Fontan operation is still a main procedure for treatment of complex congenital heart disease, such as univentricular heart. Fontan procedure has undergone many revisions since its introduction in 1968. The earlyapplied atriumpulmonary connection has been replaced by total cavopulmonary connection. The midterm and late results of both the intraatrial lateral tunnel and extracardiac total cavopulmonary connections were compared and analyzed in this article. Extracardiac conduit is better. The Fontan circulation failure would appear at last because of nopump function of the right ventricle. Once Fontan circulation failure occurred and could not recover by medicine, heart transplantation is mandatory, but the source of donor heart is lacking. The study of mechanical cavopulmonary assist device, to “biventricularize” the univentricular Fontan circulation, has been developed, which is quite promising. Following the development of diagnostic and treatment techniques for fetal heart disease, the treatment procedure of complex congenital heart disease has been broadened in recent years, such as to prevent the severe aortic stenosis from developing into hypoplastic left heart syndrome with fetal cardiac intervention so as to increase the chance of biventricular repair, and to terminate gestation to decrease its birth rate of complex heart abnormalities, which could not be completely repaired to date.
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.
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 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.
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.
Objective To summary the experience of extracardiac conduit total cavopulmonary connection (TCPC) and study the operative indication, design, method, and therapeutic efficacy. Methods 29 patients of extracardiac conduit TCPC were reviewed:the average age was 10 years. Of them, there were 9 cases of tricuspid atresia, 9 double inlet ventricle with left ventricular type, 3 mitral atresia, 3 corrected transposition of the great arteries with anatomically right ventricular hypoplasia and 5 double outlet of right ventricle with left ventricular hypoplasia. All patients underwent cardiopulmonary bypass, 12 cases with heart arrested, and 17 without heart arrested. In them, 20 cases’ superior vena cava were anastomosed directly to the upper margin of right pulmonary artery, 9 cases deviated to the left side of right pulmonary artery to enlarge the stoma. For the inferior vena cava stoma, 22 cases’ anterior walls of right atrium were partially incised, and sutured to the posterior wall, then anastomosed with Gore-Tex blood vessel prostheses and connected to pulmonary trunk, and the other 7 cases’ bottom of right atrium was totally incised, the proximal was closed, and the distal was anastomosised with Gore-Tex blood vessel prostheses and connected to the lower margin of right pulmonary artery, deviated to the right sidedness. Results 5 died in the first 22 cases, and the next 7 cases all survive. All patients were followed up for 3 months to 10 years with no late death. Of them, 12 cases had low cardiac output syndrome, and 11 cases of chylothorax. Conclusions Compared with other types of Fontan operation, the extracardiac conduit TCPC has better long-term effects in older or grown-up children. Nevertheless, strict operative adoption, reasonable operative design, refined procedures, carefully observation and treatment are the key points of improving therapeutic efficacy.
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.
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 study the inflammation response and the biocompatibil ity of valved bovine jugular vein conduit (BJVC) and valved bovine jugular vein patch (VBJV-P) in treating complex congenital heart disease (CHD). Methods From December 2007 to March 2008, 16 patients with complex CHD were treated. Of 16 patients, 6 underwent conjunction right ventricular to pulmonary artery with BJVC and broaden right ventricular outflow tract (RVOT) with VBJV-P (BJVC group), and 10 underwent broaden RVOT with self pericardial patch (control group). In BJVC group, there were 3 males and 3 females, aging (5.6 ± 3.6) years, and including 1 case of type I truncus arteriosus, 1 case of type I truncus arteriosus with ventricular septal defect and patent foramen ovale, 1 case of congenital pulmonary atresia with ventricular septal defect and patent arterial duct, and 3 cases of Fallot’s tetrad. In control group, there were 5 males and 5 females, aging(4.3 ± 3.1) years, all being Fallot’s tetrad. The periphery vein blood of the two groups was collected during operation and after operation, and the levels of cytokine were detected with ELISA method. Meanwhile the cl inical data of the two groups were collected. Results There were no significant differences at levels of TNF-α and IL-6 between BJVC group and control group 1 week after operation (P gt; 0.05), and there was significant difference at level of IL-10 [(25.7 ± 5.0) pg/mL vs (19.5 ± 4.7) pg/ mL, P lt; 0.05]. There were no significant differences at levels of IL-6 and IL-10 within groups both in control group and in BJVC group (P gt; 0.05) between 1 week after operation and the anesthesia inducing period. And there was significant difference at level of TNF-α in BJVC group [(77.0 ± 1.6) pg/mL vs (82.9 ± 1.3) pg/mL, P lt; 0.05] and in control group [(78.6 ± 3.4) pg/mL vs (83.1 ± 1.9) pg/mL, P lt; 0.05] between 1 week after operation and the anesthesia inducing period. There were no statistically significant differences (P gt; 0.05) in leukocyte count and body temperature between BJVC group and control group. The X-ray films showed no abnormal ity in BJVC group and control group before operation and after operation. No hepatic and renal dysfunction occurred in control group; and 2 patients had hepatic dysfunction, which may be caused by antibiotics. Conclusion BJVC has a good biocompatibil ity in treating complexty CHD.
Objective To investigate the surgery experience of modified intra/extracardiac conduit total cavopulmonary connection (TCPC). Methods We retrospectively analyzed clinical data of 47 patients of complex congenital heart disease undergoing intra/extracardiac conduit total cavopulmonary connection in our hospital between January 2008 and December 2015. There were 29 males and 18 females with a median age of 7 years (range 4 to 9 years) and median body weight of 22 kg (range 14 to 38 kg). The heart echocardiography and cardiac imaging confirmed diagnosis suitable for TCPC surgery. Results There was no early death in the whole group. The mean pulmonary arterial pressure was 16 (12–20) mm Hg and the ventilation time was 14 (7–97) h. The main complications were intractable pleural effusion in 7 patients, low cardiac output syndrome in 3 patients, repeated supraventricular tachycardia in 1 patient. All the patients recovered after treatment. At the end of discharge, the percutaneous oxygen saturation was 85%–96% (92.6%±3.3%). The echocardiography showed the conduit pressure was 0–2 mm Hg. Patients were followed up for 1 to 7 years. Three patients were lost. One patient had intestinal nutrition loss, receving repeated pleural effusion, the treatment was ineffective, died after 4 years. Four patients of repeated pleural effusion improved after treatment. One patient repeated attacks supraventricular tachycardia within 1 year, controlled by amiodaronum, already stopped about 28 months. No recurrence occurred. All survivors were in New York Heart Association (NYHA) functional class Ⅰ or Ⅱ, with good activity tolerance. Conclusion The modified intra/extracardiac conduit TCPC combines the advantages of both the lateral tunnel and the extracardiac conduit. The operation is simple, used in the treatment of complex congenital heart disease. The short-term and mid-term results are encouraging.