Objective To optimize the surgical strategies and managements of doubleoutlet right ventricle(DORV)with atrioventricular discordance, the anatomic features, the surgical managements and results were reviewed. Methods From September 1990 to August 2004, 7 cases of DORV with atrioventricular discordance received surgical therapy. The age ranged from 3 years to 7 years. Surgical managements included: ventricular septal defect (VSD )repair and homograft connected the morphologic left ventricle with pulmonary artery in 3 cases, double-switch in 2 cases, modified Fontan in 2 cases. Results The early mortality rate was 14.3% (1/7). The death was because by Homograft infection 2 months after repair. Complete atrioventricular block occurred in one case who received permanent pacemaker later. Obstruction of superior vena cava return and pericardial effusion occurred in one Fontan case who was recovered at the time of discharge. The time of ICU stay was shortest in double-switch patients in 6 days postoperation. Conclusion Double-switch operation is the first choice in correction of DORV with atrioventricular discordance, especially in patients with right ventricular disfunction or tricuspidal regurgitation. If the heart can not be corrected because of combined complicated malformations, the strategy of one ventricular repair can be chosen.
Objective To summarize clinical experience of a single stage surgical approach on patients with TaussigBing anomaly combined with side by side relationship of great arteries. Methods From May 2000 to Sep. 2007, 26 patients (age 3.1±2.2 months) with TaussigBing anomaly with side by side great arteries underwent the single stage operation, including arterial switch operation (n=25), and Kawashima operation (n=1). Aortic arch obstruction was present in 13 patients. The corrections of aortic arch obstruction included descending aorta end to end anastomosis to aortic arch or end to side anastomosis to ascending aorta. Results The hospital mortality rate was 11.5% (3/26). There was no operative death in continuous 15 patients after Jun. 2005. 21 patients were followed up for 1 to 5 years. The patients’ growth and development were improved obviously. The sizes of the hearts were smaller than those before operations. The pulmonary blood flow was decreased obviously. Two patients required re-operations because of supravalvular pulmonary stenosis. Conclusion Taussig-Bing anomaly with side by side great arteries has complex anatomical characters. In order to improve the operative outcomes, the optimized operative strategies should be considered in according with different anatomies.
ObjectiveTo compare and investigate the efficacy and differences of modified B-T shunt, central shunt and right ventricle-pulmonary artery (RV-PA) connection in the treatment of pulmonary atresia with ventricular septal defect (PA/VSD).MethodsA total of 124 children with PA/VSD underwent initial palliative repair in Shanghai Children's Medical Center from September 2014 to August 2019, including 63 males and 61 females, aged 7 days to 15 years. They were divided into in a modified B-T shunt group (55 patients), a central shunt group (22 patients) and a RV-PA connection group (47 patients). The clinical data of these children were retrospectively analyzed.ResultsThere were 9 early deaths after palliation, with an early mortality rate of 7.3%. The mean follow-up time was 26.5±20.3 months, with 5 patients lost to follow-up, 5 deaths during the follow-up period, and 105 survivors. The 1-year and 5-year survival rates were both 89.7%. The monthly increased Nakata index was 5.2 (–0.2, 12.3) mm2/m2, 9.2 (0.1, 23.6) mm2/m2, 6.3 (1.8, 23.3) mm2/m2 in the modified B-T shunt group, the central shunt group, and the RV-PA connection group, respectively, with no statistical difference among the three groups. The 1-year survival rate was 85.3%, 78.4%, 95.2%, and the 5-year (4-year in the central shunt group) survival rate was 85.3%, 58.8%, 95.2% in the three groups, respectively, with a statistical difference among them (P<0.05). The complete repair rate was 36.5%, 19.0% and 67.4% in the three groups, respectively, with a statistical difference among the three groups (P<0.001).ConclusionAll these three palliative surgical approaches can effectively promote pulmonary vascular development. But compared with systemic-pulmonary shunt, RV-PA connection has a lower perioperative mortality rate and can achieve a higher complete repair rate at a later stage, which is beneficial for long-term prognosis.
