sensible and reliable predictor in selecting patients with congenital heart disease and pulmonary hypertension for operation.

Objective: To study the correlation between radionuclide pulmonary perfusion imaging (PPI) data and quantitative analyzed data of lung biopsy specimens in congenital heart defects with left to right shunt associate with severe pulmonary hypertension in young children. Methods: 52 data of PPI were collected from 43 young children with severe pulmonary hypertension caused by congenital heart defects. Lung biopsies were performed in 26 cases and the specimens were quantitative analyzed. The correlation between PPI and lung biopsy was studied. Results: The percentage of wall thickness (%MT) and percentage of wall area (%MS) were significantly increased in cases with right upper to lower count ratio (RULR)≥0.70 (P

Objective: The expression of eNOS and iNOS in lung tissues plays an important role in lung injury after cardiopulmonary bypass. The aim of this study is to evaluate the effect of pulmonary artery perfusion with hypothermic solution on eNOS and iNOS expression in lung tissue during CPB. Methods: 34 young children with congenital heart disease and pulmonary hypertension were divided into the control group (n=23) and protective group (n=11). The basic parameters (age, weight, data of catheterization and other assistant examination) were similar. In the control group, operation were performed using routine techniques, and in the protective group, pulmonary artery was infused with 4℃ protective solution during CPB. Immunohistochemical analysis was performed on 4-?m paraffin sections of lung tissue at end of CPB. The distribution and expression of eNOS and iNOS in pulmonary endothelium, bronchi epithelium and macrophages were studied using the immunohistochemical technique. Results: The strong iNOS-expression was showed in pulmonary endothelium (2.24?0.60) and lower iNOS-expression in bronchia epithelium (0.88?0.60) and alveolar macrophages (10.6?4.0) in protective group and lower eNOS-expression in endothelia and over iNOS-expression in alveolar macrophages and bronchia epithelia in the control group (P

Objective: To review the experience of valvuloplasty in children aged 4 months to 6 years. Methods: 199 children aged 4 months to 6 years (mean age, 2.94 years) and weight 3.1 kg to 22.0 kg (mean, 11.7 kg) underwent valvuloplasty under CPB from January 1990 to December 2001. 21 patients with isolated valvular lesions mitral incompetence(MI) in 7, tricuspid incompetence(TI) in 6, aortic incompetence(AI) in 1, aortic stenosis(AS) in 1, MI and TI in 2, MI and AI in 2, MI and AS in 1,MI and mitral stenosis in 1, the remain 178 patients had valvular disease with other pathologies (MI=122, TI=26, AI=9, MI+TI=13, tricuspid stenosis=2, AS=2, MI and AI=2, MI+AS=2). The procedures of valvuloplasty included leaflet resection and repair, annulus remodeling, choral shortening, transferal etc. depended on the anatomical variation of the lesions. Associated cardiac anomalies were corrected simultaneous. Results: There were 4 early deaths (2.0%) including 2 heart failure, 1 severe infection and 1 pulmonary hypertension. No late death was encountered during the period of 4.7 years (range 2 months-8 years) follow-up. Conclusion: Good result may be expected in valvuloplasty in children aged 4 months to 6 years.