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.
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.
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.
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 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 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 Tho evaluate the outcomes of early percutaneous occlusion of these residual major aortopulmonary collateral arteries after heart surgery. Methods This was a retrospective review of children undergoing early percutaneous embolization of major aortopulmonary collateral arteries after cardiac surgery. From January 2013 to February 2017, 52 consecutive patients with postoperative residual major aortopulmonary collateral arteries were treated with percutaneous embolization (38 males, 14 females; median age of 10.0 months, interquartile range 14.0 months; median weight 8.6 kg, interquartile range 4.4 kg). Fifty-one patients were cyanotic and 1 patient was acyanotic. Forty-nine patients underwent corrective surgery and 3 patients underwent B-T shunt. Results Typical symptoms and signs of major aortopulmonary collateral arteries included: elevated left atrial pressure; focal lung infiltration, pink or blood-stained frothy sputum. The median time interval from cardiac surgery to percutaneous occlusion of major aortopulmonary collateral arteries was 5 (9) d, median duration of mechanic ventilation support since occlusion was 72 (159) h, mechanic ventilation support was 239 (480) h and median duration of intensive care unit was 19 (29) d. There was no death in this group. Conclusion Angiocardiography could be able to demonstrate the existence of postoperative major aortopulmonary collateral arteries. The early percutaneous occlusion appears to be simple, safe and effective.