Abstract: Objective To investigate the clinical anatomy of ventricular septal defect(VSD) in tetralogy of Fallot(TOF)，reassess its classification and technique of surgical closure. Methods The data of one hundred consecutive patients with TOF (between January 2002 and June 2006) were reviewed. Their ages ranged from 2 months to 13 years, weights ranged from 5 to 38kg, percutaneous oxygen saturation(SpO2) ranged from 57％ to 92％, haematocrit(HCT) ranged from 0.34 to 0.74, Nakata index ranged from 90 to 210mm2/m2 and McGoon ratio ranged from 0.8 to 2.0. The clinic anatomy of the VSD was studied intraoperatively. Results Among them, seventy one patients had fibrous continuity between the leaflets of the aortic and tricuspid valve. Ninteen patients had a muscular postero-inferior border. Ten patients had subpulmonary VSD’s. There was no third degree atrioventricular block (3°AVB). Two patients had transient-atrioventricular dissociation but subsequent returned to sinus rhythm. Twenty two patients had incomplete right bundle branch block. Three patients were found to have very small residual VSD (less than 0.2cm) in the posteroinferior borders which closed spontaneously after 6 months. ConclusionVSD in TOF when classified as peri-membranous, muscular and subpulmonary VSD may improve the accuracy of surgical closure.
Objective To evaluate long-term outcomes of surgical repair for pulmonary atresia with ventricular septal defect （PA-VSD） and major aortopulmonary collateral arteries （MAPCAs）.?Methods?We retrospectively analyzed the clinical data of 29 patients with PA-VSD and MAPCAs who underwent surgical repair in Shanghai Children’s Medical Center from February 2001 to February 2010. There were 13 patients in the one-stage operation group and 16 patients in the staged operation group. There were 8 males and 5 females in the one-stage operation group with their age ranging from 8 to 168 （26.0±17.0）months. There were 9 males and 7 females in the staged operation group with their age ranging from 4 to 149 （26.5±15.8）months. Eight patients underwent their second operation with the time interval between the two operations ranging from 14 to 40 （28.3±11.7） months. For MAPCAs， 9 patients underwent unifocalization， 16 patients underwent ligation or occlusion via cardiac catheterization， and the other 4 patients left them open or unrepaired. We compared postoperative mortality， complications， long-term outcomes， and quality of life between the two groups. Quality of life of the 29 PA-VSD patients was measured according to Pediatric Quality of Life Inventory version 4.0 and compared with 100 children from a kindergarten as a control group.?Results?Postoperative mortality of one-stage operation group was significantly higher than that of staged operation group at 15.4% （2/13） vs. 0.0% with χ2=4.12 and P=0.04. There was no statistical difference in the rate of postoperative complications between the two groups at 75.0% （9/12） vs. 56.2% （9/16） with χ2=0.51 and P=0.47. Patients were followed up for 3 to 88 months， and the follow-up mortality of one-stage operation group was not significantly higher than that of staged operation group at 27.3% （3/11） vs. 6.2% （1/16） with χ2= 2.28 and P=0.13. There was no statistical difference in overall quality of life score between one-stage operation group and staged operation group （66.7±8.6 vs 70.2±13.0， t?=?-0.71， P=0.48） . The overall quality of life score of PA-VSD patients was significantly lower than that of control group （68.7±11.2 vs. 77.8±15.7， t?= 3.14， P=0.01）， mainly because the physical functioning score of PA-VSD patients was significantly lower than that of control group （57.7±11.7 vs. 83.0±19.6， t?=5.67， P=0.00）， but there was no statistical difference in emotional well-being score， social functioning score， and school functioning score between PA-VSD patients and the control group.?Conclusion It is the key to analyze different pulmonary artery conformation， choose individualized surgical procedure and properly manage MAPCAs so as to reduce postoperative mortality and complications of surgical repair for patients with PA-VSD and MAPCAs.