ObjectiveTo explore the appropriate method of biventricular repair and analyze the risk factors for reoperation, by summarizing the 15-year treatment experience of biventricular repair for double outlet right ventricle with non-committed ventricular septal defect (DORVncVSD).MethodsClinical data of 162 consecutive patients with DORVncVSD who had biventricular repair from 2005 to 2019 in our center were retrospectively analyzed. The children were divided into two groups according to the path of intracardiac tunnel: 110 patients with ventricular septal defect rerouted to the aorta were recruited into a group A (75 males and 35 females aged 3.6±3.2 years); 52 patients with ventricular septal defect rerouted to the pulmonary artery were into a group B (30 males and 22 females aged 2.8±2.7 years). In order to establish a smooth intracardiac tunnel, enlargement of VSD, the resection of conus muscle and the transfer of tricuspid tendon or papillary muscle, etc were performed at the same time.ResultsIn the patients with biventricular repair, there were 9 (5.6%) early deaths and 6 (3.7%) early intracardiac baffle obstructions. During the follow-up of 7.5±7.0 years, and 8 (4.9%) late deaths occurred. The 1-year, 5-year, 10-year and 15-year survival rates of the group A were 92.7%, 91.1%, 91.1%, 85.4%, respectively and those of the group B were 92.2%, 85.2%, 85.2%, 85.2%, respectively. The difference between the two groups was not statistically significant (P=0.560). The follow-up results showed that 10 (6.2%) patients had late-onset intracardiac tunnel obstruction, and 8 patients underwent reoperation. There were more late-onset intracardiac tunnel obstruction patients and overall intracardiac tunnel obstruction patients in the group A than those in the group B (9 patients vs. 1 patient, P=0.017; 15 patients vs. 1 patient, P=0.001). No significant difference of early mortality and late mortality was noted for the group A (P=0.386) and the group B (P=0.223). Also it was noted that performing tricuspid valve operation at the same time in the group A had a significant impact to reduce the occurrence rate of intracardiac obstruction (1/46 vs. 15/64, P=0.004), without any tricuspid regurgitation or stenosis. The reoperation rate of patients with Rastelli after right ventricular outflow tract lesions was significantly higher than that of REV surgery and double root replacement surgery (5/14 vs. 0/38, P<0.001).ConclusionThe effect of biventricular repair for DORVncVSD is satisfactory. And concomitant tricuspid procedures can help reduce the occurrence of intracardiac obstructions. Reconstruction of right ventricular outflow tract with biological valved conduit is a risk factor for reoperation.
Objective To investigate the differences in postoperative mortality and identify potential influential factors in patients with a systemic left ventricle (SLV) versus a systemic right ventricle (SRV) following total cavopulmonary connection (TCPC). MethodsWe retrospectively collected data from functional single ventricle patients who underwent TCPC at the Department of Cardiac Surgery, Guangdong Provincial People’s Hospital, between October 2004 and July 2021. The cohort was categorized based on ventricular morphology into two groups: a SLV group and a SRV group. All procedures were performed via a median sternotomy under cardiopulmonary bypass. ResultsA total of 195 patients were included, comprising 108 patients in the SLV group (69 males, 39 females) and 87 in the SRV group (61 males, 26 females). The median age at surgery was 5.7 (IQR, 4.0-11.2) years, and the median body mass index (BMI) was 15.1 (IQR, 13.5-16.2) kg/m2 for the SLV group. For the SRV group, the median age was 5.7 (IQR, 4.1-8.9) years, and the median BMI was 14.7 (IQR, 13.6-15.9) kg/m2. The proportion of patients with situs inversus, heterotaxy syndrome, and moderate or greater atrioventricular valve regurgitation was significantly higher in the SRV group. Patients in the SRV group had a higher rate of fenestration and experienced longer aortic cross-clamp, cardiopulmonary bypass, and operative times, as well as prolonged postoperative hospital stays and chest tube durations. However, there were no statistical differences in early or late mortality between the two groups (P>0.05). Multivariate analysis identified pulmonary vascular resistance, postoperative aspartate aminotransferase, and postoperative creatinine as independent risk factors for mortality, while postoperative percutaneous oxygen saturation and hemoglobin levels were identified as protective factors. Conclusion The post-TCPC survival rate in patients with a SRV is non-inferior to that in patients with a SLV. However, the overall long-term mortality for both groups remains high, warranting close monitoring of the long-term survival outcomes in this patient population.
Objective To evaluate the efficacy of 3-dimensional printing model (3DPM) aiding decision making and surgery rehearsal for the treatment of double outlet right ventricle (DORV) with non-committed ventricular septal defect (NC-VSD). Methods From January 1st, 2012 through December 30th, 2014, 12 patients with DORV and NC-VSD were operated with the aid of “3DPM guidance” to do decision making and surgical technique rehearsal preoperatively. There were 9 males and 3 females at age of 2.9±2.2 years. The “3DPM guidance” consisted of step by step procedures: computerized tomography (CT) scan for the patients, CT based 3DPM rendering, 3DPM exploration, decision making, and surgery rehearsal. During surgery rehearsal, surgeons did patch designing, VSD enlargement planning, muscle bundle resection etc. Eight out of the twelve patients underwent biventricular repair, 4 patients underwent single ventricle repair. Six of the eight biventricular repair patients had intra-ventricular baffle repair, 1 patient had intra-ventricular baffle repair and arterial switch procedure, 1 had modified Nikaidoh procedure. VSD enlargement was performed in all the patients in biventricular repair group. The reasons not to do a biventricular repair included very restrictive VSD, tricuspid attachments across the sub-aortic passway. Results The operation findings correlated well with the 3DPM in all the cases. There was no hospital death, no major complication. One patient had a mild sub-aortic stenosis and he was under close follow-up. There was no late death and reoperation. Surgeons involved were satisfied with the “3DPM guidance”. Conclusions 3-D printing model is an excellent way to help decision making for DORV with NC-VSD and can provide surgery simulation which decrease complication rate and help achieve good outcomes.
