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。
ObjectiveTo investigate the management of hepatic vein (HV) in patients with functional single ventricle (FSV) and separate hepatic venous drainage (SHVD) undergoing total cavopulmonary connection (TCPC) and evaluate this kind of surgery.MethodsThe clinical data of 32 consecutive patients with SHVD who underwent modified TCPC operation from August 2005 to January 2017 in our center were retrospectively analyzed. There were 25 males and 7 females with an average age of 2-19 (8.0±5.0) years and body weight of 11-66 (25.4±15.8) kg.ResultsThere were 20 patients with heterotaxy syndrome and 12 patients with other types of FSV. SHVD was diagnosed preoperatively in 27 patients, among whom 20 patients were connected by intra-extracardiac Gore-Tex conduit, and the other 7 patients were connected by extracardiac Gore-Tex conduit. Because of the missed diagnosis of SHVD, the other 5 patients showed severe decrease of blood oxygen saturation in the early postoperative period and underwent re-operation soon. The postoperative blood oxygen saturation was 92.0% (90.0%, 96.0%), central venous pressure was 10-23 (15.5±3.5) mm Hg, mechanical ventilation assisted time was 16.0 (7.5, 24.0) h, and ICU stay time was 3.0 (2.0, 5.5) d. There were 3 early and 1 late deaths.ConclusionIntra-extracardiac conduit is an effective and feasible modified TCPC operation for patients with FSV and SHVD, while the surgical details need to be formulated in combination with individual anatomical structure. Preoperative missed diagnosis of SHVD must be avoided. Otherwise, after TCPC, a large amount of stealing blood from HV with low circulation pressure into atrium would lead to unacceptable hypoxemia.
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.
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 explore current results after staged operations in patients with functional single ventricle anomalies and pulmonary hypertension. Methods We retrospectively analyzed the clinical data of 129 patients with functional single ventricle anomalies and pulmonary hypertension undergoing pulmonary artery banding in our hospital between April 2008 and December 2015. There were 81 males and 48 females. There were 71 patients with double outlet of right ventricle, 17 patients with tricuspid atresia, 7 patients with transposition of great arteries, 33 patients with uni-ventricular heart, and one patient with complete atrio-ventricular septal defect. The surgical results, transition to Glenn procedure and subsequent transition to Fontan procedure were analyzed. Results The 129 patients underwent 159 operations of pulmonary artery banding totally. Hospital mortality was 4.7% (6/129). Nine patients were lost to follow-up. Eighty-seven (67.4%) patients underwent the second-stage Glenn procedure, and 43 patients(33.3%) underwent third-stage Fontan procedure. Two patients died after Glenn and 3 patients died after Fontan separately. There were 32 patients who accompanied with coarctation, interruption of aortic arch, heterotaxy, total anomalous pulmonary venous connection or atrio-ventricular valve regurgitation. Fifteen (46.9%) patients succeeded in transition to Glenn, and 6 (18.8%) patients succeeded in transition to Fontan. Fourteen patients developed obstruction of left ventricular outlet tract or bulbo-ventricular foramen. Conclusion Early pulmonary artery banding is an acceptable strategy for patients with single ventricle anomalies and pulmonary hypertension. Outcomes and results of subsequent Glenn and Fontan procedures are generally good. Accompanied complex anomalies are risk factors for lower ratio of transition to Glenn and Fontan procedure.
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.
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.
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.