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.
Objective To investigate the surgical treatment methods and effects for pulmonary atresia with ventricular septal defect (PAVSD) in elder children and adults in order to promote the treatment effects. Methods From October 1996 to October 2008, we performed stage1 or staged biventricular repair on 39 PAVSD patients including 21 males and 18 females, ranging from 8 to 27 years old with an average age of 13.43 years. There were 14 cases of type A, 11 cases of type B, and 14 cases of type C. Among them, 23 patients underwent stage1 radical repair in which either human blood vessel with valves or bovine jugular vein with valves were used to connect the pulmonary artery and the right ventricular outflow tract. In these 23 patients, 3 patients complicated with major aortopulmonary collaterals(MAPCAs) underwent unifocalization (UF) operation. The other 16 patients received staged repair, including 9 cases of systemic to pulmonary artery shunt and 7 of staged radical cure. Results There were 6 perioperative deaths with a total mortality of 15.38%(6/39), including 4 (17.39%) stage1 radical repair cases and 2 (12.50%) staged radical repair cases. The former 4 were all type C patients, dying from low cardiac output due to increased pulmonary arterial pressure. In the latter 2 deaths, 1 was a type B secondary shunt patient, and the other was a type C staged radical repair case, both of whom died of bleeding caused by aortic injury in the succeeding operations. Followup was done on 28 cases with a followup rate of 84.85%. The followup time ranged from 14.0 months to 9.2 years with 5 cases missing. No patient died during the followup, and 9 patients maintained their cardiac function at class Ⅰ, 13 at class Ⅱ, 5 at class Ⅲ and 1 at class Ⅳ. Three patients had aortic valve regurgitation of small to medium volume, the treatment of which included an administration of oral potassium diuretic medication and regular follow-up. Conclusion Pulmonary vessels of elder children and adults with PAVSD are usually injured severely and oftentimes it is complicated with MAPCAs. Standard for stage1 radical repair should be defined more strictly based on the present one.
Objective Comparing postoperative change of blood gas and hemodynamic status in patients underwent a right ventricletopulmonary artery (RVPA) conduit or a modified BlalockTaussig (mBT) shunt for pulmonary atresia with ventricular septal defect and without major arterial pulmonary collaterals (MAPCAs), to affirm the effect on oxygen supply /demand with different procedure. Methods From July 2006 to October 2007, 38 patients with pulmonary atresia and ventricular septal defect without MAPCAs were divided into two groups according to different procedures: RVPA group (n=25) and mBT group (n=13).Perioperative mortality, blood gas and hemodynamic data during postoperative 48 hours, including heart rate, blood pressure, systemic oxygen saturation, mixed venous oxygen saturation, oxygen excess factor, inotropic score were compared in both groups. Results The difference in the mortality between RVPA group (4.0％，1/25) and mBT group （7.7％，1/13) showed no statistical significance（Pgt;0.05）. The total of 33 patients were followed up, the followup time was from 6 to 18 months.11 patients (4 patients in mBT group, 7 patients in RVPA group) underwent corrected procedures during 9 to 18 months after palliative procedures, one case died of elevated pulmonary vascular resistance and right ventricle failure. The mixed venous oxygen saturation at 24h and 48h after surgery were higher than that at 6h after surgery (Plt;0.01) both in RVPA group and mBT group. The systolic blood pressures at 6h, 24h, 48h after surgery in RVPA group were lower than those in mBT group (P=0.048，0.043， 0.045)，the mean systemic blood pressures in RVPA group were higher than those in mBT group (P=0.048, 0.046, 0.049),the diastolic blood pressures in RVPA group were higher than those in mBT group (P=0.038, 0.034, 0.040)， the inotropic scores in RVPA group were lower than those in mBT group (P=0.035， 0.032，0.047). Conclusion The blood pressures and inotropic scores are found significantly different in RVPA conduit and mBT procedures, while postoperative systemic oxygen delivery areequivalent. Both RVPA and mBT patients decline to nadir in hemodynamic status at 6 h after surgery.
