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.
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.
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 The earlyapplied atriumpulmonary connection has been replaced by total cavopulmonary connection.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 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 assess the mid- and long-term outcomes of right ventricular outflow tract reconstruction for children with congenital heart disease. Methods We retrospectively analyzed the clinical data of 3 138 children with complex congenital heart disease in right heart system admitted to our hospital from January 2007 to January 2017. There were 1 660 males and 1 478 females. The age at surgery was 9 days to 84 months, and the body weight was 2.2 to 28.6 kg. Pulmonary patch enlargement was performed in 2 335 patients (1 477 patients of valve-sparing repair and 858 patients of transannular repair); autologous tissue (direct anastomosis, left auricle or pericardial conduit) was used to connect with right ventricle in 289 patients; extracardiac conduits were used for reconstruction in 514 patients. Results There were 181 in-hospital deaths with a mortality of 5.8%. The early postoperative causes of death were low cardiac output syndrome (LCOS), severe pulmonary hypertension and right heart failure. Fifteen patients died of cardiac insufficiency or sudden death during follow-up (6–27 months postoperatively). The echocardiography showed 408 patients with right ventricular outflow tract obsturction (RVOTO), 340 patients with pulmonary trunk or branches stenosis, 609 with pulmonary regurgitation (morderate or severe). 12.6% (394/3 138) of patients underwent reintervention or reoperation with 39 deaths. About 92.4% of patients exhibited an improvement of New York Heart Association (NYHA) functional class from Ⅲ or Ⅳ preoperatively to Ⅰ or Ⅱ at follow-up. Conclusion The anatomical structure of right ventricular outflow tract is complicated and various, and each operation method has different strengths and favorable outcomes. The operation should be individually designed according to pathological types, anatomical features, clinical symptoms and operation conditions.
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.
Objective Complex congenital heart defects are sometimes treated by Fontan palliation for various reasons. However, the middle- and long-term prognosis of single-ventricle repair is worse than that of two-ventricle repair. In this study we reported the results of biventricular conversion in these challenging patients initially palliated towards single-ventricle repair. Methods Eight patients underwent biventricular repair conversion from prior bidirectional Glenn shunt palliation in our hospital between October 2013 and March 2016. The median age in bidirectional Glenn shunt was 2.6 years (range, 1.0 to 5.9 years) and in biventricular repair conversion was 6.6 years (range, 4.5 to 11.1 years). Three patients suffered complete transposition of great arteries combined with ventricular septal defect and left ventricular outflow tract obstruction, three double outlet right ventricle and non-committed ventricular septal defect combined with pulmonary stenosis or atresia, one double outlet right ventricle combined with complete ativentricular septal defect and pulmonary stenosis and one Tetralogy of Fallot. Results Bidirectional Glenn shunt was taken down and superior vena cava was reconnected to the right atrium in all patients. Mean cardiopulmonary bypass and aortic cross-clamp time was 275.6±107.1 min and 165.9±63.6 min, respectively. Mean length of hospital stay and ICU stay were 33.6±23.0 d and 20.3±21.0 d, respectively. At a mean follow-up of 1.4±0.7 years, there was no mortality and reoperation. No patients presented with sinoatrial node dysfunction and superior vena cava anastomotic stenosis. According to the New York Heart Association (NYHA) Functional Classification, all patients were classified asⅠ-Ⅱ. Conclusion Biventricular repair conversion can be safely performed with favorable mortality and morbidity in specific patients palliated towards single-ventricle repair. Further follow-up is needed to investigate the long-term outcomes.