Abstracts： Objective To summarize clinical experience and surgical outcomes of congenital coronary arterial fistula （CAF）. Methods We retrospectively analyzed clinical records of 12 patients （6 males, 6 females）, aged from 4 to 77 （50.90±23.8） years, who underwent surgical repair of CAF in Nanjing First Hospital between February 2005 and June 2011. There were 3 CAF patients associated with coronary artery aneurysms, one with patent foramen ovale and 2 with coronary artery disease （CAD）. One CAD patient had concomitant severe aortic valve stenosis. One patient underwent surgical repair without cardiopulmonary bypass （CPB） and 11 patients underwent surgery under CPB, among whom 3 patients underwent surgery with beating heart. One patient underwent concomitant aortic valve replacement and coronary artery bypass grafting.?Results?All the patients recovered uneventfully. Operation time was 151.25±42.65 min （ranging from 90 to 245 min）, cardiopulmonary bypass time was 65.06±29.16 min （ranging from 31 to 116 min）, mean aortic cross-clamping time was 43.00±33.41 min （ranging from 18 to 97 min） and postoperative hospital stay was 12.50±1.45 d （ranging from 10 to 15 d）. There was no early or late death. All the patients were followed up from 4 months to 6 years and no patient had symptom recurrence, myocardial ischemia or residual fistula during the follow-up. Conclusions All CAF patients should be surgically treated once diagnosis are made with satisfactory surgical outcome.
ObjectiveTo observe the mutation and expression of Nkx2.5 in congenital heart disease patients with diminutive pulmonary blood. We preliminarily explored the association between Nkx2.5 gene and pathogenesis of congenital heart disease patients with diminutive pulmonary blood.
MethodsFifty six patients of congenital heart disease with diminutive pulmonary blood in the first affiliated hospital of Bengbu medical college and Anhui province children, s hospital between May 2012 and May 2014 were as an experimental group. Sixty three patients of ventricular septal defect were as a control group. In the trial group, there were 30 males and 26 females averagely aged 5.82± 4.23 years ranking from 6 months to 14 years. In the control group, there were 36 males and 27 females averagely aged 6.93± 4.56 years ranking from 6 months to 14 years. Before operation, peripheral venous blood of all the patients were collected. We used polymerase chain reaction combined with DNA sequencing technology to detect Nkx2.5 gene exon sequence and to analyze the association between Nkx2.5 gene mutation and congenital heart disease with diminutive pulmonary blood. And we got some hypertrophic myocardial tissue from right ventricular outflow tract in the operation, whose size was 0.5× 0.5× 0.5 cubic centimeter. And we extracted myocardial tissue RNA. The expression changes of Nkx2.5 gene mRNA were detected by real-time fluorescence quantitative polymerase chain reaction technique.
ResultsThere was no mutations tested out in the peripheral venous blood in both two groups. The expression of mRNA in Nkx2.5 gene of the trial group was lower than that in the control group with a statistical difference.
ConclusionNkx2.5 gene mutation may be associated with multiple factors. The occurrence of congenital heart disease with diminutive pulmonary blood may be related with a decline of Nkx2.5 gene expression in the myocardial tissue.
ObjectiveTo reveal the risk factors for delayed recovery and complications in infants with weight≤5.0 kg after surgical ventricular septal defect (VSD) closure.MethodsWe retrospectively reviewed a consecutive series of 86 patients with weight≤5.0 kg who were admitted to our institution for surgical VSD closure between January 2016 and July 2019, including 31 males and 55 females with an age of 17-266 (80.3±40.4) d and a weight of 2.5-5.0 (4.4±0.6) kg. The VSDs were divided into perimembranous (n=65, 75.6%), subaortic (n=17, 19.8%) and subaortic combined muscular types (n=4, 4.7%). Mechanical ventilation (MV) time≥24 h or ICU stay≥72 h were defined as delayed recovery. Death, sudden circulatory arrest, complete heart block requiring a permanent or temporary pacemaker implantation, neurological complications, reoperation (for residue shunt or valvular regurgitation), reintubation and diaphragmatic paralysis were considered as significant major adverse events.ResultsThere was no death, reoperation due to residual VSD or neurological complication. Totally 51 (59.3%) patients had MV timec≥24 h and 51 (59.3%) patients stayed in the ICU≥72 h. Two (2.3%) patients required temporary pacemaker and six (7.0%) patients required reintubation. During the follow-up of 3-36 (15.8±8.8) months, 1 patient died of pneumonia after discharge, 5 patients suffered mild tricuspid valve regurgitation and 1 patient suffered decreased left ventricular systolic function in the follow-up. No aortic valve injuries occurred.ConclusionFor patients whose weight≤5.0 kg, short-term results of surgical VSD closure are excellent. Low weight and age may prolong MV time; low birth weight and pulmonary hypertension may prolong ICU stay, but are not independent risk factors.
