Objective To investigate the accuracy of pulse oximetry screening for congenital heart disease （CHD） in asymptomatic children. Methods We electronically searched VIP database，CNKI database，CBMdisc，Wanfandatabase，PubMed，Excerpta Medica Database （EMBASE） and the Cochrane Library from the time of database establishment to February 2013 to identify literatures regarding pulse oximetry screening for CHD in asymptomatic children. Data extraction was performed by two researchers independently. Quality of the included literatures was evaluated with qualityassessment for diagnostic accuracy studies （QUADAS）. Meta-analysis was performed using RevMan 5.1.1. ResultsTwelve studies were included in this study with a total of 137 582 newborns. Fourteen QUADAS criteria were fulfilled by all the 12 studies. Among the 12 studies，only the studies with positive test results received verification using a gold standardtest，and the execution of the gold standard test was described in sufficient detail only in 1 study. There were 10 studies in which the gold standard test results were interpreted with knowledge of the diagnostic test results. Meta-analysis showed that pooled sensitivity and specificity of pulse oximetry test for the diagnosis of CHD were 22% with 95% CI （19%，25%） and 99% （99%，99%） respectively. Pooled positive likelihood ratio （LR＋） and negative likelihood ratio （LR-） were 157.30 with 95% CI （11.80，2 096.95） and 0.61 with 95% CI （0.46，0.82） respectively. Pooled diagnostic odds ratio was 398.25 （34.5，4 596.81）. The area under summary receiver operating characteristic （SROC） curve was 0.809，and Q index was 0.744. Conclusion Pulse oximetry is a lowly sensitive and highly specific diagnostic method for children with CHD，and is helpful for early diagnosis of CHD.
Abstract： Objective To summarize early clinical result of total cavopulmonary connection, and analyze the risk factors contributing to prolonged postoperative recovery. Methods Between February 2009 and August 2010, 58 patients with functional univentricular complex congenital heart disease received total cavopulmonary connection in Beijing Fu Wai Hospital. All of them were diagnosed by echocardiogram and angiography including 26 patients with single ventricle, 10 patients with tricuspid atresia, 4 patients with pulmonary artery atresia, 5 patients with double outlet rightventricle, 1 patient with transposition of great arteries, and 12 patients with corrected transposition of the great arteries.Fifty seven patients underwent extracardiac conduit total cavopulmonary connection, and only one patient underwent total cavopulmonary connection with an intracardiac lateral tunnel. According to their postoperative pleural drainage volume and duration, these 58 patients were divided into a large pleural drainage volume group (17 patients with 10 males and 7 females, mean age of 8.61±6.73 years）that included patients with large volume and long duration of pleural drainage, and a little pleural drainage volume group (41 patients with 15 males and 26 females, mean age of 7.21±4.24 years) . A univariable analysis was conducted to compare the risk factors that effected recovery result between the two groups. Results There was no death in hospital period. The average length of hospital stay was 12.30±9.80 d . Average drainage time (18.00±5.50 d versus 5.00±2.20 d , t= -1.967, P ＜ 0.05）, drainage volume (12.30±2.60 ml/(kg·d) versus 2.80±1.70 ml/(kg·d), t=-3.221, P ＜ 0.05), and hospital stay (20.10±7.20 d versus 7.20±1.10 d, t=-2.003, P ＜ 0.05） of the large pleural drainage volume group were significantly larger or longer than those of the little pleural drainage volume group. Univariate analysis showed that preoperative pulmonary pressure measured by catheter in the large pleural drainage volume group was significantly higher than that in the little pleural drainage volume group (17.42±5.34 mm Hg versus 13.91±5.22 mm Hg，t=-2.073，P ＜ 0.05). Conclusions The mortality and major morbidities after total cavopulmonary connection are low in the current era. Preoperative high pulmonary pressure is a risk factor for large amount of pleural drainage and prolonged recovery.
