Objective To assess clinical results of three different conduit materials （Gore-Tex synthetic graft，bovinejugular vein and autologous pericardium）for palliative right ventricle-to-pulmonary artery （RV-PA） shunt，and explore the correlation between suitable conduit size and patients’ body weight and McGoon ratio. Methods We retrospectively analyzed clinical data of 24 patients with congenital heart diseases who underwent palliative RV-PA shunt in Department of Pediatric Cardiovascular Surgery of Fu Wai Cardiovascular Hospital from July 2010 to July 2012. There were 11 males and 13 females with their age ranging from 60 days to 6 years and body weight of 10.22±7.41 kg. There were 22 patients with pulmonary atresia and ventricular septal defect （PAVSD），1 patient with tetralogy of Fallot （TOF） and 1 patient with doubleoutlet right ventricle （DORV）. Among different conduit materials，autologous pericardium was used for 17 patients，Gore-Texsynthetic graft was used for 5 patients，and bovine jugular vein was used for 2 patients. Conduit size and children’s body weight were analyzed with linear regression，then the equation was corrected with McGoon ratio. Results There was no perioperative death. Postoperative percutaneous saturation （SpO2）of the 24 children was 20.37%±28.33% higher than preoperative SpO2 . Electrocardiogram showed sinus rhythm in all the patients. Twenty-three patients were NYHA classⅡ，and 1 patient was NYHA classⅢ. Postoperative mechanical ventilation time of patients with autologous pericardium were significantly shorter than those of patients with other 2 materials （P=0.017）. Sixteen patients were followed up from 10 months to 2 years after discharge，including 12 patients with autologous pericardium，3 patients with Gore-Tex synthetic graft and 1 patient with bovine jugular vein. During follow-up，McGoon ratio of patients with autologous pericardium，Gore-Tex synthetic graft and bovine jugular vein were 1.98±0.46，1.83±0.33 and 1.68 respectively，all of which weresignificantly higher than preoperative McGoon ratio （P＜0.05）. Six patients underwent radical corrective surgery，including5 patients with autologous pericardium and 1 patient with Gore-Tex synthetic graft. There was no complication directly related to surgery during follow-up. Linear regression was performed to form an equation between suitable conduit size and patients’ body weight：conduit diameter （mm）=0.327×body weight （kg）＋4.599. McGoon ratio，conduit size and equationresult were compared to find a practical choice of conduit size. If McGoon ratio＜0.8，the first integer greater than the equation result could be chosen. If McGoon ratio＞1.2，the first integer less than the equation result could be chosen. If 1.2＞McGoon ratio＞0.8，the first integer either less or greater than the equation result could be chosen. Group analysis showed that patients who recovered better postoperatively were those whose conduit sizes were closer to equation results as well as equation results corrected with McGoon ratio. Conclusion All the 3 materials can be conventionally chosen for RV-PA shunt. Appropriate conduit size can be decided upon patients’ body weight and McGoon ratio for RV-PA shunt.
