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.
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.
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.
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.
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.
Abstract: Objective To summarize the clinical experience for complex congenital heart disease treated with modified Fontan operation. Methods From November 1996 to May 2005,124 patients (male 83,female 41; including tricuspid atresia, single ventricle, double outlet of right ventricle, malposition of great arteries, pulmonary atresia, corrected transposition of great arteries, hypoplastic rightheart syndrome, etc.) underwent modified Fontan operation at age 7.6±5.5 years. Noncardiopulmonary bypass was used in 19 patients, 105 patients with cardiopulmonary bypass. Right atria-pulmonary artery connection were performed in 17 patients, right atria-ventricular connection were performed in 19 patients, and total cavopulmonary connection (TCPC) were performed in 88 patients. Staged operation were performed in 23 patients. Results The hospital mortality (30 days postoperative) was 13.7% (17/124). The hospital mortality of patients undergone right atria-pulmonary artery connection was 23.5%(4/17), patients undergone right atria-ventricular connection was 15.8%(3/19), patients undergone TCPC was 11.4%(10/88), patients undergone operation with fenestration was 14.6%(6/41), and the patients undergone staged operation was 8.7%(2/23). Low cardiac output syndrome, multiple organ failure, and ventricular fibrillation were the cause of death. Morbidity of complications was 16.9％(21/124) in early period. Complications consisted of pleural effusion, arrhythmia, pericardial effusion and low cardiac output syndrome, etc. Eightynine patients were followed up, followup time was from postoperative 6 months to 65 months. Re-hospitalization rate was 6.5%, and re-operation rate was 0.9%. There were pleural effusion in 3 patients, pericardial effusion in 3 patients, and obstruction of inferior vena cava in 1 patient. All patients recovered. Conclusion Modified Fontan operation is an optimal procedure for functional single ventricle, fenestration seems to decrease postoperative pleural effusions.
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 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 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.