Abstract： Objective To analyze the early outcomes of open heart surgery for congenital heart diseases in sixty low birth weight infants and premature infants. Methods Sixty low birth weight infants （body weight＜2 500 g） and premature infants with congenital heart diseases undergoing surgical repair from May 2003 to October 2011 were studied retrospectively in Guangdong Cardiovascular Institute. There were 43 male patients and 17 female patients with their mean gestational age of 33.5±4.1 weeks （ranging from 26 to 42 weeks） and mean age at operation of 24.9±12.5 d（ranging from 4 to 55 d）. Among them there were 47 premature infants with their mean birth weight of 1 729.3±522.5 g（ranging from 640 to 2 500 g）and mean weight at operation of 1 953.2±463.6 g （ranging from 650 to 2 712 g）. All the patients received preoperative treatment in newborn intensive care unit（NICU） and underwent surgical repair under general anesthesia, including 29 patients without cardiopulmonary bypass （CPB）and 31 patients with CPB . All surviving patients received postoperative monitoring and treatment in NICU, and their postoperative complications and in-hospital death were reported. Results A total of 13 patients died during hospitalization with a total in-hospital mortality of 21.7%（13/60）, including 4 intra-operative deaths, 6 early deaths （within 72 h postoperatively） and 3 patients giving up postoperative treatment. CPB time was 121.0±74.7 min, aortic clamp time was 74.8±44.7 min, and postoperative mechanicalventilation time was （136.9±138.1）h. Thirteen patients underwent delayed sternal closure. Eight patients underwentreexploation for postoperative bleeding. Ten patients had severe pneumonia, 2 patients had pulmonary hypertensive crisis, and 8 patients had low cardiac output syndrome. All the postoperative complications were resolved or improved after proper treatment. Follow-up was complete in 47 patients from 2 to 12 monthes, and all the patients were alive during follow-up. Conclusion Early surgical repair for low birth weight infants and premature infants with congenital heart diseases is safe and effective.
Abstract： Objective?To evaluate clinical experiences and long-term outcome of morphologic left ventricle （mLV） retraining for congenitally corrected transposition of the great arteries （cCTGA）. Methods From May 2005 to May 2011, 24 patients with cCTGA anomaly underwent left ventricle retraining by means of pulmonary artery banding in Fu Wai Hospital. There were 13 males and 11 females with their age of 0.17-22.00 （3.73±4.35） years and body weight of 5.10-61.00（15.71±10.95）kg. Major concomitant malformations included tricuspid valve insufficiency （TR）in 23 patients （mild in 11 patients, moderate in 7 patients, severe in 5 patients）, restrictive ventricular septal defect in 18 patients, atrial septal defect in 5 patients, patent foramen ovale in 5 patients, patent ductus arteriosus in 4 patients, mild pulmonary stenosis in 5 patients, and aortic coarctation in 1 patient. All the patients were preoperatively diagnosed by echocardiography, cardiovascular angiography or cardiac catheterization. The mLV end diastolic diameter （mLVEDD） was 8-32（21.56±6.60）mm, posterior wall thickness of mLV was 2-7 （4.29±1.52）mm , mLV to morphologic right ventricle （mRV） pressure ratio （mLV/mRV） was 0.12-0.65 （0.41±0.12）. Pulmonary artery banding operation was performed through upper partial sternotomy or median sternotomy without circulatory arrest. Results The mLV/mRV pressure ratio reached to 0.57-0.93 （0.76±0.10） under direct pressure monitoring after surgery. There was no in-hospital death in this group. Echocardiography before discharge showed that the structure and function of the two ventricles were good, the interventricular septum moved partially towards mRV, mLVEDD was increased slightly, and there was a tendency of reduced TR. Postoperative follows-up was from 1 to 35 months, and there was no late death during follow-up. All the patients were in good general condition with stable vital signs and New York Heart Association （NYHA） classⅠ-Ⅱ. The mLVEDD was 14-40 （26.17±7.11） mm, posterior wall thickness of mLV was 4-9 （4.95±1.44）mm, mLV/mRV pressure ratio was 0.52-0.98 （0.72±0.16） , and TR was significantly decreased. Fourteen patients successfully underwent staged complete double-switch procedure. Conclusion Left ventricle retraining is a safe and effective method to train mLV for cCTGA patients. Pressure load and posterior wall thickness of mLV are increased, mLV cavity is dilated, and TR is significantly reduced after the surgery.