Objective To observe and describe anatomical types of the pulmonary arteries to keep safety of lung resection. Methods Between November 25, 2005 and January 22, 2013, 194 patients who underwent right upper lobectomy/sleeve lobectomy or combined lung resection including right upper lobectomy were included in Peking University Cancer Hospital. There were 128 males with a median age of 59 (37-86) years and 66 females with a median age of 60 (42-77) years. We separated the pulmonary arteries and recorded the number and positions of them. Some patients were recorded photographically. Results There were 10 types of right upper lobe pulmonary artery branches in this study. Type 1: 1 apicoanterior segmental artery, 1 ascending segmental artery, 96 patients (49.5%); Type 2: 1 apicoanterior segmental artery, 2 ascending segmental arteries, 48 patients (24.7%); Type 3: 2 apicoanterior segmental arteries, 1 ascending segmental artery, 28 patients (14.4%); Type 4: 2 apicoanterior segmental arteries, 2 ascending segmental arteries, 9 patients (4.6%); Type 5: 1 apicoanterior segmental artery only, 6 patients (3.1%); Type 6: 1 apicoanterior segmental artery, 3 ascending segmental arteries, 3 patients (1.5%); Type 7: 4 apicoanterior segmental arteries, 1 ascending segmental artery, 1 patient (0.5%); Type 8: 3 apicoanterior segmental arteries, 1 ascending segmental artery, 1 patient (0.5%); Type 9: 2 apicoanterior segmental arteries, 1 patient (0.5%); Type 10: 3 apicoanterior segmental arteries, 2 ascending segmental arteries, 1 patient (0.5%). Conclusion The types of pulmonary artery branches are predictable in some way. It would be helpful to reduce the risk of pulmonary artery injury and improve the operation safety by following the rules. Variations of pulmonary artery should be noticed to avoid the major bleeding due to the pulmonary artery injury.
ObjectiveTo compare early clinical outcomes between systemic-pulmonary shunts (SPS) and right ventricular to pulmonary artery connection (RV-PA connection) for patients with pulmonary atresia and ventricular septal defect (PA/VSD), and investigate early management strategies for these 2 different palliative procedures. MethodsWe retrospectively analyzed clinical data of 89 PA/VSD patients who underwent SPS or RV-PA connection in Fu Wai Hospital from January 2009 to December 2011. According to different surgical procedures, all the 89 patients were divided into 2 groups. In SPS group, there were 59 patients including 35 males and 24 females with their median age of 25 months (4 months to 8 years). In RV-PA connection group, there were 30 patients including 19 males and 11 females with their median age of 24 months (28 days to 7 years and 2 months). Early clinical outcomes including mechanical ventilation time, length of ICU stay, morbidity, reexploration, improvement of oxygen saturation (SO2) and mortality were compared between the 2 groups. ResultsAmong 59 patients in SPS group, 3 patients (5.1%) died postoperatively. There was no in-hospital death among 30 patients in RV-PA connection group. The improvement of percentage of SO2 of RV-PA connection group was significantly higher than that of SPS group (31.7% vs. 22.2%, P < 0.05). There was no statistical difference in length of ICU stay (3.6±2.5 days vs. 4.2±5.1 days, P > 0.05), mechanical ventilation time (34.8±33.5 hours vs. 44.3±39.6 hours, P > 0.05), postoperative morbidity (37.3% vs. 30.0%, P > 0.05) or reexploration rate (15.3% vs. 6.7%, P > 0.05) between SPS group and RV-PA connection group. Incidence of serious postoperative complications of SPS group was signi-ficantly higher than that of RV-PA connection group (25.4% vs. 6.7%, P < 0.05). ConclusionEarly clinical outcomes of RV-PA connection is better than SPS for PA/VSD patients including greater SO2 improvement and lower mortality. Mid-term and long-term clinical results as well as larger study samples are needed for better evaluation.
ObjectiveTo analyze the early outcomes of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) patients with severe left ventricular dysfunction after surgical repair, and to explore the predictors for extracorporeal membrane oxygenation (ECMO) support for these patients.MethodsThe clinical data of ALCAPA patients with severe left ventricular dysfunction (left ventricular ejection fraction<40%) who underwent coronary artery reimplantation in the pediatric center of our hospital from 2013 to 2020 were retrospectively analyzed. The patients were divided into an ECMO group and a non-ECMO group. Clinical data of the two groups were compared and analyzed.ResultsA total of 64 ALCAPA patients were included. There were 7 patients in the ECMO group, including 4 males and 3 females aged 6.58±1.84 months. There were 57 pateints in the non-ECMO group, including 30 males and 27 females aged 4.34±2.56 months. The mortality of the patients was 6.25% (4/64), including 2 patients in the ECMO group, and 2 in the non-ECMO group. The postoperative complications rate was significantly higher in the ECMO group than that in the non-ECMO group (P=0.041). There were statistical differences in the cardiopulmonary bypass time [254 (153, 417) min vs. 106 (51, 192) min, P=0.013], aortic cross-clamping (ACC) time (89.57±13.66 min vs. 61.58±19.57 min, P=0.039), and preoperative left ventricular end-diastolic diameter/body surface area (132.32±14.71 mm/m2 vs. 108.00±29.64 mm/m2, P=0.040) between the two groups. Multivariate logistic regression analysis showed that ACC time was an independent risk factor for postoperative ECMO support (P=0.005). Receiver operating characteristic (ROC) curve analysis showed that the area under the ROC curve was 0.757, the sensitivity was 85.70%, specificity was 66.70%, with the cut-off value of 66 min.ConclusionACC time is an independent risk factor for postoperative ECMO support. Patients with an ACC time>66 min have a significantly higher risk for ECMO support after the surgery.
