Though the 10 year survival rate of Fontan procedure has increased from 69% in 1990 to 85% in 2006. Recently researchers have strived to improve the longterm outcome of this procedure and have made great achievements. They found that among the ten items of standard, the limits associated with age, anomalous systemic or pulmonary venous drainage, sinus rhythm, ratio of pulmonary artery/aorta should be relaxed. New operative designs such as extracardiac lateral tunnel Fontan and new types of HemiFontan procedure using shortened extracorporeal circulation may help to decrease the postoperative complications. Standardized postoperative therapeutic plan after Fontan procedure may decrease the duration of chest drainage，shorten the inpatient days and also avoid longterm treatment. However, longterm followup after Fontan procedure showed that the incidence rate and risk of longterm postoperative complications were still high. This article was directed to review the researches about Fontan on operative indications, optimizing the operative strategies, modifying the postoperative monitoring techniques and longterm follow-up.
Objective To summarize the treatment experiences of Modified lateral tunnel(LT) Fontan operation on complex congenital heart disease in children and investigate the advantages of this operation. Methods From March 1999 to August 2008, 86 patients with cynosis complex congenital heart disease underwent LT Fontan operation in our hospital. There were 47 male and 39 female aged 1.9-11.5 years with a mean age of 4.7 years and weighed 8.6-52.0 kg with a mean weight of 17.0 kg. There were 33 cases with asplenia syndrome, 17 cases with polysplenia syndrome, 11 cases with tricuspid atresia(TA), 11 cases with double outlet right ventricle(DORV) of atrioventricular discordance, 8 cases with complete transposition of great arteries(D-TGA) complicated with pulmonary stenosis, 5 cases with corrected transposition of great arteries(cTGA) and 1 case with Ebstein’s anomaly. Unilateral superior bidirectional superior cavopulmonary anastomosis(BSCPA), bilateral bidirectional superior cavopulmonary anastomosis and hemiFontan opertion were done before operatipon. The time between two operations was 0.7-7.8 years（3.6±2.9 years）. LT Fontan operation(LT-group, 47cases) and Modified LT Fontan operation(M-LT group, 39cases) were used in operation to drain blood from inferior vena cava to right pulmonary artery. Partly completed secondstage M-LT Fontan operation. Results There were 7 deaths in two groups(9%), 5 in LT group and 2 in M-LT group. There was no statistical significance（χ2=0.865，P=0.448）. In stagemodified LT Fontan operation, there were significantly more cases who had BSCPA operation preoperatively in MLT group than that in LT group. Twentytwo cases had low cardiac output syndrome after operation, 13 cases underwent peritoneal dialysis because of renal dysfunction, and theirurine volume recovered after 2-5 days’ dialysis. There were significantly more cases who had arrhythmia in LT group than that in M-LT group（χ2=8.763，P=0.003）. The time of chest drainage was longer in LT group than that in M-LT group（t=2.970，P=0.003）. The follow-up time was 3 months8 years. No death was found. In M-LT group 33（85%） cases were followed up and in LT group 39（83%）cases were followed up. No severe complication was found. Patients’ activity ability improved significantly. Conclusion The M-LT Fontan operation is an advanced operation to improve the success rate of operation and reduce postoperative complications.
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 report the surgical treatment for double outlet of ventricle with atrioventricular discordance. Methods 11 patients aged from 3 to 25 years underwent surgical treatment for double outlet of ventricle with atrioventricular discordance. 10 of them were double outlet right ventricle and the other one was double outlet left ventricle. The surgical treatment included biventricular repair (n=9) and single ventricular repair (n=2). The biventricular repair was performed by intraventricular patch repair and extracardiac valved conduit or homograft valved conduit. The single ventricular repair included bidirected Glenn operation and total cavopulmonary connect. Results There were two early deaths in biventricular repair and no death in single ventricular repair. The cause of death was severe low cardiac output syndrome. Four survivors in biventricular repair were followed up from 1 to 11.5 years, three of them were in NYHA class Ⅰ or Ⅱ and one in NYHA class Ⅲ. Conclusions Double outlet of ventricle with atrioventricular discordance can be treated by biventricular repair or single ventricular repair according to the development of ventricle and pulmonary arteries. Closure of ventricular septal defect , no obstruction from ventricle to great artery and no harm of conduction system are the keys of conventional management to achieve good results.
At present, the operative results of complex congenital heart disease are suboptimal which is closely correlated to the understanding of the anatomy and function of complex congenital heart disease, and operative techniques. With the further understanding to pathology and physiology of congenital heart disease, strategies and techniques in well-known operations and complex procedures have developed in recent years. Currently, designing and applying individual operative method in terms of patient’s characteristics of anatomy and physiology is very important trend. This article reviewed the advances of knowledgement and techniques in some representive complex congenital heart disease including complete atrioventricular septal defect, unifocalization for major aortopulmonary collateral arteries, transopsition of the great artery and Fontan type operation.