ObjectiveTo explore the method and feasibility of establishing patent ductus arteriosus (PDA) model in Bama miniature pig by using autologous jugular vein, and to provide a large animal model for the development of PDA occluder and the study of pulmonary hypertension associated with congenital heart disease. MethodsFive male Bama miniature pigs weighing about 45 kg were selected to gain the PDA model of the autogenous jugular vein, which was fixed by glutaraldehyde and anastomosed between the ascending aorta and the main pulmonary artery. The patency of PDA was confirmed by echocardiography and angiocardiography immediately and one week after the operation. Two animals were selected to undergo transcatheter closure of PDA via femoral vein 1 week after the operation, and the rest were euthanized to obtain PDA and lung tissue for pathological examination. ResultsThe PDA model was successfully established in all five animals with a success rate of 100.0%. Immediately and 1 week after the operation, echocardiography and angiography showed that PDA blood flow was unobstructed, and hematoxylin-eosin staining showed that PDA endothelialization was good. One week after the operation, two animals were successfully treated with transcatheter femoral vein occlusion. The pathological examination of lung tissue showed thickening of the intima and muscular layer of pulmonary arterioles, thickening of pulmonary interstitium and infiltration of neutrophils. ConclusionIt is safe and feasible to establish a large animal model of PDA by using autogenous jugular vein anastomosis between the ascending aorta and the main pulmonary artery. The model can be used for the development of PDA interventional occlusive devices and the pathophysiological study of congenital heart disease-related pulmonary hypertension.
Objective To compare the clinical effect between high flow rate modified ultrafiltration （HMUF） and conventional modified ultrafiltration（CMUF）, and the effect on hemodynamic data and inflammatory mediators. Methods Forty children were divided into two groups with random number table, HMUF group and CMUF group, 20 cases each group. Hematocrit （HCT） and hemodynamic changes were recorded and the concentration of tumor necrosis factor （TNF） and interleukin 6 （IL-6） were measured. Results The operations were done uneventfully with moderate hypothermia cardiopulmonary bypass in 40 patients. Duration of ultrafiltration of HMUF group （7.83±0. 75 min） was less than that of CMUF group （13.86±1.95 min, P〈0.01）. The volumes ultrafiltrated of HMUF group （440.00±91.86ml） was more than that of CMUF group （372.22±56.52ml, P〈0.01）. There are no significant differences about the hemodynamic data, HCT, TNF and IL-6 between two groups. Conclusion The HMUF is safe and efficient,when it was used after pediatric cardiopulmonary bypass, the duration of ultrafiltration can be shortened significantly.
Objective To study the feasibility, indication, technique of emergency operation and the perioperative management on critical congenital heart disease in infants. Methods From May 1998 to May 2003, 67 emergency operations of critical congenital heart disease were performed in infants . The age was 14d-32 months (mean , 11.8±8.9 months), the weight was 2.6-14.8 kg( mean, 8.4±3.0 kg). The diagnosis included ventricular septal defect in 39, tetralogy of Fallot in 13, complete atrioventricular septal defect in 5, total anomalous pulmonary venous drainage in 4, atrial septal defect with pulmonary valve stenosis in 3, D-transposition of the great arteries in 2, and postoperative pulmonary valve vegetation in 1. Corrective operation in 64 and palliative operation in 3 were done. Results The preoperative managing time was 0-9 d(mean , 3.7±2.6 d), the lasting time of intubation was 1-14d( mean, 3.8±3.2 d), and ICU staying time was 2-18 d (mean, 5.7±2.8 d). The operative mortality was 7.5% (5/67), the postoperative complications in other infants include 14 low cardiac output syndrome, 14 atelectasis, 8 lung infection, 2 worsened pulmonary hypertension, 1 pneumothorax , and 1 phrenic nerve paralysis. Following up 2-60 months with 1 case late death, the cardiac function of the others were class Ⅰ-Ⅱ/Ⅵ, the operative result was satisfactory. Conclusions The emergency operation on critical congenital heart disease in infants is safe, and can save most of their lives, the good result can be obtained from surgical treatment. Accurate choice of indications, and perioperative good nutritional and cardiorespiratory function support are important.
Objective To observe the effect of glutamine （Gln） on intestinal permeability after surgery of children, also its influence on the plama level of interleukin-2（IL-2）, endotoxin and synthesize of protein through a random nutrition trial. Methods Twenty children suffered from congenital heart disease were divided into Gln group and control group with random number table, 10 cases in each group. They were all given isonitrogenous and isocaloric total paraenteral nutrition after 24 h postoperatively. In Gln group the Dipeptiven [-N （2）-L-alanyl-Lglutamine] was used with 2 ml/kg · 24h additionly. Before operation, 24h and 96 h after operation, intestinal permeability, serum level of endotoxin, IL-2, C-reaction protein, prealbumine were measured. Results Intestinal permeability increased in 24 h after cardiac surgery in two groups, while the concentration of endotoxin also increased, 96 h after surgery the intestinal permeability recovered, but the endotoxin level did not decrease in control group （P〈0. 01）. Conclusion Utilization of Gln can improve immune suppression, elevate the IL-2 level, decrease the endotoxin concentration, alleviate the infection, but has no effect on the protein synthesis after congenital cardiac operation of children.