Objective To explore the treatment method of congenital heart disease (CHD) with pulmonary artery hypertension (PAH) in infants with Down syndrome (DS). Methods The clinical data of 60 CHD patients with PAH from March 2015 to August 2016 in our hospital were retrospectively analyzed. There were 30 infants with DS classified as a DS group (trial group, 17 males and 13 females with a mean age of 1.15±0.25 years) and the other 30 patients without DS were classified as a control group (20 males and 10 females with a mean age of 1.24±0.30 years). All the patients underwent surgical treatment and fasudil combined with sildenafil were used to prevent pulmonary hypertension crisis postoperatively. Results There was no significant difference in cardiopulmonary bypass time, aortic cross-clamping time, modified ultrafiltration time and the incidence of postoperative respiratory complications between the two groups. The pulmonary systolic blood pressure significantly decreased at 24 h after operation in the two groups (both P<0.05). The arterial oxygen pressure and oxygenation index of the trial group were lower than those of the control group at 6 h after operation (both P<0.05). The mechanical ventilation time and intensive care time of the trial group were significantly longer than those of the control group (P=0.007 and P=0.000, respectively). There were no reoperations or early death. Conclusion The effects of surgical repair of CHD with PAH in infants with DS are satisfactory by grasping the indication, protecting lung function and controlling PAH in the early postoperative period, although there is a high incidence of pulmonary complications.
ObjectiveTo explore surgical methods and risk factors of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). MethodsClinical data of 28 ALCAPA patients who underwent surgical repair from October 1993 to September 2013 in Beijing Anzhen Hospital were retrospectively reviewed. There were 8 male and 20 female patients with their age of 0.6-l6.8 (4.3±0.7)years including 10 patients less than 1 years old. Surgical procedures included simple ligation of left coronary artery, intrapulmonary tunnel procedure (Takeuchi)and direct coronary reimplantation of the anomalous artery. Postoperative death, complication and cardiac function were observed. ResultsAmong the 28 patients, 1 patient received simple ligation of left coronary artery, and 7 patients received intrapulmonary tunnel procedure (Takeuchi), among whom 2 patients died postoperatively. Twenty patients received direct implantation of the anomalous artery into the ascending aorta, and 3 patients died postoperatively. Five patients who died postoperatively were 10.20±3.27 months old, including 3 patients with moderate mitral regurgitation (MR)and 2 patients with mild MR preoperatively. Preoperative heart function of the patients who died postoperatively was significantly reduced. Preoperative left ventricular ejection fraction of the patients who died postoperatively was significantly lower than that of the patients who survived (36.6%±8.5% vs. 60.9%±10.7%, P=0.000). Low cardiac output syndrome was the reason for all postoperative death. All survival patients were followed up from 1 month to 18 years. One patient who underwent intra-pulmonary tunnel procedure (Takeuchi)received pulmonary artery balloon dilatation for pulmonary supravalvular stenosis 15 years after discharge. None of the other patients received a secondary operation. During follow-up, left ventricular function was improved. Growth and development of all the patients was normal. MR did not significantly aggravate in all the patients. ConclusionPatients with younger age and worse left ventricular function have greater surgical risks of ALCAPA.