ObjectiveTo analyze risk factors contributing to prolonged postoperative recovery after Fontan operation. MethodsClinical data of 60 patients undergoing Fontan operation between January 2012 and June 2013 in Beijing Fu Wai Hospital were retrospectively analyzed, including their demographic data, preoperative angiography and echocardiogram, and preoperative, intraoperative and postoperative hemodynamic data and blood test results. According to different length of hospital stay (LOS), all the 60 patients were divided into 2 groups. In the normal recovery group, there were 45 patients including 33 males and 12 females with their age of 5.7±1.7 years, whose LOS was shorter than 32.5 days. In the prolonged recovery group, there were 15 patients including 10 males and 5 females with their age of 4.9±1.6 years, whose LOS was longer than 32.5 days (over 75th percentile of LOS). LOS of the 60 patients ranged from 12 to 53 days, and 75th percentile of LOS was 32.5 days. Clinical results were compared between the 2 groups, and risk factors for prolonged postoperative recovery were analyzed. ResultsPreoperatively, their oxygen saturation by pulse oximetry was 80.5%±7.4%, ejection fraction (EF)was 64.1%±6.6%, Nakata index was 370.6±234.2 mm2/m2, Mcgoon ratio was 2.2±0.7, and pulmonary arterial pressure (PAP)was 12.4±4.0 mm Hg. Twenty-seven patients (45.0%)received Glenn procedure before Fontan operation at the age of 0.9-4.0 years, and the duration from Glenn procedure to Fontan operation was 1.0-5.1 years. Two patients (3.3%)died after Fontan operation. Cardiopulmonary bypass time of 55 patients who received Fontan operation under parallel circulation was 112.0±52.4 minutes. Aortic cross-clamping time of 5 patients who received concomitant repair of intracardiac anomalies under circulatory arrest was 44.8±9.2 minutes. The duration of mechanical ventilation was 18.8±6.4 hours, and ICU stay was 5.1±2.1 days. Univariate analysis showed that risk factors for prolonged postoperative recovery included higher preoperative PAP (P < 0.05), lower preoperative EF (P < 0.05), right ventricle as functional single ventricle (P < 0.05), previous Glenn procedure history (P < 0.05), concomitant total anomalous venous connection (P < 0.05), higher postoperative lactate level (P < 0.05), higher postoperative central venous pressure (P < 0.05), the need for greater volume of fluid resuscitation during the first 24 hours postoperatively (P < 0.05), long duration of chest drainage (P < 0.05)and postoperative infection (P < 0.05). ConclusionShort-term clinical results of Fontan operation for the treatment of functional single ventricle are satisfactory. Careful assessment and appropriate management of risk factors are helpful to improve postoperative recovery after Fontan operation.
Objective To explore the effect of pulmonary arterial hypertension on the children with functional single ventricle in the early period after Fontan operation. Methods Forty-three children with pulmonary arterial hypertension after Fontan operation were enrolled in our department between January 2015 and December 2016. There were 24 males and 19 females at a median age of 4.3 years ranging from 2.5 to 4.8 years. The pulmonary arterial pressure was evaluated by cardiac catheterization. There were 23 children diagnosed without pulmonary hypertension (a non-PAH group) including 16 males and 7 females, while 20 patients were diagnosed with pulmonary hypertension (a PAH group) including 8 males and 12 females. Postoperative parameters related to outcomes were compared between the two groups. Results There was no death in the non-PAH group, but the mortality of children in the PAH group was 20.0% (4/20, χ2=5.34, P=0.02). The central venous pressure (t=–2.50, P=0.02), N-terminal prohormone of brain natriuretic peptide (NT-proBNP, Z=–3.50, P<0.01), peritoneal dialysis rate (χ2=5.40, P=0.02), incidence of arrhythmia (χ2=4.40, P=0.03) in the PAH group were significantly higher than those of the non-PAH group. The early postoperative utilization rate of pulmonary vascular targeting agents in the PAH group was significantly higher than that in the non-PAH group (χ2=6.30, P=0.04). Conclusion Pulmonary arterial hypertension is one of the most important factors which influence the early postoperative prognosis of children with functional single ventricle after Fontan operation.
