Abstract: Eisenmenger’s syndrome is a kind of pathophysiologic syndrome that occurs in the end stage of congenital heart disease. The diagnosis of Eisenmenger’s syndrome is clear, but its early clinical presentations are nonspecific and its prognosis is poor. Recently, the therapeutic regimen is steadily improved by the endless efforts that lots of scientists have made. Treatments including new drugs, modified surgery, extracorporeal membrane oxygenation, transplantation and gene therapy can not only relieve symptoms, correct abnormal pulmonary hemodynamics, but also can improve prognosis to some extent. We will review and introduce the advance of research on Eisenmenger’s syndrome, including its pathogenesis, diagnosis and treatment.
Objective To summarize our clinical experience of pulmonary artery banding （PAB） for the treatment of complex congenital heart diseases as a palliative procedure.?Methods?From January 1997 to November 2010, 138 patients with complex congenital heart diseases underwent PAB in Fu Wai Hospital. There were 87 male patients and 51 female patients with their age of 22.2±26.5 months and average body weight of 7.5±4.6 kg. All the 138 patients were divided into 3 groups according to the purpose of PAB：left ventricular retraining （group 1, n=55）, initial procedure for functional single ventricle with unobstructed pulmonary blood flow （group 2, n=32） and initial palliative procedure followed by later biventricular repair （group 3, n=51）. The intraoperative and postoperative clinical parameters of all participants were observed, and follow-up was made for these 3 groups of patients.?Results?The in-hospital mortality of PAB was 5.1% （7/138）. Three patients underwent re-banding procedure to adjust the size of banding. In group 1, there was 1 postoperative death. Among the 55 patients, 36 patients with dextro-transposition of great arteries received PAB at an average age of 19.6±29.5 months, 29 patients of whom underwent concomitant modified Blalock-Taussig shunt. After an average training interval for 42 days, 83.3% of them（30/36）successfully received arterial switch operation. The other 19 patients in group 1 with isolated corrected transposition of great arteries received PAB at an average age of 45.3±27.2 months. Afteran average training interval for 9 months, 42.1% of them （8/19） successfully received double-switch operation. In group 2, there was 2 postoperative death. Thirty-two patients with functional single ventricle and unobstructed pulmonary blood flow received PAB at an average age of 14.1±14.9 months. Their postoperative mean pulmonary artery pressure decreased significantly from 34.00±10.00 mm Hg to 23.00±7.40 mm Hg, and their oxygen saturation of blood （SpO2） significantly decreased from 92.60%±5.90% to 83.30%±6.30%. After a median interval of 2 years, 18.8% of them （6/32） underwent right heart bypass operation. In group 3, there was 4 postoperative death. Fifty-one patients received PAB at an average age of 20.60±25.60 months. After PAB procedure, the ratio of systolic pulmonary artery pressure and systolic blood pressure significantly decreased from 0.81±0.14 to 0.46±0.15, and their SpO2 significantly decreased from 93.10%±7.60% to 85.00%±10.00%. After a median interval of 6 months, 23.5% of them （12/51） received biventricular repair.?ConclusionAlthough PAB is a palliative procedure with comparatively high risks, it still plays an indispensable role in terms of protecting pulmonary vascular beds, retraining ventricular function and two-stage surgical correction for the treatment of complex congenital heart diseases.
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.
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 investigate the results of the surgical treatment of adult congenital heart disease (ACHD). Methods We retrospectively analyzed the clinical data of 502 patients with ACHD underwent operation treatment in Fu Wai Hospital between January 2012 and June 2015. There were 205 males and 297 females at age of 18-65(34.01±11.97) years and weight of 19-96 (58.60±12.60) kg. Results Atrial septal defect had the highest incidence of ACHD, the proportion accounted for 43.8%.The followed was ventricular sepal defect, accounting for 26.6%. And the third is tetralogy of Fallot, accounting for 5.4%. The age ranged from 18 to 65 years. A percentage of 65.13% patients received operation when they are younger than 40 years, with the most patients at age of 20-29 years, the least patients at age of 30-39 years. We completed a total of 471 patients of early orthodontic treatment with operation success rate at 99.57%. We completed 29 patients palliative operation with early operation success rate at 96.55%. And there were 2 patients with re-operation at success rate of 100.00%. The postoperative patients with ventilator assisted time was 19.03 h. The mechanical ventilation time was 19.03 h. ICU treatment time was 2.22 d. Postoperative complications occurred in 51 patients with incidence rate at 10.15%. Conclusion It is very important to impose proper surgical method on different patients. The operation doctor should be trained skillfully. Perioperative management is also a key stage to the success of the operation.