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.
Double outlet right ventricle (DORV) is a complex cardiac malformation with many anatomic variations and various approaches for surgical repair. DORV is mainly defined as the congenital heart disease with ventriculoarterial connection in which both pulmonary artery and aorta arising primarily (>50%) from the right ventricle, associated with continuity or discontinuity between the aorta and mitral valve. DORV can be subclassified by various ways. Now subclassification is usually performed according to the relationship between the ventricular septal defect (VSD) and the great arteries. Various approaches for surgical repair of DORV ranging from single ventricle palliation to biventricular repair are reported from many centers. However, the high-grade guideline of surgical management of DORV is still absent. Hence, we developed the Chinese expert consensus on DORV as the evidence for surgical strategies.
ObjectiveTo summarize the experience and lessons of right ventricular decompression in children with pulmonary atresia and intact ventricular septum (PA/IVS) and to reflect on the strategies of right ventricular decompression.MethodsThe clinical data of 12 children with PA/IVS who underwent right ventricular decompression in our hospital from March 2015 to December 2019 were reviewed retrospectively. There were 10 males and 2 females with a median age at the time of surgery was 5 d (range, 1-627 d). Correlation analysis between the pulmonary valve transvalvular pressure gradient and changes in Z score of tricuspid valves after decompression was performed.ResultsOne patient died of refractory hypoxemia due to circulatory shunt postoperatively and family members gave up treatment. There were 2 (16.67%) patients received postoperative intervention. The pulmonary transvalvular gradient after decompression was 31.95±21.75 mm Hg. Mild pulmonary regurgitation was found in 7 patients, moderate in 2 patients, and massive in 1 patient. The median time of mechanical ventilation was 30.50 h (range, 6.00-270.50 h), and the average duration of ICU stay was 164.06±87.74 h. The average postoperative follow-up time was 354.82±331.37 d. At the last follow-up, the average Z score of tricuspid valves was 1.32±0.71, the median pressure gradient between right ventricle and main pulmonary artery was 41.75 mm Hg (range, 21-146 mm Hg) and the average percutaneous oxygen saturation was 92.78%±3.73%. Two children underwent percutaneous balloon pulmonary valvoplasty at 6 and 10 months after surgery, respectively, with the rate of reintervention-free of 81.8%. There was no significant correlation between pulmonary transvalvular gradients after decompression and changes in Z score of tricuspid valves (r=–0.506, P=0.201).ConclusionFor children with PA/IVS, the simple pursuit of adequate decompression during right ventricular decompression may lead to severe pulmonary dysfunction, increase the risk of ineffective circular shunt, and induce refractory hypoxemia. The staged decompression can ensure the safety and effectiveness for initial surgery and reduce the risk of postoperative death.
Right ventricular (RV) failure has become a deadly complication of left ventricular assist device (LVAD) implantation, for which desynchrony in bi-ventricular pulse resulting from a LVAD is among the important factor. This paper investigated how different control modes affect the synchronization of pulse between LV (left ventricular) and RV by numerical method. The numerical results showed that the systolic duration between LV and RV did not significantly differ at baseline (LVAD off and cannula clamped) (48.52% vs. 51.77%, respectively). The systolic period was significantly shorter than the RV systolic period in the continuous-flow mode (LV vs. RV: 24.38% vs. 49.16%) and the LV systolic period at baseline. The LV systolic duration was significantly shorter than the RV systolic duration in the pulse mode (LV vs. RV: 28.38% vs. 50.41%), but longer than the LV systolic duration in the continuous-flow mode. There was no significant difference between the LV and RV systolic periods in the counter-pulse mode (LV vs. RV: 43.13% vs. 49.23%). However, the LV systolic periods was shorter than the no-pump mode and much longer than the continuous-flow mode. Compared with continuous-flow and pulse mode, the reduction in rotational speed (RS) brought out by counter-pulse mode significantly corrected the duration of LV systolic phase. The shortened duration of systolic phase in the continuous-flow mode was corrected as re-synchronization in the counter-pulse mode between LV and RV. Hence, we postulated that the beneficial effects on RV function were due to re-synchronizing of RV and LV contraction. In conclusion, decreased RS delivered during the systolic phase using the counter-pulse mode holds promise for the clinical correction of desynchrony in bi-ventricular pulse resulting from a LVAD and confers a benefit on RV function.