目的 总结单心室瓣膜反流的外科治疗经验，观察治疗效果。 方法 回顾性分析2006年7月至2012年1月上海交通大学医学院附属新华医院61例单心室患者的临床资料，其中男36例，女25例；手术年龄2个月至 20岁；体重2～58 kg。右心室型41例，左心室型13例，未定型型7例。根据瓣膜反流程度不同分为3组，无/微量反流组：28例，瓣膜未行处理；轻/中度反流组：21例，瓣膜未行处理；重度反流组：12例，手术同期行瓣膜成形。收集所有患者住院及随访资料，分析轻/中度反流组、重度反流组瓣膜反流变化趋势，以及影响瓣膜反流的因素。结果 住院死亡5例，住院死亡率8.2% （5/61）。重度反流组患者行瓣膜成形术后反流程度较术前明显减轻（由术前4.00级下降至术后2.08级）。随访56例，随访时间6～38个月，重度反流组随访10例，随访期间死亡2例，其余8例中重度反流2例，中度反流3例，轻度反流2例，微量反流1例；瓣膜反流程度增加趋势明显（由术后平均2.08级增加至平均2.75级）。轻/中度反流组随访19例，随访中无死亡，其中反流程度增加至重度3例（原1例轻度反流，2例中度反流），反流程度由轻度增加至中度3例，瓣膜反流程度由术后平均2.33级增加为平均2.58级。轻/中度反流组瓣膜反流增加率与无/轻微反流组比较差异无统计学意义（瓣膜反流增加率为31.5% vs. 19.2%，χ2=0.36，P=0.55）。单因素分析结果显示，瓣膜反流增加者在随访过程中心功能较瓣膜反流无变化或减轻者明显降低（术后左心室射血分数53.11%±5.61% vs. 59.65%±3.32%，t =-5.49，P=0.00），而左心室舒张期末容积较瓣膜反流无变化或减轻者明显增加（t =2.58，P=0.01）。 结论 单心室合并重度瓣膜反流行瓣膜成形术近期效果较好，但随着心功能下降、心室扩张，瓣膜反流程度加重趋势明显；轻/中度瓣膜反流可暂不进行处理，但部分患者瓣膜反流有增加趋势，提示应注重术后随访。
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 The earlyapplied atriumpulmonary connection has been replaced by total cavopulmonary connection.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.
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 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.
ObjectiveTo analyze the outcomes of patients with one-stage Fontan procedure and short-term prognosis at a single institute. Method We retrospectively analyzed clinical records of 116 patients with one-stage Fontan procedure in our hospital from January 2008 through September 2013. There were 77 males and 39 females (36%), with median age 6.27 years (ranged 3.15 to 17.47 years) at the time of surgery and the median weight at 17.5 kg (ranged 10.0 to 80.0 kg). There were 55 patients with standard extracardiac conduit, 13 patients with direct extracardiac connection, 17 patients with intra-atrial or intra/extracardiac conduit, and 31 patients with lateral tunnel. ResultsMedian cardiopulmonary bypass time was 124 (61-256) minutes. Median cross-clamp time was 60 (19-152) minutes. There were six early deaths (5.1%). The overall median time of the cardiac intensive care unit stay was 4 (1-17) days, with a median ventilator support of 7.3 (1.0-181.0) hours. The mean room air saturation was 90.00%±4.68% before discharge. Median length of chest tube drainage was 10 (4-45) days. Multiple logistic analysis confirmed that heterotaxy syndrome was the only independent predictor for postoperative renal insufficiency. Operations with aortic cross-clamping (OR=26.184, 95% CI 1.712-400.451), preoperative sinus mode dysfunction (OR=6.777, 95% CI 1.495-30.721) and cross-clamp time over 60 minutes (OR=1.036, 95% CI 1.002-1.076) were predictors for prolonged chest tube drainage. A total of 110 patients were followed up for 17 (8-47) months with 2 deaths and 1 with thrombosis. No reoperation occurred. ConclusionThe one-stage Fontan procedure can be performed with satisfactory outcomes. Staged strategies for operations may be appropriately loosen for selected elder children.
Significant atrioventricular valve (AVV) insufficiency has been associated with increased mortality and morbidity in patients with single ventricle. Although techniques such as annuloplasty, chordal elongation, and the use of pericardial patches can improve AVV function in many patients, a number of patients continue to have significant AVV insufficiency and may require AVV replacement. The present review made a comprehensive literature review concerning the outcome, optimal timing, key points of technique skills and prognosis evaluation.
Objective Complex congenital heart defects are sometimes treated by Fontan palliation for various reasons. However, the middle- and long-term prognosis of single-ventricle repair is worse than that of two-ventricle repair. In this study we reported the results of biventricular conversion in these challenging patients initially palliated towards single-ventricle repair. Methods Eight patients underwent biventricular repair conversion from prior bidirectional Glenn shunt palliation in our hospital between October 2013 and March 2016. The median age in bidirectional Glenn shunt was 2.6 years (range, 1.0 to 5.9 years) and in biventricular repair conversion was 6.6 years (range, 4.5 to 11.1 years). Three patients suffered complete transposition of great arteries combined with ventricular septal defect and left ventricular outflow tract obstruction, three double outlet right ventricle and non-committed ventricular septal defect combined with pulmonary stenosis or atresia, one double outlet right ventricle combined with complete ativentricular septal defect and pulmonary stenosis and one Tetralogy of Fallot. Results Bidirectional Glenn shunt was taken down and superior vena cava was reconnected to the right atrium in all patients. Mean cardiopulmonary bypass and aortic cross-clamp time was 275.6±107.1 min and 165.9±63.6 min, respectively. Mean length of hospital stay and ICU stay were 33.6±23.0 d and 20.3±21.0 d, respectively. At a mean follow-up of 1.4±0.7 years, there was no mortality and reoperation. No patients presented with sinoatrial node dysfunction and superior vena cava anastomotic stenosis. According to the New York Heart Association (NYHA) Functional Classification, all patients were classified asⅠ-Ⅱ. Conclusion Biventricular repair conversion can be safely performed with favorable mortality and morbidity in specific patients palliated towards single-ventricle repair. Further follow-up is needed to investigate the long-term outcomes.