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 whether nesiritide (recombinant human brain natriuretic peptide, rh-BNP) could be used to treat pulmonary artery hypertension. MethodsA 34-year-old female patient with severe symptomatic idiopathic pulmonary artery hypertension was reported, who was refractory to routine therapies, including prostacyclin. Therapy with continuous nesiritide infusion resulted in significant decrease in pulmonary vascular resistance and an improvement in dyspnea. The relevant literature was reviewed. ResultsThe clinical symptoms of this patient relieved significantly after nesiritide therapy. Literature review showed that nesiritide could increase the production of nitrogen oxides and cyclic guanosine monophosphate in the body, so as to dilate the vessels which were shrunk due to hypoxia and low down the pulmonary vascular resistance. ConclusionNesiritide is useful to treat severe pulmonary artery hypertension, and combination with phosphodiesterase type-5 inhibitors may be a brand new therapy of value.
ObjectiveTo compare the benefits and drawbacks of primary patch expansion versus pericardial tube right ventricular-pulmonary artery connection in patients diagnosed with pulmonary atresia with ventricular septal defect (PA/VSD). MethodsA retrospective study was conducted on patients diagnosed with PA/VSD who underwent primary right ventricular-pulmonary artery connection surgery at our center between 2010 and 2020. Patients were categorized into two groups based on the type of right ventricular-pulmonary artery connection: a pericardial tube group and a patch expansion group. Clinical data and imaging findings were compared between the two groups. ResultsA total of 51 patients were included in the study, comprising 31 males and 20 females, with a median age of 12.57 (4.57, 49.67) months. The pericardial tube group included 19 patients with a median age of 17.17 (7.33, 49.67) months, while the patch expansion group consisted of 32 patients with a median age of 8.58 (3.57, 52.72) months. In both groups, the diameter of pulmonary artery, McGoon index, and Nakata index significantly increased after treatment (P<0.001). However, the pericardial tube group exhibited a longer extracorporeal circulation time (P<0.001). The reoperation rate was notably high, with 74.51% of patients requiring further surgical intervention, including 26 (81.25%) patients in the patch expansion group and 12 (63.16%) patients in the pericardial tube group. No statistical differences were observed in long-term cure rates or mortality between the two groups (P＞0.005). Conclusion In patients with PA/VSD, both patch expansion and pericardial tube right ventricular-pulmonary artery connection serve as effective initial palliative treatment strategies that promote pulmonary vessel development and provide a favorable foundation for subsequent radical operations. However, compared to the pericardial tube approach, the patch expansion technique is simpler to perform and preserves some intrinsic potential for pulmonary artery development, making it the preferred procedure.
All four patients were female, with an average age of 28.8 days and an average weight of 3.64 kg. Only case 4 was born prematurely at 34 W+5 and was treated conservatively until 71 days to complete operation. All the others completed primary corrective surgery in the neonatal period, and all survived after operation. Two different surgical techniques were used to repair the aortic-pulmonary window and the aortic origin of the right pulmonary artery, including 1 case using the aortic internal baffle technique and another 3 cases underwent replantation of the right pulmonary artery (1 case was reconstructed in situ, and the other 2 cases were reconstructed by moving the right pulmonary artery in the anterior of aorta). Case 2 who received aortic internal baffle technique underwent two reoperation because of right pulmonary artery stenosis. While, right pulmonary artery of cases 3 and 4 developed well after being reconstructed the right pulmonary artery anterior translocation. After the diagnosis of Berry syndrome through cardiac color ultrasound and CT angiography, a primary surgical radical treatment should be performed as soon as possible at an experienced pediatric cardiac center. Although the surgery is high-risk and complex, it is safe and effective.