Objective To discuss the surgical technique in correction of tetralogy of Fallot with complete atrioventricular septal defect (TOF-AVSD). Methods 16 patients aged 2-16 years underwent correction of TOF-AVSD. The atrioventricular septal defect was closed through a right atriotomy and longitudinal right ventriculotomy in each case. The three-patch technique was used for the first 7 cases and two-patch technique for the later 9 cases. The commissure between the superior and inferior bridging leaflets of the left portion of the common atrioventricular valve was closed in each patient. RVOT obstruction was relieved by a transannular patch. Results Results There were 4 deaths in the early postoperative period, 3 deaths in the first 7 cases compared to 1 death in the later 9 cases (P

Objective To report the outcomes of double switch operation as the primary approach for congenital corrected transposition of great arteries (cTGA) with heart anomaly. Methods From April 2002 to June 2004, seven patients ranged 4 to 15 years with cTGA underwent double switch operation. Six patients were situs solitus (SLL segmental anatomy) while one patient was situs inversus (IDD segmental anatomy). The heart defects included ventricular septal defect in 6 cases, secondary atrial septal defect in, double outlet of right ventricle in 1, pulmonary stenosis in 6, dextrocardia in 3 and levocardia in 1. The operative procedures comprised of 4 modified Senning+Rastelli, Mustard +Rastelli +bidirectional Glenn, 1 Senning +Rastelli, 1 modified Senning+switch. Results There was one early operative death after modified Senning+switch operation. The cause of death was left ventricular failure. The postoperative complications included severe low cardiac output syndrome in 1, temporary atrioventricular block in 1, pleurisy and low plasma protein in 2. The survivors were followed up from 2 to 24 months, all of them were in sinus cardiac rhythm except one case with junction cardiac rhythm. All were in NYHA I class. Conclusion Anatomic correction of cGTA by double switch operation can be performed with lower operative mortality and good medial-term outcome. In the SLL cases, modified Senning operation may yield better outcomes than Mustard operation.

Objective To evaluate the experiences of complete repair of tetralogy of Fallot with pulmonary atresia (TOF-PA). Methods From June1984 to December 2003, 24 patients with TOF-PA underwent complete surgical repair. There were ten males and fourteen females. The age ranged from 6 months to 9 years. 14 patients were in type I TOF-PA, type II 8, type III and type IV 1 each. Through a standard median sternotomy incision, all patients were operated on under moderate hypothermia and cardiopulmonary bypass. An artificial vessel patch with a monocuspid valve was applied to enlarged the outlet of right ventricle in typeIpatients and a conduit with valves from right ventricle to pulmonary artery was applied in typeII patients. Midline one-stage complete unifocalization and repair of ventricular septal defect and major aortopulmonary collaterals were used in patients with type III and IV. Results Early mortality was 13.3% (4 patients). The causes of death were severe low cardiac output (2 patients), respiratory function failure (1 patient) and multiorgan function failure (1 patient). There was no late death. 18 patients were followed up from one month to 15.5 years. Postoperative heart function (NYHA) was class I or II in 16 patients and class III or IV in 2. Conclusion Surgical repair of patients with TOF-PA can be achieved with acceptable mortality and good results. In selected patients one stage surgical correction can be done through a midline sternotomy approach.

Objective To evaluate the accuracy and clinical practicality of DNA chip in comparison with serology in typing of human leukocyte antigen A (HLA-A) in Han's individuals of donor-recipients of transplantation. Methods 120 peripheral blood samples were obtained from donor-recipients of transplantation. Each sample was divided into two parts and HLA-A antigens were identified by DNA chip in one part and by serology in another. Samples in which the HLA-A typing results by these 2 methods were discordant were verified by polymerase chain reaction with sequence specific primers (PCR-SSP). Accuracy and clinical practicability of both methods were compared according to the typing results. Results Serological typing for HLA-A took 3 h, while DNA chip typing 4. 5-5 h. 112 samples have been typed successfully. Typing results were same in 91 samples and discordant in 21 cases. The verified results showed that DNA chip made 2 incorrect typing and the error rate was 2%. Meanwhile, serology made 19 mistakes, consisting of 5 antigens being incorrectly interpreted and 14 "blanks" turning out to be definable alleles. The discrepancy rate was 17 %. Conclusions DNA chip typing for HLA-A is suitable for clinical application in Chinese Han's population with a greater precision than serology. It may replace the serology in future after being improved and perfected.