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.
Abstract: Objective To review the shortterm outcome of modified Nikaidoh operation, aortic translocation and biventricular outflow tract reconstruction as an alternative surgical procedure for the treatment of transposition of the great arteries with ventricular septal defect and pulmonary stenosis (TGA/VSD/PS). Methods Between January 2004 and December 2005, 8 consecutive patients had undergone Nikaidoh procedure for the treatment of TGA/VSD/PS at Shanghai Children’s Medical Center. All patients had ventriculoarterial discordance and atrioventricular concordance. Associated lesions included a straddling atrioventricular valve in one patient and hypoplastic left pulmonary artery. The median age at operation was 11.4±7.6months (4 to 29months). Weight of body was 8.0±1.9kg (5.2 to 11.0kg). No patient had previous palliative procedure. The surgical technique used was a modification of the Nikaidoh procedure. Results The median total cardiopulmonary bypass time was 176±50 minutes (range,112 to 250 minutes), and the median aortic crossclamp time was 101±27 minutes (range, 73 to 139minutes). The median length of stay in the intensive care unit was 12±9 days, with a median hospital stay of 19±12 days. There was 1 hospital death as a esult of severe left ventricle failure. There was no residual left ventricular outflow tract obstruction (LVOTO) and right ventricular outflow tract obstruction (RVOTO), but 3 patients with mild to moderate pulmonary regurgitation and 4 patients with moderate. At a median follow-up of 8.8 months (range, 3 to 18months), all patients were alive. All have the normal ventricular function. There were ejection fraction (EF) 0.64±0.02 and fractional shortening (FS) 0.33±0.02. None of the patients developed aortic insufficiency and progressed LVOTO or RVOTO. Conclusions Nikaidoh procedure is a valuable surgical option for TGA/VSD/PS in infant, especially in the presence of “inadequate anatomy” for a Rastelli repair. Big evidence and longer follow-up are required to fully assess the potential longterm benefits of this procedure compared with the Rastelli repair.
Surgical treatment of complete transposition of great arteries with ventricular septal defect and pulmonary stenosis (TGA/VSD,PS) consists of Rastelli procedure, Lecompte procedure, Nikaidoh procedure, Yamagishi procedure and Ross-Konno switch procedure. Rastelli procedure and Lecompte procedure cause less myocardial lesion but more late complications. Nikaidoh procedure and Yamagishi procedure achieve better anatomical repair but involve more myocardial lesion. Ross-Konno switch procedure has a narrow surgical indication. So for patients with TGA/VSD,PS, different surgical methods should be used according to surgical indications and individual conditions. In this paper, the advantages and disadvantages, indication, contraindication, outcome and prospect of them are reviewed.
Objective To summarize the experience of surgical treatment of complete atrioventricular canal defect （CAVCD） in 94 patients. Methods Ninety-four patients with CAVCD underwent surgical therapy. CAVCD were repaired by using two-patch technique in 65 patients and using single-patch technique in 29 patients. Additional cardiovascular anomalies were corrected simultaneously. Results There were 10 hospital deaths （10.6%）, 4 patients were less than 6 month old. Four patients died of severe mitral valve regurgitation, 3 died of pulmonary hypertensive crises and 3 died of low cardiac output syndrome, cerebral complications and aerothorax separately. Follow-up was completed in 84 patients, with a duration of 3-6 months. Mild degree mitral valve regurgitation was observed in 18 patients by echocardiography, mild to middle degree mitral valve regurgitation was observed in 12 patients. Conclusions Postoperative severe mitral regurgitation and pulmonary hypertensive crises were the main cause of deaths for correction of CAVCD. Early correction of CAVCD and satisfactory reconstruction of atrioventricular valve could obtain a satisfactory result, routine evaluation with intraoperative transesophageal echocardiography could result in a low operative mortality.
