Pulmonary atresia (PA) with ventricluar septal defect(VSD) is a complex congenital heart disease. The lack of knowledge on embryology, anatomy and pathophysiology of this disease with or without heterogenetic major aortopulmonary collateral arteries(MAPCA)leads to chaos of its surgical classification and management as well as poor results of surgical management. The operative technique in this field has been advancing a lot since the innovation of unifocalization. The surgical result is getting better and better, boosted by the updating of the principle and knowledge, but there is still a large room for progress. We reviewed the literatures on classification,surgical strategy, procedures and operationrelated issues and put them together here to delineate its past, present and future.
Abstract： Objective To analyze risk factors for perioperative mortality in the arterial switch operation (ASO), in order to provide better operation and decrease the mortality rate. Methods We enrolled 208 ASO patients including 157 males and 51 females at Fu Wai Hospital between January 1, 2001 and December 31, 2007. The age ranged from 6 h to 17 years with the median age of 90 d and the weight ranged from 3 kg to 43 kg with the median weight of 5 kg. Among the patients, 127 had transposition of great artery (TGA) with ventricular septal defect (VSD), and 81 patients had TGA with intact ventricular septum (IVS) or with the diameter of VSD smaller than 5 mm. Coronary anatomy was normal (1LCX2R) in 151 patients and abnormal in the rest including 15 patients with single coronary artery, 6 with intramural and 36 with inverse coronary artery. Preoperative, perioperative and postoperative clinical data of all patients were collected to establish a database which was then analyzed by univariate analysis and multivariate logistic regression analysis to find out the risk factors formortality in ASO. Results There were 24 perioperative deaths (11.54%) in which 12 died of postoperative infection with multiple organ failure (MOF), 10 died of low cardiac output syndrome, 1 died of pulmonary hypertension, and 1 died of cerebral complications. Among them, 20 patients (18.30%) died in early years from 2001 to 2005, while only 4 (4.00%) died in the time period from 2006 to 2007, which was a significant decrease compared with the former period (Plt;0.05). The univariate analysis revealed that cardiopulmonary bypass (CPB) time was significantly longer in the death group than in the survival group（236±93 min vs. 198±50 min, P=0.002）, and occurrence of major coronary events (33.3% vs. 2.2%, P=0.000) and unusual coronary artery patterns（33.3% vs. 6.5%,P=0.000） were much more in the death group than in the survival group. Multivariate logistic regression analysis showed that early year of [CM(159mm]operation (OR=7.463, P=0.003), unusual coronary artery patterns (OR=6.303,P=0.005) and occurrence of majorcoronary events (OR=17.312, P=0.000) were independent predictors for perioperative mortality. Conclusion The ASO can be performed with low perioperative mortality in our hospital currently. Occurrence of major coronary events, unusual coronary artery patterns and year of surgery before 2006 are independent predictors for perioperative mortality.
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.
Abstract： Objective To summarize early clinical result of total cavopulmonary connection, and analyze the risk factors contributing to prolonged postoperative recovery. Methods Between February 2009 and August 2010, 58 patients with functional univentricular complex congenital heart disease received total cavopulmonary connection in Beijing Fu Wai Hospital. All of them were diagnosed by echocardiogram and angiography including 26 patients with single ventricle, 10 patients with tricuspid atresia, 4 patients with pulmonary artery atresia, 5 patients with double outlet rightventricle, 1 patient with transposition of great arteries, and 12 patients with corrected transposition of the great arteries.Fifty seven patients underwent extracardiac conduit total cavopulmonary connection, and only one patient underwent total cavopulmonary connection with an intracardiac lateral tunnel. According to their postoperative pleural drainage volume and duration, these 58 patients were divided into a large pleural drainage volume group (17 patients with 10 males and 7 females, mean age of 8.61±6.73 years）that included patients with large volume and long duration of pleural drainage, and a little pleural drainage volume group (41 patients with 15 males and 26 females, mean age of 7.21±4.24 years) . A univariable analysis was conducted to compare the risk factors that effected recovery result between the two groups. Results There was no death in hospital period. The average length of hospital stay was 12.30±9.80 d . Average drainage time (18.00±5.50 d versus 5.00±2.20 d , t= -1.967, P ＜ 0.05）, drainage volume (12.30±2.60 ml/(kg·d) versus 2.80±1.70 ml/(kg·d), t=-3.221, P ＜ 0.05), and hospital stay (20.10±7.20 d versus 7.20±1.10 d, t=-2.003, P ＜ 0.05） of the large pleural drainage volume group were significantly larger or longer than those of the little pleural drainage volume group. Univariate analysis showed that preoperative pulmonary pressure measured by catheter in the large pleural drainage volume group was significantly higher than that in the little pleural drainage volume group (17.42±5.34 mm Hg versus 13.91±5.22 mm Hg，t=-2.073，P ＜ 0.05). Conclusions The mortality and major morbidities after total cavopulmonary connection are low in the current era. Preoperative high pulmonary pressure is a risk factor for large amount of pleural drainage and prolonged recovery.