ObjectiveTo study the pulmonary arterial development over five years in patients underwent extracardiac total cavopulmonary connection (ETCPC).Methods43 survived patients,who had undergone ETCPC were examined with pulmonary perfusion at one month and five year following the operation.Central venous pressure (CVP) and arterial oxygenation saturation (SatO2 ％ ) were measured by right cardiac catheter,pulmonary arterial index (PAI) and pulmonary vascular resistance (PVR) were calculated.Pulmonary blood distribution were measured and calculated by 99m Tc-MAA perfusion imaging.ResultsThe PAI and PVR of the follow-up group reduced significantly ( t =2.41,P ＜ 0.05 ; t =2.08,P ＜ 0.05 ),CVP also reduced significantly ( t =2.69,P ＜ 0.05 ),but SatO2 ％ did not changed significantly.Total radionuclide counts and the ratio of rightorleft pulmonary perfusion did not change significantly.( t =0.38,P ＞ 0.05 ;t =1.12,P ＞ 0.05 ),but the ratio of the superior and inferior lobe decreased significantly( t =2.54,P ＜ 0.05 ).ConclusionThe weak pulsation and low dynamic of Fontan circulation also can promote pulmonary vascular development.However,the improvement of hemodynamic in pulmonary circulation at mid-term follow will not lead to an increased amount of pulmonary perfusion or oxygen supply,which is probably due to the massive opening of the arteriovenous shunt and increased futile circulation.

Objective To study the long-term effect of total right heart bypass on pulmonary perfusion after extracardiac total cavopulmonary connection (ETCPC).Methods Fifty-three patients (29males,24 females; average age (10.8±6.1) years) undergoing ETCPC from March 1990 to December 2005were retrospectively analyzed.Forty-three patients had full set of pulmonary perfusion data at 1 month and 5years postoperation.The perfusion ratios of each lung segment were calculated based on 99Tcm-MAA radionuclide imaging.The PVR and pulmonary artery index were calculated from angiocardiography measurements.All data were compared with paired t test.Results Compared with the early postoperative data,the perfusion ratio of superior/inferior segment(0.72±0.20 vs 0.75±0.01; t =2.54,P＜0.05),the PVR ((142.98±2.61) vs (146.95±2.54) dyn · s · cm-5; t=2.08,P＜0.05; 1 dyn · s · cm-5 =0.1 kPa · s · L-1) and vena cava pressure ((9.35±0.24) vs (9.95±0.23) mmHg; t=2.69,P＜0.05; 1 mmHg=0.133 kPa) decreased significantly in follow-up data; while the ratio of posterior basal segment perfusion increased significantly (0.12±0.00 vs 0.10±0.03; t=2.16,P＜0.05).The arterial oxygen saturation ((92.70±0.30)％ vs (92.86±0.29) ％ ; t =1.12,P＞0.05),the total pulmonary nuclear counts ((701.91 ± 8.26) × 103 vs (698.93 ± 12.0) ×103 ; t=0.38,P＞0.05) and the perfusion ratio of inferior vena cava to the right lung (0.61±0.06 vs 0.60±0.06 ; t =0.74,P ＞ 0.05) were similar between the two terms of follow-up.The early angiographic and radionuclide perfusion studies did not match in 5 patients.Conclusions Hypostatic redistribution of pulmonary blood flow is the characteristics in long-term follow-up of ETCPC patients.The radionuclide imaging is superior to angiocardiography in revealing functional pulmonary blood perfusion.

Objective To evaluate the indication and surgical technique for treating tetralogy of F allot with pulmonary atresia (TOF-PA).Methods From June 1984 to June 2009,66 patients with TOF-PA underwent 69 operations.Among them,34 were males and 32 females.Their age ranged from 6 months to 29 years.The anatomic characteristics of TOF-PA included 31 cases of Type Ⅰ,14 Type Ⅱ,12 Type Ⅲ and 9 Type Ⅳ.The operations included palliative aorto-pulmonary shunts in 11 cases,one-stage unifocalization with unpatched VSD in 2 cases,one stage complete repair in 40 cases,one-stage unifocalization with VSD repair in 13 cases,and delayed intracardiac repair after shunt procedures in 3 cases.Results There were 6 early deaths,including 1 death happened after aorta-pulmonary shunt and 5 after complete repair.The causes of death were severe low cardiac output in 3 cases,respiratory failure in 1,multiorgan function failure in 1 and severe wound infection with endocarditis in 1 after aorta-pulmonary shunt.The postoperative oxygen saturation of the patients undergone shunt and one stage unifocalization with unpatched VSD increased to 82％ ～ 91％.The postoperative ratio of right ventricular pressure/left ventricular pressure after complete repair was ＜ 0.5 in 31 cases,18 cases were between 0.5 and 7 cases ＞ 0.75.47 patients were followed up from 3 months to 15.5 years.The heart function(NYHA) of 44 patients were in class Ⅰ or Ⅱ and 3 in class Ⅲ or Ⅳafter operation.Conclusion The surgical strategy for TOF-PA mainly depends on the anatomic characteristics of the pulmonary and aortopulmonary collateral arteries.An individualized approach based on the anatomy of the pulmonary circuits permits a better result in the patients with TOF-PA.Patients with well developed pulmonary arteries should undergo one stage complete repair as early as possible.

