Objective To summarize the experience of open heart operation on neonates with critical and complex congenital heart diseases and evaluate the methods of perioperative management. Methods From May 2001 to January 2003, 12 patients of neonates with congenital heart diseases underwent emergency operation. Their operating ages ranged from 6 to 30 days, the body weights were 2.8 to 4.5 kg. Their diagnoses included D-transposition of the great arteries in 4 cases, ventricular septal defect with atrial septal defect in 5 cases, complete atrioventricular septal defect, obstructed supracardiac total anomalous pulmonary venous drainage and cardiac rhabdomyomas in 1 case respectively. 12 cases were operated under moderate or deep hypothermic cardiopulmonary bypass. Results All cases were observed in ICU for 2-11 days and discharged 7-19 days after operation. The postoperative complications included low cardiac output, mediastinal infection, respiratory distress syndrome, systemic capillary leak syndrome and acute renal failure. All cases were cured and the follow-up (from 6 months to 2 years) showed satisfactory outcome. Conclusion A particular cardiopulmonary bypass and proper perioperative management is very important to ensure the successful outcome. Peritoneal dialysis is an effective and safe method for treating acute renal failure after cardiac operation in neonates.
Objective To evaluate the clinical outcomes of total cavopulmonary connection (TCPC) and bidirectional Glenn shunt for treating complex congenital heart diseases with single functional ventricles. Methods From January 2002 to May 2004, twelve children, who had complex congenital heart diseases with single functional ventricles, underwent TCPC and bidirectional Glenn shunt. Among them, male was 3 and female was 9. Ages were from 4 to 13 years and body weights were from 14 to 34 kilograms. The diseases included mitral atresia 1 case, tricuspid atresia 3 cases, right ectopic heart with transposition of great arteries 3 cases, D-transposition of great arteries 3 cases, and single ventricle 2 cases. Results Eleven children survived and one child died in acute renal failure 19 hours after operation. The hospital mortality was 8.3%. Four children had chyle-thorax postoperatively, and eight children had uneventful recovery. In the follow-up period, one child died 12 months postoperatively for pulmomary arteriovenous fistula, and there were no complications like severe arrhythmia, thrombosis and cerebral problems. Conclusions TCPC and bidirectional Glenn shunt are safe and effective techniques for treating complex congenital heart diseases with single functional ventricles, and the clinical outcomes are satisfactory. The key points for the successful operation are big enough cava-pulmonary anastomosis as well as aggressive perioperative management.
Bidirectional superior cavopulmonary anastomosis(BCPA)is a palliative method used in the single ventricular repair. It mainly includes bidirectional Glenn shunt and hemi-Fontan operation. The indications of BCPA are those as an intermediate option of total cavopulmonary anastomosis, partial biventricular or 1 1/2 ventricle repair and a practical approach to complex congenital heart surgery. The choise of age,influence on pulmonary artery maturation,remain of additional pulmonary flow,formation of collaters and time to Fontan are demand of study.
Objective To investigate the operative characteristic and results of the modified Fontan procedure, and improve the application of Fontan procedure in the clinic. Methods From Sep. 1992 to June 2006, 77 cases (aged 2.5 years to 20.0 years) with a wide range of complicated congenital heart diseases underwent the modified Fontan procedure.Right atriumpulmonary artery connection were performed in 21 cases, intraatrium fenestrated baffle or conduit total cavopulmonary connection were performed in 28 cases, extracardiac conduit total cavopulmonary connection were performed in 24 cases, atrium wall lateral tunnel total cavopulmonary anastomosis were performed in 2 cases, extracardiac pericardialtube total cavopulmonary anastomosis was performed in 1 case, and extracardiac direct total cavopulmonary connection was performed in 1 case. Results In early postoperative period, there were 1 case of successful reoperation and 5 death, the cause of death were heart failure (3 cases), arrhythmia (1 case) and cerebral hemorrhage (1 case). The early survival rate was 93.5%(72/77), with 92.0% free from failure. The eject fractions of left ventricle (LVEF) after operation were increased than those before operation （68.5%±4.0% vs.62.0%±4.5%,P=0.032） and left ventricular enddiastolic diameter (LVEDD) were decreased than those before operation （52.5±7.8mm vs.62.5±11.0mm, P=0.013）. A total of 63 cases (87.5%) were followed up from 1 to 15 years after operation. In late postoperative period, there were 4 cases of death, and 1 of successful re-operation. Late survival rate was 88.3%, with 86.0% free from failure. Conclusion In the treatment for complicated congenital heart diseases, the modified Fontan procedure could result in good early and longterm outcome, and the choices for surgical procedure are various.
