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.
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.
ObjectiveTo evaluate the surgical strategy and follow-up for pulmonary atresia with intact ventricular septum (PA/IVS) in our heart center.MethodsFrom January 2008 to December 2018, 151 patients with PA/IVS were divided into two groups: a one-stage surgery group (26 patients), including 17 males and 9 females at an average age of 14.7±13.2 months, and a staged surgery group (125 patients) including 72 males and 53 females at an average age of 6.4±6.3 months. The clinical effectiveness of the two groups were analyzed.ResultsAll patients were followed up for 1-11 years. Eighteen patients died and 19 patients were lost to follow-up. The 1-year, 5-year and 10-year survival rate was 90.2%, 87.0%, and 85.2%, respectively. Two patients died in the one-stage surgery group. Twelve patients died after initial surgery, and 4 patients died after final operation in the staged surgery group. The Z value of tricuspid valve (P=0.013) and severe right ventricular dysplasia (P=0.025) were the risk factors of postoperative death in the patients with PA/IVS. Furthermore, 58 patients completed final operation, and the total number of the final operation (including one-stage radical surgery) accounted for 55.6% (84/151). Five patients accepted the re-operation intervention in the medium-term follow-up. The rest of the patients recovered well. Only 2 patients were classified as grade Ⅲ in cardiac function, and the rest patients were classified as gradeⅠ-Ⅱ.ConclusionAccording to the degree of right ventricular hypoplasia, the age at operation and the presence or absence of coronary artery malformation, the individualized surgical strategy could significantly improve the success rate of PA/IVS, and early completion of right ventricular decompression operation is conducive to improve the chance for biventricular repair.
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.
The single ventricular circulation is notable for coexisting systemic venous hypertension and pulmonary arterial hypotension. The use of mechanical circulatory support (MCS) devices is a viable therapeutic treatment option for patients with congestive heart failure. Ventricular assisted devices, cavopulmonary assisted devices, and total artificial heart pumps continue to gain acceptance as viable treatment strategies for single ventricular physiology patients as bridge-to transplantation, bridge-to-recovery, and longer-term circulatory support alternatives. Patients with single ventricular physiology had the lower survival rates compared with those with biventricular circulation. We present a review of the current and future MCS devices for patients with univentricular circulations.
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 investigate the value and significance of the changes of plasma level of brain natriuretic peptide（BNP） in evaluating ventricle performance of functional single ventricle after total cavopulmonary connection （TCPC）. Methods We studied 11 patients with functional single ventricle undergone TCPC procedure after 2.1 years, who were followed-up at our ward between April 2004 and November 2004, 7 of them were males and 4 of them were females （TCPC group）. The clinical heart function of patients was scored according to the modified scoring system described by Ross. We obtained 3ml blood samples from the extremital vein of all subjects. Blood was collected into chilled tubes containing EDTA and aprotinin （4.5mg and 1 500u/ml blood, respectively）. The blood samples were promptly centrifuged （-4℃, 3 000r/min for 10 min） and the plasma was separated. BNP concentration was determined using immunoradiometric assay kits. Magnetic resonance imaging （MRI） examination was undertaken in 6 patients of TCPC group to analyse the relative factors with the change of BNP. Control group included 9 healthy children. Results （1） Median plasma BNP level for TCPC group and control group was 400pg/ ml （IQR200-690） and 110 pg/ml （IQR90-190）, respectively. There was a significant difference in plasma BNP between them （P=0. 003）. （2） The results of the index of heart function of TCPC group determined by MRI were 65. 76±8. 65 ml/m2 in end-diastolic volume index, 31. 90±6. 36ml/m2 in end-systolic volume index, 39.09±11.76ml/m2 in stroke volume index, 0. 52± 0. 06 in ejection fraction（EF）, 2.38±0.58L/min·m2 in cardiac index （CI）, 103.49±21.57g/m2 in mass index and 1.57±0.24 in mass/EDV. （3） The plasma BNP level for TCPC group was significant correlation with operation ages（r=0.632, P=0.041 ）. There was no correlation between plasma BNP level with EF, CI, score of Ross, gender, ages, percutaneous oxygen saturation（SpO2） and the type of dominant ventricle, respectively. Conclusions Raised concentration of plasma BNP in patients 2 years after undertaken TCPC procedure indicates that nervous-endosecretory system is still under stress condition. This pattern suggests that neurohormonal activation is primarily related to the altered postoperative physiology. the significance of BNP in patients of functional single ventricle after undertaken TCPC is different from tat in biventricular physiology patiens. The plasma BNP level could not be correctly evaluated the cardiac function after TCPC operation.
Objective To explore the surgical management method and outcome of functional univentricular heart with total anomalous pulmonary vein drainage (UVH-TAPVD). Methods We reviewed the surgical procedures and results for 44 UVH-TAPVD patients in our hospital between the year 2010 and 2016. There were 34 males and 10 females. The age of the patients was 312 (77-4 220) d when they accepted the first surgical treatment. Results There were 8 deaths in stage Ⅰ palliation, 1 death in stage Ⅱ palliation and 5 deaths during the follow-up. The overall survival rate was 68.2% (30/44). Glenn operation was undertaken in 34 patients with 5 deaths. Fontan operation was undertaken in 9 patients with 2 deaths. Conclusion Surgical strategies for UVH-TAPVD should be planned according to different anatomical and pathophysiological conditions in different patients. Staged palliations can reduce mortality and morbidity. But pulmonary venous obstruction and heterotaxy syndrome are still risk factors for these patients.
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.
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.