Objective To investigate the effect of surgical treatment on ventricular septal defect （VSD） in infants under 6kg weight, including the operative indication, surgical techniques and perioperative therapy. Methods All clinical data of 148 consecutive infants under 6kg weight with VSD were collected and studied retrospectively. The infants, age was 1-13（mean 5.3） months with the body weight of 3.5-6.0 （mean 5.3） kg. VSD was perimembranous in 105 cases, subpulmonary in 25, muscular inlet tract in 8, muscular outlet tract 9, and muscular trabecular in 1 case. Other associated cardiac abnormalities included atrial septal defect in 39, patent ducts arteriosus in 17, insufficiency of mitral valve in 9 and moderate to severe pulmonary hypertension in 52. The operations were performed under cardiopulmonary bypass at moderate to low flow, moderate hypothermia and cold crystalloid cardioplegia. Patch repair was used in 85, direct sutures in 63 and 23 cases repaired with partial sternal incision and beating heart. Results The hospital mortality was 4. 1% （6/148）, the causes of death were severe pulmonary hypertention in 2, aortic arch interruption in 2, severe malnutrition in 1 and poor result of mitral valvuloplasty in 1. Other major operative complications included residual shunts （1- 2mm） in 2, and Ⅲ° A-V block in 2, who recoveried 5 days after the operation. The hospital stay was 6 15 （mean 8） days. Follow-up was complete in all 142 survived cases for 4 months-6 years. Two residual shunts healed in first year after the cardiac operation, others recovery smoothly, and are developing well. Conclusion With the improvement of the surgical techniques, the surgical treatment for VSD in infants with low weight is safe and effective, and it is also essential to further improve the effects of surgical treatment in VSD associated with complex abnormalities.
Objective To discuss the methods and results of the surgical treatment of apical muscular ventricular septal defects （VSD） , in order to improve the surgical outcome of this disease. Methods The clinical data of 29 patients with apical muscular VSD of Fuwai hospital through Jan. 1999 to July 2004 were analyzed retrospectively. The apical VSDwere repaired via 4 different approaches： right atrium, apical right ventriculotomy, apical left ventriculotomy and the incision of the outflow tract of right ventricle combined with apical left ventriculotomy. The operative outcomes between different approaches were analyzed and compared. Results There were 2 perioperative deaths （6. 9%）. One patient repaired via apical left ventriculotomy died of low cardiac output syndrome, the other died intraoperatively from endocardial fibroelastosis, which was confirmed by myocardial biopsy. There were residual VSD in 4 （13. 8%） patients, who were repaired via the right atrium, which did not necessitate reoperation. Other patients were discharged uneventfully. Follow up was completed {n 11 patients and extended 2 to 34 months, none of the 6 survivors had apical ventricular aneurysms. Conclusions The operative outcome of patients with apical VSD is satisfactory although the incidence of residual VSD is higher. The intraoperative transesophageal echocardiography （TEE） should be performed routinely. Surgical approach should be chosen on the actual site of the VSD at operation. Try to repair the VSD via the right atrium as possible, via the apical right ventrieulotomy if needed and try to avoid left ventriculotomy.
Objective To access the feasibility, effectiveness and safety of transcatheter closure of ventricular septal defects(VSD) in 17patients. Methods Seventeen patients, aged 4 23 years, were selected by transthoracic echocardiography. The location of VSD was perimembranous in 16 patients and was muscular in 1 patient. And 1 patient with aneurysm formation. All cases had no severe pulmonary hypertension or right to left shunt. Membranous VSD underwent transcatheter closure with Amplatzer membranous VSD occluder or membranous VSD occluder made in China. Muscular VSD was closed by Amplatzer patent ductus arteriosus occluder. Results The VSD diameter ranged from 2.3 10.5 mm(5.75±2.10 mm). The device diameter ranged from 412 mm (7.12±1.67 mm). After application of the prosthesis there was no residual shunt in all patients. One patient developed grade I atrioventricular block and complete right bundle branch block. Two patients developed right bundle branch block after 3 4 days. No other compli...更多cations were observed in 1 12 months follow up. Conclusion The transcatheter closure of VSD appears to be a safe and effective method.