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.
Objective To assess clinical results of three different conduit materials （Gore-Tex synthetic graft，bovinejugular vein and autologous pericardium）for palliative right ventricle-to-pulmonary artery （RV-PA） shunt，and explore the correlation between suitable conduit size and patients’ body weight and McGoon ratio. Methods We retrospectively analyzed clinical data of 24 patients with congenital heart diseases who underwent palliative RV-PA shunt in Department of Pediatric Cardiovascular Surgery of Fu Wai Cardiovascular Hospital from July 2010 to July 2012. There were 11 males and 13 females with their age ranging from 60 days to 6 years and body weight of 10.22±7.41 kg. There were 22 patients with pulmonary atresia and ventricular septal defect （PAVSD），1 patient with tetralogy of Fallot （TOF） and 1 patient with doubleoutlet right ventricle （DORV）. Among different conduit materials，autologous pericardium was used for 17 patients，Gore-Texsynthetic graft was used for 5 patients，and bovine jugular vein was used for 2 patients. Conduit size and children’s body weight were analyzed with linear regression，then the equation was corrected with McGoon ratio. Results There was no perioperative death. Postoperative percutaneous saturation （SpO2）of the 24 children was 20.37%±28.33% higher than preoperative SpO2 . Electrocardiogram showed sinus rhythm in all the patients. Twenty-three patients were NYHA classⅡ，and 1 patient was NYHA classⅢ. Postoperative mechanical ventilation time of patients with autologous pericardium were significantly shorter than those of patients with other 2 materials （P=0.017）. Sixteen patients were followed up from 10 months to 2 years after discharge，including 12 patients with autologous pericardium，3 patients with Gore-Tex synthetic graft and 1 patient with bovine jugular vein. During follow-up，McGoon ratio of patients with autologous pericardium，Gore-Tex synthetic graft and bovine jugular vein were 1.98±0.46，1.83±0.33 and 1.68 respectively，all of which weresignificantly higher than preoperative McGoon ratio （P＜0.05）. Six patients underwent radical corrective surgery，including5 patients with autologous pericardium and 1 patient with Gore-Tex synthetic graft. There was no complication directly related to surgery during follow-up. Linear regression was performed to form an equation between suitable conduit size and patients’ body weight：conduit diameter （mm）=0.327×body weight （kg）＋4.599. McGoon ratio，conduit size and equationresult were compared to find a practical choice of conduit size. If McGoon ratio＜0.8，the first integer greater than the equation result could be chosen. If McGoon ratio＞1.2，the first integer less than the equation result could be chosen. If 1.2＞McGoon ratio＞0.8，the first integer either less or greater than the equation result could be chosen. Group analysis showed that patients who recovered better postoperatively were those whose conduit sizes were closer to equation results as well as equation results corrected with McGoon ratio. Conclusion All the 3 materials can be conventionally chosen for RV-PA shunt. Appropriate conduit size can be decided upon patients’ body weight and McGoon ratio for RV-PA shunt.
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.
Objective To assess clinical outcomes of valve-sparing aortic root replacement for patients with aortic root aneurysm.?Methods?From February 2001 to September 2010， sixty patients with aortic root aneurysm underwent valve-sparing aortic root replacement in Fu Wai Hospital. There were 44 male patients and 16 female patients with an average age of 37.2±13.0 years （ranging from 9-64 years）. Fifteen patients had aortic dissection， 10 patients had ascendingaorta aneurysm， 25 patients had Marfan syndrome， and 2 patients had bicuspid aortic valve. Aortic root reconstruction was performed in 53 patients and aortic valve reimplantation was performed in 7 patients. Preoperative and postoperative heart function and degree of aortic insufficiency （AI） were compared.?Results?Valve-sparing aortic root replacement was performed in all patients. There was no in-hospital death or severe postoperative complications in this group. The median duration of mechanical ventilation was 13 hours （ranging from 2 to 1 110 hours） ， while the mean intensive care unit stay was 2.7±2.5 days （ranging from 1 to 18 days） . Postoperative echocardiography showed AI degree was significantly reduced in all the patients. Only three patients had moderate to severe AI， and all the other patients had none or trivial AI. All the patients were followed up for 2-122 （61.5±35.9） months. During follow-up， 4 patients were lost， 9 patients died， and the overall survival rate was 83.9% （47/56）. Two patients underwent aortic valve replacement in the 13 th and 14 th postoperative month respectively because of severe AI. Heart function of 47 patients was significantly improved compared with their preoperative heart function. Thirty-five patients （74.4%）were in New York Heart Association （NYHA） functional classⅠ， and 8 patients （17.0%） were in class II. Forty patients （85.1%）were free from moderate or severe AI.?Conclusion Valve-sparing aortic root replacement has satisfactory long-term outcomes for patients with aortic root aneurysm， and valve-related complication rate is low.
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.
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.
Objective To improve the operative effects of patients who had tetralogy of Fallot with aortopulmonary collateral arteries (TOF-APCAs) and evaluate the clinical effects of staging and onestop hybrid approach for TOFAPCAs. Methods From January 2003 to December 2007, thirty patients with TOF-APCAs had undergone combined therapy of APCAs embolization and complete surgical repair. Fifteen patients had APCAs embolization therapy before or after TOF radical operation(staging hybrid group ); Fifteen had onestop hybrid treatment(onestop hybrid group). Results Angiography revealed that there were 19 APCAs in staging hybrid group, and of which 15(78％) were embolized successfully. Five cases had complications and one died from respiratory circulating failure. The rest all recovered and discharged. And 22 APCAs were found in one-stop hybrid group, eighteen (82％) of them were embolized successfully. Only one case had pulmonary effusion. The time of hospitalization(median 37 d vs. 22 d, P=0.011),ICU staying（median 7.0 d vs. 4.7 d,P=0.029）and endotracheal intubation（median 131 h vs. 19 h,P=0.009） was obviously longer, and the hospitalization expenses（median 64 101 [CM(159mm]yuan vs. 48 021 yuan, P=0.033）were obviously higher in staging hybrid group than that in one-stop hybrid group.And there was no statistical significance in cardiopulmonary bypass time（P=0.126） and aortic clamping time（P=0.174） between two groups. Conclusion In comparison with traditional staging hybrid approach, one-stop hybrid approach can simplify the operative process for patients who have TOFAPCAs, improve the operative successful rate and cut down expenses.
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.
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.