Abstract： Objective To analyze the mid-term outcomes after correction of type Ⅰ and type Ⅱ persistent truncus arteriosus in all patients operated in our institution over the past 5 years. Methods Between May 2006 and October 2010, 17 patients, mean age 4.7（ 0.7-19.0）years, underwent repair of truncus arteriosus（ type Ⅰ in 13 and type Ⅱ in 4） in Fu Wai Cardiovascular Hospital. Some other concomitant cardiovascular malformations included truncal valve regurgitation, partial anomalous pulmonary venous connection, mitral regurgitation and atrial septal defect. Their average pulmonary vascular resistance was （4.4±2.2） Wood units detected by cardiac catheterization before operation. Repair with reconstruction of the right ventricular to pulmonary artery continuity was performed using a valved conduit in all 17 patients （aortic homografts in 3, pulmonary homografts in 2, and bovine jugular vein in 12 patients）. Survivors were followed up for assessment of residual heart lesions. Results The early mortality was 5.8% （1/17）. The mean cardiopulmonary bypass time was （165±52） min, mean aortic cross-clamping time was （114±29） min, and mean postoperative ventilation time was （106±148） h. Two patients had pleural effusion after surgery, 2 patients underwent tracheostomy, and other patients recovered uneventfully. The surviving 16 patients were followed up for 0.6-5.0 years. All patients were alive with their original conduit during follow-up. No patient required re-operation for conduit dysfunction after correction. Conclusion Truncus arteriosus remains a challenging congenital heart disease. For patients with type Ⅰ and type Ⅱ persistent truncus arteriosus who have missed their best age for correction, cardiac catheterization should be routinely examined, and the operation should be performed if the pulmonary vascular resistance is under 8 Wood units before operation. Although the short- and mid-term results of surgery are good, more observations are needed to assess its long-term effect.
Abstract: Objective To investigate the safety and feasibility of fast track (FT) treatment in young children with atrioventricular septal defect (CAVSD) and pulmonary artery hypertension (PAH) following surgical repair. Methods A total of 51 young children patients including 24 boys and 27 girls with age at 12.5±8.9 months from 4 to 36 months, underwent CAVSD repair in the pediatric surgery department of Fu Wai Hospital from January 2006 to March 2009. Among them, 21 patients were administered FT management. PICU length of stay and the rate of reintubation were analyzed retrospectively and the decrease of pulmonary artery pressure (PAP) after operation was also measured. Results Twentyone patients under FT treatment were extubated within 8 hours after operation. The mean pulmonary artery pressure(MPAP) decreased significantly after surgery (39.59 mm Hg vs.24.50 mm Hg,t=5514,Plt;0.05). PICU length of stay was 2.05±0.87 d (18 h-3 d). One patient was reintubated due to lung infection, which had nothing to do with the FT treatment. During the followup which lasted for 3 to 6 months, 21 patients had good heart function with no reoperation or death. Conclusion FT treatment is safe and feasible to some CAVSD patients associated with PAH, and shorter PICU length of stay can be achieved. The validation of FT model for the CAVSD patients with severe PAH needs research with large sample.
To investigate the anatomical classification, diagnosis, indications, surgical methods, surgical techniques, intraoperative management, postoperative management and surgical efficacy of mitral valve malformation. Using the internationally used Delphi procedure, PubMed, Medline, The Cochrane Library, Wanfang and other databases were searched to review domestic and foreign literatures on congenital mitral valve deformity from January 1940 to February 2020, and select the ones of higher quality as evidence. In addition, with regard to the multiple disputes in the diagnosis and treatment of congenital mitral valve, experts from pediatric cardiac surgery and related disciplines have been convened for discussion for many times, and finally the following consensus has been formed to guide the clinical treatment and provide theoretical and technical guidance for the surgical treatment of congenital mitral valve malformation in China.
Abstract: Objective To investigate the clinical application of a novel modified aortic and pulmonic translocation in surgical repair of transposition of great arteries(TGA) with ventricular septal defect(VSD) and left ventricular outflow tract obstruction(LVOTO). Methods Five patients received surgical repair of the TGA with VSD and LVOTO at our heart center. The surgical technique used was a modification of the Nikaidoh procedure by which the native pulmonary root was preserved and translocated to reconstruct the right ventricular outflow tract. Two patients with atrioventricular discordance required a Senning procedure. Results All patients survived the operation and were discharged from the hospital. There were no major complications. At a median follow-up of 5.40 months, the echocardiography demonstrated normal ventricular function in all patients. No residual aortic stenosis or insufficiency was found in all the patients. Two patients had mild pulmonary insufficiency. Conclusions The novel modification of the Nikaidoh procedure may have excellent early results with minimal postoperative pulmonary insufficiency. The procedure may also allow growth of the pulmonary root and therefore decrease the need for reoperation. However, this has to be further investigated and long-term follow-up studies are warranted.