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.
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.
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.
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 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.
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.
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.
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 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.
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.