Abstract： Objective To summarize our experience of surgical treatment for anomalous origin of one pulmonary artery in infants and children. Methods From March 2005 to May 2010，11 patients with anomalous origin of one pulmonary artery and other concomitant congenital cardiovascular malformations underwent surgical repair in Xijing Hospital of Fourth Military Medical University．The mean age of the patients was 11.5 months with a range from 2 months to 36 months．Their mean body weight was 7.1 kg with a range from 4 to 13 kg. Seven patients had anomalous origin of the right pulmonary artery from the ascending aorta, and four patients had anomalous origin of the left pulmonary artery from the ascending aorta. All the eleven patients had other concomitant intracardiac anomalies or vascular malformations as well as pulmonary hypertension, and underwent one stage surgical repair via median sternotomy under hypothermia and cardiopulmonary bypass. Results Their operation time was 169 - 293 （231±55） min, cardiopulmonary bypass time was 87-210 （138±47） min, and aortic-clamping time was 45-133 （86±28） min. There was one postoperative death who had low cardiac output syndrome after repair for tetralogy of Fallot and anomalous origin of the right pulmonary artery. The overall postoperative mortality was 9.1％. Postoperative echocardiography of all the surviving patients showed their left and right pulmonary artery origined from the right ventricle and pulmonary artery with satisfactory malformation correction but no residual shunt and pulmonary stenosis . All the surviving ten patients were followed up with a follow-up rate of 100% and mean follow-up time of 13.5 months with a range from 3 to 32 months. Their echocardiography during follow-up showed that there was no pulmonary stenosis in all the patients, and pulmonary blood pressure significantly decreased in 9 patients. Conclusion Patients with anomalous origin of one pulmonary artery should undergo surgical repair as early as possible with satisfactory short-term outcomes in infants and children. For elder patients with irreversible pulmonary hypertension, the choice of surgical treatment should be more cautious. During the surgery, the anomalous pulmonary artery and ascending aorta should be dissociated fully, and transection of the ascending aorta is helpful to get a satisfactory operating field view for the surgeon. Repairing aortic defect with autologous pulmonary arterial patch can effectively avoid the occurrence of postoperative aortic aneurysm.
Abstract： Objective To evaluate clinical outcomes of painless flexible fiberoptic bronchoscopy in the treatment for pulmonary complications in postoperative pediatric patients with congenital heart diseases. Methods We retrospectively analyzed clinical data of 58 patients who received fiberoptic bronchoscopy for pulmonary complications after surgical repair for atrial septal defect， ventricular septal defect， tetralogy of Fallot， double outlet right ventricle or transposition of the great arteries in First Affiliated Hospital of Harbin Medical University From August 2009 to February 2012. There were 26 male patients and 32 female patients with their age ranging from 20 days to 2 years. Olympus xp-60 fiberoptic bronchoscopy was used for removal of airway secretions and bronchial alveolar lavage under anesthesia with propofol， fentanyl and lidocaine. Pulse oximetry， respiratory sound and chest X-ray changes were observed. Results The examination time of painless fiberoptic bronchoscopy was 10-25 minutes in the 58 patients. After removal of airway secretions by fiberoptic bronchoscopy， their respiratory status improved significantly， pulse oxygen saturation increased by 5%-12%， and their pulmonary crackles were significantly reduced. In 29 patients with atelectasis， their pulmonary lobes demonstrated significant reexpansion in chest X-ray reviews on the next day after fiberoptic bronchoscopy， and 8 patients received another fiberoptic bronchoscopy treatment on the next day to achieve complete pulmonary reexpansion. One patient with severe pulmonary hypertension （PH） stayed in intensive care unit （ICU） for 6 days， 3 patients with transposition of the great arteries stayed in ICU for 5 days， 3 patients with double outlet of right ventricle and moderately high PH stayed in ICU for 4 days， and all the other patients were discharged from ICU within 48 hours after admission. There was no severe complication related to fiberoptic bronchoscopy， except 2 patients with minor nasal mucosa bleeding who were cured with hemostatic drugs and local compression. Conclusion Painless flexible fiberoptic bronchoscopy can significantly enhance clinical outcomes and shorten ICU stay for postoperative pediatric patients with congenital heart diseases.