ObjectiveTo identify the pulmonary artery growth, restenosis and regurgitation of the valve after right ventricle outflow (RVOT) reconstruction with pericardial tube in patients with pulmonary atresia and ventricle septal defect (PA/VSD).
MethodsWe retrospectively analyzed the clinical data of 41 patients with PA/VSD undergoing PA/VSD repair to reconstruct RVOT for radical or palliative repair in our hospital from November 2002 through September 2013. There were 25 males and 16 females with operation age of 4.00 months to 22.70 years (56.60±63.92 months). Late pulmonary artery growth, pulmonary artery, and tricuspid regurgitation of the patients were followed up. Pulmonary atresia and ventricular septal defect repair with pericardial tube were performed in the patients.
ResultsThere were 5 (12.19%) early hospital deaths. Thirty-three patients were followed up for 4.00 months to 10.75 years (3.00±2.35 years). Three patients (7.31%) were lost during the follow-up.One patient was dead after stageⅡsurgery. There was no significant growth on the diameters of the tube and the pulmonary artery branches during the follow-up. There were 10 patients with severe stenosis in pericardial tube and 5 patients with moderate or severe stenosis in pulmonary artery branches. The echocardiography suggested the pulmonary artery and tricuspid regurgitation were more serious (P<0.05). No correlation was found between regurgitation quantity and follow-up time.
ConclusionThe early postoperative results is satisfactory. However, there is no potential growth on the pericardial tube. So the patients should be followed up closely for restenosis.
Corresponding author: XU Zhi-wei, E-mail: zwxumd@online.sh.cn
Abstract: Objective To compare the two different ways of right ventricle pulmonary artery (RV-PA) reconstruction at repair of persistent truncus arteriosus(PTA), the direct RV-PA anastomosis and extra conduit connection, in order to find the better way. Methods From Feb. 2000 to Sept. 2006, 23 patients had undergone the repairs of truncus arteriosus in our hospital, age at operation from 1.5 to 63.3 months. Patients were divided into 2 groups according to the way of RV PA reconstruction. Group Ⅰ : 18 of them, using direct RV-PA anastomosis, group Ⅱ : 5 of them, using valved homograft or Gore-Tex conduit. 3 patients were associated with interrupted aortic arch (IAA). Kaplan-Meier was used to calculate postoperative mortality, survival time and re-operation situation. Paired t-test and group t-test were used to evaluate late pulmonary growth and cardiac function.Results There were 2 early hospital death, there were 17 patients in follow-up for 2.14 ± 1.97y (32.00d-6.95y). No later death during follow-up. Total survival rateo was 91.30%(21/23), 95% CI of survival time was 5.55-7.15y. Survival ratio of group Ⅰ was 94.40%, and that of group Ⅱ was 80%. One patient had undergone re operation for right ventricular outflow tract obstruction (RVOTO). The difference between the diameter of postoperative RV-PA anastomosis was statistically significant. The early diameter of group Ⅰ was 1.01 ± 0.26cm, later was 1.32 ± 0.45cm(P=0.019). The velocity of flow at the position of anastomosis and the peristome of right pulmonary artery (RPA)/left pulmonary artery (LPA) was acceptable. Compared the postoperative cardiac function, late left ventricle ejection fraction (LVEF) really improved with a significant difference ［ group Ⅰ , early was 62.82%, late was 69.87%(P=0.026); group Ⅱ , early was 58.17%, late was 64.00%(P=0.029) ］ . No re-operation for truncal valve regurgitation was needed. Conclusions The postoperative survival and follow-up results are satisfactory. A direct anastomosis of RV-PA continuity has the potential for right ventricle outflow tract (RVOT) growth and associated with low rate of pulmonary artery and bifurcation obstruction. The heart function is really improved during follow-up. IAA and truncal valve regurgitation are two major risk factors of associated with hospital death.
Objective Summarize and review on surgical repair of congenital heart disease in infants. Methods Between January 1988 and June 2003, seven hundred and eighty seven less than 6 months of age patients were operated. There were 109 cases of complete transposition of the great arteries(D-TGA), 51 total abnormal pulmonary venous connection(TAPVC), 16 pulmonary atresia with ventricular septal defect(VSD), 33 coarctation of aorta, 299 VSD with pulmonary hypertention, 44 tetralogy of Fallot, 23 double outlet right ventricle, 9 pulmonary atresia with intact ventricle septum, et al. The operative procedure was dependent on different disease. Results There were 77 patients died, the total mortality was 9.78%(77/787). Following improvement of surgical procedure, the mortality was decreased from 25% in 1988-1995 to 4.11% in 2003. In the D-TGA patients, there was one residual VSD who was repaired 3 months late, and two had mild pulmonary and aortic supravalve obstruction. They were still be followed up. Two patients with intracardiac type of TAPVC had venous return obstruction, one was died and another was re-operated 4 days later. In the VSD patients, there were 5 patients with residural VSD, but all of them did not need to repair. Conclusion The time of surgical repair is very important, especially for complex congenital heart disease. The best operative procedure will be lost, if the infants is repaired too late, the postoperative events and mortality will be increased.