Objective To assess the results of the Fontan procedure in patients with visceroatrial heterotaxy syndrome and complex cardiac anomalies.Methods From April 2002 through December 2010,25 patients (male 10,female 15) had undergone the Fontan procedure for heterotaxy syndrome or atrial isomerism and complex congenital heart disease 28 times.Median age at operation was (9.1 ± 5.5) years (2 to 18 years).Heterotaxy syndrome were associated with right atrial isomerism (n =18) or left atrial isomerism (n =7),asplenia (n =13) or polysplenia (n =7),double inlet of left ventricle (n =15),double inlet and outlet of left ventricle (n =5),double outlet right ventricle with pulmonary atresia (n =2) and with pulmonary stenosis (n =1),tricuspid or mitral atresia (n =2).A bidirectional cavopulmonary shunt was performed in 8 patients (bilateral in 3 patients).A cavopulmonary shunt placement,so-called Kawashima operation,was performed in 4 patients.An extracardiac conduit Fontan connection was pefformed in 15 patients and intracardiac lateral tunnel Fontan connection in one patient.Results 2 patients died in hospital caused by ventricular failure.Five patients developed early postoperative atrial arrhythmias and 2 patients had sinus node dysfunction.Mean arterial oxygen saturation at discharge was 0.86 ± 0.07 (range,0.78 to 1.00).Follow-up (range,0.5 to 7 years) was available on 15 patients.Mean arterial oxygen saturation was 0.82 ±0.08 (range,0.68 to 0.97).Ventricular function was normal in 13 patients (EF range,0.50 to 0.66) and depressed in 2 patients.Four patients had a junctional rhythm.Conclusion The Fontan procedure was still the main procedure for patients with visceroatrial heterotaxy syndrome and complex cardiac anomalies,which can reach satisfactory early and medium-term results.The choice of Fontan procedure,extracardiac conduit Fontan connection,aggressive treatment of concomitant malformations were essential to improve the outcomes.

Objective To analyze the relation of right ventricular outflow tract (RVOT) procedures for repair of tetralogy of Fallot and postoperative right ventricular function in order to improve the postoperative results at mid-long term follow-up.Methods A total of 125 patients for data collection with repair of TOF between January 2003 and January 2006 were prospectively enrolled in the study.Among them,66 were male and 59 were female.The ages ranged from 1 to 5 (3.4 ± 1.1) years,weight from 7 to 26 (15.2 ± 5.7) kg.20 healthy controls with a similar age and body weight were included.The type of RVOT procedure included transannular patch repair (M1) in 67 patients,patch to infundibulum (M2) in 6 patients,tranright-ventricle (RV) no patch repair (M3) in 18 patients and transatrial-transpulmonary no patch repair (M4) in 34 patients.Doppler echocardiogram and tissue Doppler imaging were performed to evaluate RV function.RV systolic function was evaluatedusing tricuspid annular plane systolic excursion (TAPSE) and index of myocardial performance (MPI).RV diastolic function was evaluatedusing ratio of Em/Am and E/Em.Unvariate and logistic regression analysis were used to identify the affective factors.Results In five years fellow-up,MPI and E/Em were higher in patents with M1 and M2 than those in the patients with M3 and M4.Univariate analysis revealed Nakata index,previous shunt operation,operative techniques、postoperative pulmonary regurgitation and during of QRS as risk factors for MPI.In the multivariate analysis,operative techniques and during of QRS were risk factors for MPI.Univariate analysis revealed Nakata index,operative techniques,PR and during of QRS as risk factors for E/Em.In the multivariate analysis,PR was the risk factor for E/Em.Conclusion RV dysfunction is associated with the type of RVOT procedure,diastolic dysfunction is correlative with postoperative PR and systolic dysfunction is correlative with enlarge of RVOT with patch and during of QRS.Echocardiographic imaging is a simple and reliable method for assessment of RV function after repaired TOF.

Objective Study the management and outcomes of one-stage repair of aortic coarctation or interrupted aortic arch associated with cardiac anomalies through median sternotomy.Methods From July 2002 to June 2009,43 patients with aortic coarctation(34 cases)or interrupted aortic arch(9 cases)and associated with cardiac anomalies underwent one-stage repair.There were 27 males and 16 females.The age ranged from 5 months to 9 years and the body weight from 3.5 kg to 29.0 kg.The associated cardiac anomalies included ventricular septal defect in 42 patients,patent ductus arterious in 34,secundum atrial septal defect in 12,subaortic stenesis in 5,mitral valve regurgitation in 2 and double outlet of right vantricule in 1.All patients underwent one-stage repair through median sternotomy.The aortic continuity was reestablished by direct anastomosis between the descending aortic segment and aortic arch.Results There was one postoperative death.The causs was pulmonary hypertension and severe low cardiac output syndrome.The postoperative complications included severe low cardiac output syndrome in 3 patients,hypoxemia in 6,pneumonia in 11,atelectasis in 14,injury of recurrent laryngeal nerve in 19,and supra ventricular tachycardia in 23.34 patients were followed up from 3 months to 5 years and were in good condition without recoarctation.Conclusion The outcomes of early and medium term for one-stage repair of aortic coarctation or interrupted aortic arch and associated cardiac anomalies through median sternotomy is excellent.Technique of extended anastomosis between the descending aortic segment and aortic arch may reduce the incidence of recoarctation