ObjectiveTo investigate the prognosis and risk factors of mild to moderate or moderate atrioventricular valve regurgitation (AVVR) after Fontan operation.MethodsA total of 34 patients with mild to moderate or moderate AVVR who accepted Fontan operation and atrioventricular valve (AVV) repair between 2004 and 2018 in our center were selected as an AVV repair group. The patients in the same period were matched as a control group by the ratio of 1 : 1-2. Finally 99 patients were included into this study, including 64 males and 35 females, with an average age of 63.4±36.3 months and weight of 17.3±6.7 kg. Grades of AVVR decreased more than 1 was defined as significant improvement. Endpoints of the study were death, Fontan takedown, AVV replacement. Risk factors including Fontan procedures, AVV repair procedures, cardiac anatomy were analyzed.ResultsPatients were followed up for 1.5 (0.3-4.0) years. Overall mortality was 15.2%. Most (82.4%) of AVV repair group accepted single AVV repair procedure while partial annuloplasty was the most common (52.9%). With the extension of follow-up, the degree of AVVR in the whole group showed a gradually increasing trend (r=0.352, P=0.000). Mild to moderate AVVR improved spontaneously after Fontan operation, while moderate AVVR did not. AVV repair could improve the degree of AVVR after moderate regurgitation, without increasing the surgical mortality, and regurgitation significantly decreased in 8.8% patients. AVV repair was not effective for mild to moderate AVVR and would increase surgical mortality. ConclusionAVV function shows a gradual downward trend after Fontan operation. AVV repair is effective for moderate AVVR, does not increase mortality, but the degree of improvement is limited. AVV repair is not effective for mild to moderate AVVR and increases surgical mortality.
Objective To compare the early and mid-term results between Fontan operation and anatomic correction for congenitally corrected transposition of the great arteries (ccTGA). Methods The clinical data of 53 patients with ccTGA who underwent anatomic correction and Fontan operation from January 2009 to September 2021 in our hospital were reviewed, including 41 males and 12 females with a mean age of 55.02 (3-168) months. They were divided into an anatomic correction group (16 patients) and a Fontan operation group (37 patients) according to the operation. The hospitalization mortality, survival rate, postoperative complications, and free rate from re-intervention between the two groups were compared. Another 180 healthy children were recruited as a control group, and 14 children were matched with the propensity score matching method as a Fontan control group. The results of cardiopulmonary exercise testing (CPET) between the Fontan operation group and the Fontan control group were compared. Results There were 2 (12.5%) early deaths and 3 (18.8%) early re-intervention in the anatomic correction group, while 1 death and 2 re-intervention in the Fontan operation group. In addition, there were 9 patients (56.3%) in the anatomic correction group and 6 (16.2%) patients in the Fontan operation group suffering from arrhythmia after operation, respectively. Compared with the anatomic correction group, cardiopulmonary bypass time, aortic cross-clamping time, intubation time and ICU stay were significantly shortened in the Fontan operation group (P<0.05). CPET results showed that, percent predicted max VO2 in the Fontan operation group was lower than that in the Fontan control group (0.84±0.11 vs. 0.99±0.12, P＜0.05). The patients were followed up for 0.5-126.0 months. Two patients were lost in the Fontan operation group. There was no death and 1 re-intervention in the anatomic correction group, while no death or re-intervention in the Fontan operation group. The 1-year, 5-year and 10-year transplant-free survival rate of the anatomic correction group and the Fontan operation group was 87.5%, 87.5%, 87.5% and 97.3%, 97.3%, 97.3%, respectively (P＞0.05). The 48 patients were classified as grade Ⅰ-Ⅱ in cardiac function in the last follow-up. Conclusion There is no statistical difference in the transplant-free survival rate between the anatomic correction and the Fontan operation group. The postoperative complications in the Fontan operation group are decreased than those in the anatomic correction group. The Fontan operation is also a good choice, even though the patients with ccTGA meet the condition of the procedure of anatomic correction.
From June 2002 to December 2023, there were 5 patients with criss-cross admitted to the General Hospital of the Northern Theater Command, including 3 males and 2 females, aged 18 months to 25 years, and weighing 13-49 kg. There were 5 patients of atrioventricular position, 3 patients of right ventricular loop, 2 patients of left ventricular loop, 3 patients of normal atrioventricular connection, and 2 patients of inconsistent connection. Combined intracardiac malformations: 1 patient of simple ventricular septal defect combined with pulmonary hypertension, 1 patient of corrected transposition of the great arteries combined with ventricular septal defect, atrial septal defect, and pulmonary artery stenosis, 1 patient of corrected transposition of the great arteries combined with ventricular septal defect, atrial septal defect, and left atrioventricular valve insufficiency, and 2 patients of right ventricular double outlet combined with ventricular septal defect and pulmonary artery stenosis. The surgical methods included 2 patients of intracardiac anatomical correction, 1 patient of bidirectional vena cava pulmonary artery anastomosis, and 2 patients of total extracardiac ductal cava pulmonary artery anastomosis. All 5 patients were discharged smoothly.