Objective To investigate the impact of three kinds of palliative operation on the body and growth of pulmonary artery in patients with congenital heart diseases of diminutive pulmonary blood. Methods Clinical data was reviewed in 28 cases of congenital heart diseases with diminutive pulmonary blood who had been performed cavopulmonary connection （n = 9）, systemic-pulmonary shunt （n = 8 ）, and palliative reconstruction of right ventricular outflow tract （n=11）. The period between re-hospitalized and the first was 5-54 months （19.07±10. 06 months ）. Hematocrit （HCT）, hemoglobin （Hb）, percutaneous oxygen saturation （SpO2）, body surface area （BSA）, and pulmonary artery index （PAI） etc. were observed both before palliation and before the second operation. Results After the second hospitalization, there were 7 cases of death from hemorrhage, failure of circulation and extracorporeal circulation accident etc. The time of respirator, intensive care unit and total amount of dopamine in patients of palliative reconstruction of right ventricular outflow tract were longer and more than those in patients of cavopulmonary connection （P〈0. 05）. HCT, Hb before the second operation were decreased than thoes before palliative operations in all patients, SpO2, BSA and PAI increased significantly （P 〈 0. 01 ）. Before the second operation, BSA of patients with cavopulmonary connection, BSA and PAI of patients with systemic-pulmonary shunt, SpO2, BSA and PAI of patients with palliative reconstruction of right ventricular outflow tract were increased than those before palliative operations（P〈0. 01）. HCT of palliative reconstruction of right ventricular outflow tract was decreased（P〈0. 05）. Conclusion This results suggests that pulmonary blood of patients with congenital heart diseases of diminutive pulmonary blood can be increased, development of pulmonary arteries can be improved efficiently by systemic-pulmonary shunt and palliative reconstruction of right ventricle outflow tract, but it can not be found in cavopulmonary connection patients.
Anomalous left coronary artery from pulmonary artery (ALCAPA) is a rare congenital heart disease which can induce heart failure in the early period and finally results in death. Surgery is the only way to correct the malformation. Surgical advances and heart assist devices such as extracorporeal membrane oxygenation (ECMO) widespreadly used recently achieve good clinical outcome. However, there exists a dispute about the indications and pattern of operation. The outcome of long-term follow-up is not good. This article reviewed the researches about ALCAPA in terms of operative pattern, the operative pattern and long-term 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.
Objective Comparing postoperative change of blood gas and hemodynamic status in patients underwent a right ventricletopulmonary artery (RVPA) conduit or a modified BlalockTaussig (mBT) shunt for pulmonary atresia with ventricular septal defect and without major arterial pulmonary collaterals (MAPCAs), to affirm the effect on oxygen supply /demand with different procedure. Methods From July 2006 to October 2007, 38 patients with pulmonary atresia and ventricular septal defect without MAPCAs were divided into two groups according to different procedures: RVPA group (n=25) and mBT group (n=13).Perioperative mortality, blood gas and hemodynamic data during postoperative 48 hours, including heart rate, blood pressure, systemic oxygen saturation, mixed venous oxygen saturation, oxygen excess factor, inotropic score were compared in both groups. Results The difference in the mortality between RVPA group (4.0％，1/25) and mBT group （7.7％，1/13) showed no statistical significance（Pgt;0.05）. The total of 33 patients were followed up, the followup time was from 6 to 18 months.11 patients (4 patients in mBT group, 7 patients in RVPA group) underwent corrected procedures during 9 to 18 months after palliative procedures, one case died of elevated pulmonary vascular resistance and right ventricle failure. The mixed venous oxygen saturation at 24h and 48h after surgery were higher than that at 6h after surgery (Plt;0.01) both in RVPA group and mBT group. The systolic blood pressures at 6h, 24h, 48h after surgery in RVPA group were lower than those in mBT group (P=0.048，0.043， 0.045)，the mean systemic blood pressures in RVPA group were higher than those in mBT group (P=0.048, 0.046, 0.049),the diastolic blood pressures in RVPA group were higher than those in mBT group (P=0.038, 0.034, 0.040)， the inotropic scores in RVPA group were lower than those in mBT group (P=0.035， 0.032，0.047). Conclusion The blood pressures and inotropic scores are found significantly different in RVPA conduit and mBT procedures, while postoperative systemic oxygen delivery areequivalent. Both RVPA and mBT patients decline to nadir in hemodynamic status at 6 h after surgery.
ObjectiveTo summarize and analyze the experience of subarterial ventricular septal defect (VSD) repaired with simple pulmonary artery approach.MethodsWe retrospectively anlyzed the clinical data of 102 patients with subarterial VSD repaired with simple pulmonary artery approach in our hospital from August 2015 to October 2018. There were 67 males and 35 females at median age of 3 years (ranging 4 months to 49 years).ResultsThe median operation time was 82 (54-136) min. Median cardiopulmonary bypass time was 36 (21-62) min. The median aortic cross-clamping time was 13 (7-32) min. Thirty two patients of tracheal intubation were removed from the fast-track operating room immediately after surgery. Of the 102 patients, 67 patients underwent a small incision in the lower sternum. The median postoperative ICU stay time was 26 (13-36) h. There was no planned reoperations and no early death.ConclusionSimple pulmonary artery approach for subarterial ventricular septal defect repair with less intracardiac procedures, short operation time, less trauma, quick postoperative recovery has certain advantages in the application of specific groups.
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.
ObjectiveTo summarize the surgical experience of patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) and intramural left coronary artery and analyze the early and mid-term clinical outcomes. Methods The infants with ALCAPA and intramural left coronary artery who underwent surgical treatment in Fuwai Hospital from January 2014 to September 2020 were retrospectively enrolled, and the clinical data of the patients were analyzed. Results A total of 10 patients were included. There were 8 males and 2 females, with a median age of 7.5 (3-46) months at surgery. The surgical techniques included coronary unroofing in 7 patients, coronary unroofing with coronary reimplantation in 2 patients, and coronary unroofing with ligation of left coronary artery ostium in 1 patient. Seven infants received additional procedures, including 5 mitral valve repair. Delayed chest closure was required in 2 infants, and no operative death or major complications occurred in the whole group. Postoperative chest radiograph showed that the mean cardiothoracic ratio was lower than that before surgery (0.62±0.05 vs. 0.67±0.06, P=0.006). Postoperative echocardiography indicated that the mean left ventricular ejection fraction was increased than that before surgery, but there was no statistical difference (38.7%±15.9% vs. 30.0%±16.1%, P=0.066). The follow-up was available for all 10 survivors, with an average follow-up time of 13-92 (46.6±25.0) months. During the follow-up period, the patients had no obvious symptoms, death, coronary complications or other major complications. The chest radiograph at last follow-up showed that the mean cardiothoracic ratio was further decreased (0.60±0.07 vs. 0.62±0.05, P=0.024). The echocardiography at last follow-up showed that the mean left ventricular ejection fraction was further improved (60.1%±9.3% vs. 38.7%±15.9%, P=0.002). Conclusion ALCAPA with intramural left coronary artery is a rare malformation. It can be treated with different surgical techniques with satisfactory early and mid-term outcomes.
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.
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.
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.