In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival rate. However, the resulting Fontan physiology is associated with high morbidity. Despite large improvements in outcomes of contemporary Fontan patients, a large burden of disease exists in this patient population. In face of the imbalance of medical resources in China and different understanding of Fontan treatment in various regions, there is no consensus on the current status of Fontan strategy, surgical technique and perioperative treatment. By reviewing large amounts of literature, assessing survival rate and risk factors for mortality and complications of the Fontan circulation, knowledge sharing from tens of experts, we achieved a consensus on many aspects of palliation of Fontan. We hope this consensus will help Chinese colleagues further continue their efforts to improve surgical outcomes of the univentricular heart patients.
Tetralogy of Fallot is the most common cyanotic congenital heart disease. The pathological anatomy changes include ventricular septal defect, right ventricular outflow tract stenosis, aortic stradding and right ventricular hypertrophy. At present, the diagnostic criteria and treatment strategies of this disease are basically unified. However, there are controversies about the timing and method of surgical treatment. Based on the evidence-based information provided in the literature and the opinions of domestic experts of China, we formulate a consensus of Chinese experts to further standardize the surgical treatment of tetralogy of Fallot.
Coarctation of the aorta and interrupted aortic arch are congenital anomalies affecting the aortic arch. Because of the poor natural prognosis, many patients will need early surgical repair or even emergency surgery. With the improvement of the surgical techniques, cardiopulmonary bypass techniques and perioperative intensive care, surgical mortality has now dramatically decreased. However, aortic arch restenosis, left ventricular outflow tract obstruction and long-term hypertension are problems that may be still encountered during the follow-up period. By reviewing large amounts of literature and discussing among experts, we achieved a consensus on many aspects of the management strategy. We hope this consensus will help Chinese colleagues further improve the overall surgical outcomes of coarctation of aorta and interrupted aortic arch.
Congenital coronary artery anomalies include various congenital anatomic anomalies of coronary artery. Severe congenital coronary artery anomalies usually result in ventricular dysfunction, myocardium ischemia, and even sudden death. Hence, understanding the coronary artery anomalies is critically important for the related surgeons to make reasonable strategies. This consensus is based on current literature and opinions of Chinese experts. And we mainly discuss the anatomy, clinical manifestation, diagnosis and treatment of important congenital coronary artery anomalies in clinics including anomalous aortic origin of a coronary artery, anomalous origin of the coronary artery from the pulmonary artery and coronary fistula.
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.
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.