Feature extraction methods and classifier selection are two critical steps in heart sound classification. To capture the pathological features of heart sound signals, this paper introduces a feature extraction method that combines mel-frequency cepstral coefficients (MFCC) and power spectral density (PSD). Unlike conventional classifiers, the adaptive neuro-fuzzy inference system (ANFIS) was chosen as the classifier for this study. In terms of experimental design, we compared different PSDs across various time intervals and frequency ranges, selecting the characteristics with the most effective classification outcomes. We compared four statistical properties, including mean PSD, standard deviation PSD, variance PSD, and median PSD. Through experimental comparisons, we found that combining the features of median PSD and MFCC with heart sound systolic period of 100–300 Hz yielded the best results. The accuracy, precision, sensitivity, specificity, and F1 score were determined to be 96.50%, 99.27%, 93.35%, 99.60%, and 96.35%, respectively. These results demonstrate the algorithm’s significant potential for aiding in the diagnosis of congenital heart disease.
Abstract: Objective To investigate the safety and feasibility of fast track (FT) treatment in young children with atrioventricular septal defect (CAVSD) and pulmonary artery hypertension (PAH) following surgical repair. Methods A total of 51 young children patients including 24 boys and 27 girls with age at 12.5±8.9 months from 4 to 36 months, underwent CAVSD repair in the pediatric surgery department of Fu Wai Hospital from January 2006 to March 2009. Among them, 21 patients were administered FT management. PICU length of stay and the rate of reintubation were analyzed retrospectively and the decrease of pulmonary artery pressure (PAP) after operation was also measured. Results Twentyone patients under FT treatment were extubated within 8 hours after operation. The mean pulmonary artery pressure(MPAP) decreased significantly after surgery (39.59 mm Hg vs.24.50 mm Hg,t=5514,Plt;0.05). PICU length of stay was 2.05±0.87 d (18 h-3 d). One patient was reintubated due to lung infection, which had nothing to do with the FT treatment. During the followup which lasted for 3 to 6 months, 21 patients had good heart function with no reoperation or death. Conclusion FT treatment is safe and feasible to some CAVSD patients associated with PAH, and shorter PICU length of stay can be achieved. The validation of FT model for the CAVSD patients with severe PAH needs research with large sample.
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.
Objective To investigate the value of systemic-normothermic/cardiac-hypothermic cardiopulmonary bypass（CPB）on operation of congenital heart disease. Methods Thirty patients of congenital heart disease were randomly divided into two groups, the normothermia group（n=15）and hypothermia group（n=15）. The changes of CPB time, aortic cross-clamp time,operation time and postoperative drainage and the value of blood cell were observed. Results The duration of CPB （37. 5 ±11. 6rain vs. 51. 6± 12. 0 min, P〈0. 05） and operation time （2.2± 0.6h vs. 2. 7±0. 5h, P〈0. 01） in normothermia group were shorter than those of hypothermia group statistically, the differences of postoperative drainage and the value of blood cells between two groups were not statistically significant. Conclusion The use of systemic-normothermic/cardiac-hypothermic CPB on operation of congenital heart disease shows that the time of operation is shorter remarkly , and it could be clinically used safely.
Objective To investigate the hospital outcomes and therapeutic strategy for multiple organ dysfunction syndrome （MODS） in children after cardiac surgery. Methods Seventy-seven consecutive pediatric patients （57 male/20 female, age 3.47±3.67 years, weight 13.08±7.52 kg） with MODS after cardiac surgery were enrolled in the study from 1999.7 to 2005.10. Corrective and palliative operation were performed in sixty-six patients and eleven patients, respectively. We evaluated the clinical score for all study patients according to the extent of organ injury. Results The overall mortality rate was 28. 6%（22/77）. （1） Cardiovascular, renal, hepatic, hematologic, neurologic and respiratory dysfunction was present in 100% （77/77）, 97.4% （75/77）, 84.4% （65/77）, 48.1%（37/77）, 45. 5%（35/77） and 44. 2%（34/77） of the patients, respectively. Cardiac injury appeared much earlier than other organs （P〈0. 05）. （2） Mortality rate with two, three, four, five and six dysfunctional organ systems was 0%, 12.5 %, 31.8 %, 42. 9 % and 87.5 %, respectively （r=0.487, P〈0. 001 in trend）. Furthermore, there was a positive correlation between the clinic score and mortality rate （r=0.603, P〈0. 001）. （3） Compared with survivors, non-survivors had longer cardiopulmonary bypass time, clamping time, higher incidence of accidental events and cardiopulmonary resuscitation during and after surgery （P〈0. 05）. Conclusion Mortality associated with MODS was highly correlated with the number of organ failing and clinical score. Cardiac dysfunction was the primary disease in MODS after cardiac surgery. Therefore, therapeutic strategy for MODS should be focused on management of primary disease, as well as providing consecutive evaluation and improvement for organ function.