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.
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.
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.
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 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.
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.
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.
Subvalvar aortic stenosis (SAS) refers to a type of disease in which the stenosis of the left ventricular outflow tract is below the aortic valve. It is a disease spectrum that includes multiple diseases ranging from the simple to the complex. This disease has its own characteristics according to different types. The diagnostic criteria and treatment strategies are basically unified, but there are still some disputes about the surgical treatment. Based on the evidence-based data and expert consensus, this paper makes a comprehensive summary and recommendations on the clinical classification, diagnostic criteria and surgical treatment strategies of SAS, which is more suitable for the clinical characteristics of SAS patients in China and more clinically operable.
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.