ObjectiveTo summarize the experience of right ventricular outflow tract reconstruction with self-made single-valve conduit for the treatment of complex congenital heart disease, and to explore the key points of operation, and to evaluate the short-term and medium-term results of the treatment.MethodsWe retrospectively analyzed the clinical data of 65 patients with complex congenital heart disease treated by self-made single-valve conduit from January 2006 to June 2018. There were 42 males and 23 females aged 5-23 (9.9±4.2) years with weight 15-65 (26.2±9.9) kg. There were 19 patients with single valve artificial blood vessel (an artificial vascular group), and 46 patients with single valve bovine pericardium tube (a bovine pericardial tube group). There were 48 patients of ventricular septal defect (VSD) with pulmonary atresia (PA), 10 patients of corrective transposition of great artery with pulmonary artery stenosis, 5 patients of tetralogy of Fallot with single coronary artery malformation, 2 patients of double outlet of right ventricle with pulmonary artery stenosis and single coronary artery malformation.ResultsTwo patients died early after operation, both of them were VSD/PA patients who underwent radical treatment of extravascular prosthesis. The right ventricular outflow tract pressure difference was 13-37 (25.2±4.9) mm Hg in the artificial vascular group and 5-23 (10.5±3.3) mm Hg in the bovine pericardial tube group. No obvious reflux was found at discharge. The patients were followed up for 8 months to 13 years. One patient was lost. During the follow-up period, there were 5 patients of moderate tricuspid regurgitation, 32 patients of moderate intraductal regurgitation, 7 patients of severe right ventricular outflow tract obstruction, 11 patients of moderate obstruction and 25 patients of mild obstruction. There was no late death. Heart function classification of all patients was in New York Heart Association classⅠtoⅡ.ConclusionThe self-made single-valve conduit used to reconstruct right ventricular outflow tract shows good clinical effect. Using bovine pericardium tube has less pressure difference of outflow tract in comparison with artificial blood vessel. Bovine pericardium tube is more recommended for young and low weight patients.
Objective To summarize the treatment experiences of Modified lateral tunnel(LT) Fontan operation on complex congenital heart disease in children and investigate the advantages of this operation. Methods From March 1999 to August 2008, 86 patients with cynosis complex congenital heart disease underwent LT Fontan operation in our hospital. There were 47 male and 39 female aged 1.9-11.5 years with a mean age of 4.7 years and weighed 8.6-52.0 kg with a mean weight of 17.0 kg. There were 33 cases with asplenia syndrome, 17 cases with polysplenia syndrome, 11 cases with tricuspid atresia(TA), 11 cases with double outlet right ventricle(DORV) of atrioventricular discordance, 8 cases with complete transposition of great arteries(D-TGA) complicated with pulmonary stenosis, 5 cases with corrected transposition of great arteries(cTGA) and 1 case with Ebstein’s anomaly. Unilateral superior bidirectional superior cavopulmonary anastomosis(BSCPA), bilateral bidirectional superior cavopulmonary anastomosis and hemiFontan opertion were done before operatipon. The time between two operations was 0.7-7.8 years（3.6±2.9 years）. LT Fontan operation(LT-group, 47cases) and Modified LT Fontan operation(M-LT group, 39cases) were used in operation to drain blood from inferior vena cava to right pulmonary artery. Partly completed secondstage M-LT Fontan operation. Results There were 7 deaths in two groups(9%), 5 in LT group and 2 in M-LT group. There was no statistical significance（χ2=0.865，P=0.448）. In stagemodified LT Fontan operation, there were significantly more cases who had BSCPA operation preoperatively in MLT group than that in LT group. Twentytwo cases had low cardiac output syndrome after operation, 13 cases underwent peritoneal dialysis because of renal dysfunction, and theirurine volume recovered after 2-5 days’ dialysis. There were significantly more cases who had arrhythmia in LT group than that in M-LT group（χ2=8.763，P=0.003）. The time of chest drainage was longer in LT group than that in M-LT group（t=2.970，P=0.003）. The follow-up time was 3 months8 years. No death was found. In M-LT group 33（85%） cases were followed up and in LT group 39（83%）cases were followed up. No severe complication was found. Patients’ activity ability improved significantly. Conclusion The M-LT Fontan operation is an advanced operation to improve the success rate of operation and reduce postoperative complications.
