ObjectiveTo analyze the outcomes of complicated congenital heart diseases (CCHD) patients accepting multiple (>2) re-sternotomy operations.MethodsWe retrospectively analyzed the clinical data of 146 patients undergoing multiple cardiac re-sternotomy operations between 2015 and 2019 in our center. There were 95 males and 51 females with an age of 4.3 (3.1-6.8) years and a weight of 15.3 (13.4-19.0) kg at last operation.ResultsThe top three cardiac malformations were pulmonary atresia (n=51, 34.9%), double outflow of right ventricle (n=36, 24.7%) and functional single ventricle (n=36, 24.7%). A total of 457 sternotomy procedures were performed, with 129 (88.3%) patients undergoing three times of operations and 17 (11.7%) patients undergoing more than three times. Fifty-two (35.6%) patients received bi-ventricular repair, 63 (43.1%) patients received Fontan-type procedures, and 31 (21.2%) patients underwent palliative procedures. Ten (6.8%) patients experienced major accidents during sternotomy, including 7 (4.8%) patients of urgent femoral artery and venous bypass. Eleven (7.5%) patients died with 10 (6.8%) deaths before discharge. The follow-up time was 20.0 (5.8-40.1) months, and 1 patient died during the follow-up. The number of operations was an independent risk factor for the death after operation.ConclusionSeries operations of Fontan in functional single ventricle, repeated stenosis of pulmonary artery or conduit of right ventricular outflow tract post bi-ventricular repair are the major causes for the reoperation. Multiple operations are a huge challenge for CCHD treatment, which should be avoided.
A 12-year-old girl with double outlet of right ventricle, complete atrioventricular septal defect, pulmonary valve stenosis, single atrium, inferior vena cava-hemiazygos vein drainage and left isomerism, who had undergone surgical left superior vena cava-pulmonary artery connection was referred to our hospital. Echocardiography revealed thickening, crimping and malalignment of common atrioventricular valve, moderate-to-severe regurgitation mainly from the left side and the main pulmonary artery was poorly developed. We preserved the autologous valve and used the 31# mechanical mitral valve which was sutured to mitral annulus. After the main pulmonary artery was transected, proximal end was sutured and a 20# Gore-tex artificial vessel was anastomosed to the distal end of the main pulmonary artery. We excised hepatic vein from the right atrium which was then anastomosed to another artificial vessel. Postoperative oxygen saturation was 95%-100%. Echocardiography and CT showed that the function of mechanical valve and heart was good before discharge. Common atrioventricular valvuloplasty is the preferred choice of the management of impaired valve. However, when dysfunction of valve is too severe and valvuloplasty is more likely to fail, the risk of barely performing a valvuloplasty will increase dramatically. On this condition, the mechanical valve replacement should be performed.
Objective To investigate somatic growth of children undergoing stageⅡ Fontan procedure. Methods Clinical data of 152 children who underwent stageⅡ Fontan procedure in Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine between January 2012 and December 2015 were reviewed. There were 84 males and 68 females at age of 4-16 years. The weight and height of those children in each operative stage were assessed, and WHO Z-score was obtained to describe the pattern of growth of the children. Results Mean weight for age Z-score (WAZ) and height for age Z-score (HAZ) were –0.73 and 0.06 at birth, –1.27±1.37 and –1.27±1.72 before Glenn procedure, –0.47±1.08 and –0.69±1.17 before Fontan procedure, –0.42±1.18 and –0.39±1.48 at follow-up, respectively. A significant decline (P<0.05) in WAZ and HAZ was found before Glenn procedure. After the Glenn procedure, there was significant catch-up growth (P<0.05). The WAZ was stabilized after Fontan procedure, while the HAZ was reversed (P<0.05) continually. Conclusion The somatic growth is impaired in children with stageⅡ Fontan procedure. There is a trough of growth before Glenn procedure. Although catch-up growth occurs after Glenn procedure, low body weight and growth retardation remain at follow-up. Effective interventions such as more intensive nutritional strategies before Glenn procedure should be targeted at those children in the future, so as to improve their growth.
Fontan operation is still a main procedure for treatment of complex congenital heart disease, such as univentricular heart. Fontan procedure has undergone many revisions since its introduction in 1968. The earlyapplied atriumpulmonary connection has been replaced by total cavopulmonary connection. The midterm and late results of both the intraatrial lateral tunnel and extracardiac total cavopulmonary connections were compared and analyzed in this article. Extracardiac conduit is better. The Fontan circulation failure would appear at last because of nopump function of the right ventricle. Once Fontan circulation failure occurred and could not recover by medicine, heart transplantation is mandatory, but the source of donor heart is lacking. The study of mechanical cavopulmonary assist device, to “biventricularize” the univentricular Fontan circulation, has been developed, which is quite promising. Following the development of diagnostic and treatment techniques for fetal heart disease, the treatment procedure of complex congenital heart disease has been broadened in recent years, such as to prevent the severe aortic stenosis from developing into hypoplastic left heart syndrome with fetal cardiac intervention so as to increase the chance of biventricular repair, and to terminate gestation to decrease its birth rate of complex heart abnormalities, which could not be completely repaired to date.