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 To study palliative surgical strategies for patients with complex congenital heart diseases， and improve their clinical outcomes and survival rate.?Methods We retrospectively analyzed clinical data of 95 patients with complex congenital heart diseases who underwent palliative surgical repair in Union Hospital of Tongji Medical College，Huazhong University of Science and Technology from January 2004 to May 2011. There were 68 male patients and 27female patients with their age ranging from 1 month to 37 years. Modified Blalock-Taussig shunt （B-T shunt） was performed in 12 patients， modified Brock’s procedure in 23 patients， bidirectional Glenn procedure in 55 patients and pulmonary artery banding in 5 patients. Surgical strategies and influential factors of treatment outcomes were analyzed.?Results There were 10 in-hospital death with the overall mortality of 10.5% （10/95）. All the surviving patients were discharged successfully. Main postoperative complications included low cardiac output syndrome， hypoxemia and pneumonia. All the surviving patients were followed up for 5 months to 6 years， and in New York Heart Association （NYHA） functional class ⅠorⅡduring follow-up. During follow-up， nine patients after modified Brock’s procedure received radical repair， and 6 patients after bidirectional Glenn procedure received total cavopulmonary connection.?Conclusion A considerable numberof patients with complex congenital heart diseases may miss their best timing for surgical repair， which significantlyinfluences their surgical outcomes. We need to choose best palliative surgical strategy for these patients according to their pulmonary artery development condition， heart malformation characteristics and final treatment goal.
Abstract： Objective To identify the risk factors for shortterm adverse events in infants with congenital heart diseases receiving open heart surgical correction with cardiopulmonary bypass (CPB), in order to improve the outcome by adopting appropriate treatment measures. Methods We retrospectively analyzed the clinical data of 98 consecutive children with congenital heart diseases who underwent surgical correction with CPB in Beijing Fu Wai Hospital from November 2009 to December 2009. The patients were divided into two groups according to the postoperative complications. Among the patients without complications（n=40）: there were 24 males and 16 females with an age of 7.60±0.40 months and a weight of 7.80±0.30 kg. In the patients with complications (n=58): there were 42 males and 16 females with an age of 6.20±0.40 months and a weight of 6.70±0.20 kg. In both groups, perioperative data were recorded, including preoperative fast blood glucose, creatinine, time of aortic crossclamp, modified or zerobalanced ultrafiltration, postoperative glucose level, concentration of lactate, notrope score and complications. Risk stratification was performed by Risk Adjusted Classification for Congenital Heart Surgery (RACHS-1). Univariate analysis and logistic regression analysis were used to identify the risk factors for shortterm adverse events. Results One patient(1.02%) died of circulatory failure during the perioperative period. Thirtyseven patients [CM(159mm]were supported by at least 2 vasoactive drugs for more than 48hours,29 by mechanical ventilation for more than 24 hours, 5 needed reintubation, 1 experienced tracheotomy, 31 suffered from noscomial infection, 4 had wound infection, 3 developed renal failure, and 1 developed hepatic dysfunction. By logistic regression analysis, age (OR=0.750, P=0.012), percutaneous oxygen saturation (OR=0.840,P=0.005), aortic crossclamp time (OR=1.040, P=0.008), postoperative glucose level (patients with a mean glucose level lower or equal to 8.33 mmol/L had a probability of developing adverse outcomes five times higher; OR=5.051, P=0.011) were found to be the risk factors for shortterm adverse outcomes. Conclusion Age, percutaneous oxygen saturation and aortic crossclamp time are associated with the shortterm adverse outcome of infants undergoing congenital heart disease correction with CPB. The present results do not support perioperative hyperglycemia as a risk factor for adverse outcome.
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.