At present, the operative results of complex congenital heart disease are suboptimal which is closely correlated to the understanding of the anatomy and function of complex congenital heart disease, and operative techniques. With the further understanding to pathology and physiology of congenital heart disease, strategies and techniques in well-known operations and complex procedures have developed in recent years. Currently, designing and applying individual operative method in terms of patient’s characteristics of anatomy and physiology is very important trend. This article reviewed the advances of knowledgement and techniques in some representive complex congenital heart disease including complete atrioventricular septal defect, unifocalization for major aortopulmonary collateral arteries, transopsition of the great artery and Fontan type operation.
Objective To summarize the clinical features and results of surgical treatment of complex congenital heart disease(CCHD) in infants, investigate the operative indications and improve the operative effect. Methods From November 1999 to June 2008, 323 infants with CCHD were operated in Wuhan Asia Heart Hospital. There were 202(62.5%) male and 121(37.5%) female aged from 4 days to 36 months. The average age was 18.4 months. The range of weight was 4-15 kg, and the average weight was 9.9 kg. There were 218 cases with tetralogy of fallot(TOF), 41 with double outlet right ventricle(DORV), 12 with total anomalous pulmonary venous drainage（TAPVD）, 8 with complete endocardial cushion defect（TECD）, 15 with coarctation of aorta（CoA）, 2 with aortapulmonary window（AP Window） associated with interrupted aortic arch(IAA) and patent ductus arteriosus (PDA), 2 with persistent truncus arteriosus (PTA), 9 with single ventricle（SV）, 2 with Ebstein’s anomaly, 10 with pulmonary atresia（PA）, 3 with transposition of great arteries（TGA）and 1 with corrected transposition of great arteries（cTGA）. Two hundred and ninetyseven patients underwent I stage correction, 26 underwent palliative operation. All the corrective operations were performed under hypothermic cardiopulmonary bypass（CPB）. Results The cardiopulmonary bypass(CPB) time and aortic cross clamping time were 89±34 min and 48±39 min, respectively. All the patients were followed up by telephone or mail. The follow-up time was 1-72 months. Eight patients（2.5%） died after operation, 7 of them died in the early period of operation(within 1 month). Two patients died of long operation time and CPBdependence, 3 died of ventilatordependence, 1 died of cardiac arrest caused by aspiration following multiple organ dysfunction syndrome（MODS）after resuscitation, and 1 died of continuous hypoxia and cardiac arrest after central shunt operation. There was 1 mediumterm death, which was caused by laryngitis complicated with pulmonary infection. There were 315 survivals（97.5%）. Ninetyfive cases had complications(29.4%), all discharged after symptomatic treatment. The [CM(159mm]improved cardiac function was in gradeⅠ-Ⅱ. The respiratory tract infection reduced and the weight increased significantly. Conclusion Early detection, early diagnosis and early surgical treatment are important for CCHD in infants and the surgical results are satisfactory. The surgical procedure should be chosen according to individual abnormality. Surgeons should pay attention not only to the operation indications and satisfactory correction of the abnormality, but also to the staging operation.
Objective To optimize the surgical strategies and managements of doubleoutlet right ventricle(DORV)with atrioventricular discordance, the anatomic features, the surgical managements and results were reviewed. Methods From September 1990 to August 2004, 7 cases of DORV with atrioventricular discordance received surgical therapy. The age ranged from 3 years to 7 years. Surgical managements included: ventricular septal defect (VSD )repair and homograft connected the morphologic left ventricle with pulmonary artery in 3 cases, double-switch in 2 cases, modified Fontan in 2 cases. Results The early mortality rate was 14.3% (1/7). The death was because by Homograft infection 2 months after repair. Complete atrioventricular block occurred in one case who received permanent pacemaker later. Obstruction of superior vena cava return and pericardial effusion occurred in one Fontan case who was recovered at the time of discharge. The time of ICU stay was shortest in double-switch patients in 6 days postoperation. Conclusion Double-switch operation is the first choice in correction of DORV with atrioventricular discordance, especially in patients with right ventricular disfunction or tricuspidal regurgitation. If the heart can not be corrected because of combined complicated malformations, the strategy of one ventricular repair can be chosen.