ObjectiveTo evaluate the long-term effects of fenestration on patients at different risk levels, who performed external conduit total cavo-pulmonary connection operation.MethodsThis was a retrospective analysis which enrolled 383 patients undergoing external conduit total cavo-pulmonary connection in Fuwai Hospital from 2008 to 2015. Based on the preoperative data and whether fenestration in the operation, the whole cohorts were divided into four subgroups: a high risk group with fenestration(mean age: 10.53±7.06 years, 55 males), a high risk group with non-fenestration(mean age: 9.30±7.83 years, 43 males), a low risk group with fenestration(mean age: 8.91±7.13 years, 65 males) and a low risk group with non-fenestration(mean age: 8.23±5.34 years, 67 males). Then we collected and analyzed the perioperative data and long-term prognosis of this cohorts in different risk levels.ResultIn the high-risk group, the duration of chest drainage in fenestration group was significantly shorter than that of the non-fenestration group (12.39±12.03 d vs. 23.30±15.36 d, P=0.001). The incidence of delayed chest drainage in the fenestration group was lower than that in the non-fenestration group (25.0% vs. 47.1%, P=0.002). In addition, the length of hospital stay was shorter than that of the non-fenestration group (18.91±12.79 d vs. 29.68±37.77 d, P=0.004), with significant statistical difference. In the low risk group, there were 3 (2.7%) and 2 (1.6%) deaths at the follow-up in the non-fenestration and fenestration groups respectively (P=0.761). And 1 patient (1.3%), 1 patient (1.4%) died in the fenestration and non-fenestration group (P=0.593) in high risk group. However, there was no statistically significant difference among the fenestration and non-fenestration groups in terms of long-term intestinal protein loss syndrome and arrhythmia in different risk level groups.ConclusionFenestration can reduce the incidence of early complications and hospital stay, effectively, especially for the high-risk patient. Fenestration is recommended for high-risk patients with external conduit total cavo-pulmonary connection operation.
Objective To comprehensively analyze the clinical outcomes of total cavopulmonary connection (TCPC) in the treatment of functional single ventricle combined with heterotaxy syndrome (HS). MethodsA retrospective analysis was conducted on the patients with functional single ventricle and HS who underwent TCPC (a HS group) in Guangdong Provincial People's Hospital between 2004 and 2021. The analysis focused on postoperative complications, long-term survival rates, and identifying factors associated with patient survival. Early and late postoperative outcomes were compared with matched non-HS patients (a non-HS group). Results Before propensity score matching, 55 patients were collected in the HS group, including 42 males and 13 females, with a median age of 6.0 (4.2, 11.8) years and a median weight of 17.0 (14.2, 28.8) kg. Among the patients, there were 53 patients of right atrial isomerism and 2 patients of left atrial isomerism. Eight patients underwent TCPC in one stage. TCPC procedures included extracardiac conduit (n=39), intracardiac-extracardiac conduit (n=14), and direct cavopulmonary connection (n=2). Postoperative complications included infections in 27 patients, liver function damage in 19 patients, and acute kidney injury in 11 patients. There were 5 early deaths. The median follow-up time was 94.7 (64.3, 129.8) months. The 1-year, 5-year, and 10-year survival rates were 87.2%, 85.3%, and 74.3%, respectively. After propensity score matching, there were 45 patients in the HS group and 81 patients in the non-HS group. Compared to the non-HS group, those with HS had longer surgical and mechanical ventilation time, higher infection rates (P<0.05), and a 12.9% lower 10-year survival rate. Multivariate Cox regression analysis identified asplenia was a risk factor for mortality (HR=8.98, 95%CI 1.86-43.34, P=0.006). ConclusionCompared to non-HS patients, patients with HS have lower survival rates after TCPC, and asplenia is an independent risk factor for the survival of these patients.
Objective To analyze the feasibility of bidirectional Glenn procedure (BDG) in treatment of adult congenital heart disease (ACHD). Methods From December 2004 to December 2015, 42 ACHD patients received BDG in our hospital. There were 23 males and 19 females with a mean age of 24.6±8.5 years (range: 18 to 49 years). There were functional single ventricle (FSV) in 14 patients, Ebstein’s anomaly in 11, corrected transposition of great arteries in 7, transposition of great arteries in 5, double outlet of right ventricle in 3 and tricuspid atresia in 2. Twenty patients suffered moderate or severe atrioventricular valve regurgitation (AVVR). Half of the patients were operated upon with cardiopulmonary bypass (CPB) and the others with off-pump coronary artery bypass grafting (OPCABG). Thirty-four patients underwent unilateral BDG shunt and eight bilateral BDG shunts. Concomitant procedures included correction of Ebstein’s anomaly (7 patients), atrioventricular valve replacement (7), atrial septostomy (3), ligation of patent ductus arteriosus (3), ligation of major aortopulmonary collateral arteries (2), correction of total anomalous pulmonary venous connection (1) and mitral valve repair (1). Results The early operative mortality was 9.5% (4/42). FSV and moderate or severe AVVR were risk factors for BDG in ACHD. Early postoperative oxygen saturation increased from 78.8%±11.2% to 89.3%±6.6% (P<0.05). The follow-up time was 6-132 (41.4±33.1) months. There was no death. The heart function improved (2.7±0.5 vs. 1.9±0.4, P<0.05). Conclusion The BDG shunt can be applied to ACHD. Although the early mortality is relatively high, the middle- and long-term results are satisfactory. The oxygen saturation increases and the heart function improves. The life quality of patients will also improve. FSV and moderate or severe AVVR are risk factors for BDG in ACHD.
We reported a case of a six-year-old boy diagnosed of single ventricle, pulmonary atresia and interrupted inferior vena cava. After modified Blalock-Taussig shunt and bidirectional Glenn procedure, he received the Fontan procedure. The Fontan procedure was done through a unilateral thoracotomy, using an autologous pericardial conduit to connect hepatic vein and azygos vein. The result of short-term follow-up was satisfactory.
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.