Objective To present the surgical treatment experiences of congenital choledochal cyst (CCC). MethodsOne hundred and fortyfive patients in 152 CCC were analyzed retrospectively and followed in west China hospital of Sichuan university from 1964 to 1999. ResultsOne hundred and fortyfive cases underwent operation and 6 of them died after operation. The incidence of hepatocirrhosis within first year after birth is higher than those over one year old （P＜0.05）. Thirtynine cases underwent cystoduodenostomy or cystojejunostomy. One hundred and six children underwent cyst resection and biliary tract reconstruction (with single RouxY hepaticojejunostomy 48 cases, intussusceptive valve and rectangular valve to the line of RouxY hepaticojejunostomy 37 and 21 cases respectively). Seventyseven patients were followedup (means 4.68 years). Two of 3 cases with ascending cholangitis after single RouxY hepaticojejunostomy underwent reoperations with an intussusceptive valve added to the line of RouxY hepaticojejunostomy and the symptoms disappeared. All of them have a good outcome. Conclusion The younger the patients, the less severe the liver damaged, and its prognosis are better. The procedure that cyst resection totally and an intussusceptive valve added to the line of RouxY hepaticojejunostomy should be carried out early as soon as possible.
【Abstract】Objective To evaluate the distribution of nerve growth factor receptor( P75 NGFR) in congenital choledochal cyst(CCC) and its clinical implication. Methods Specimens from 18 children with CCC and normal choledochal specimens from 9 controls were immuno-stained with P75 NGFR antibody. Results Extensive P75 NGFR staining was found in the nerve fibres of normal comnon bile duct，bly staining of ganglion cells were observed on the normal specimens. There was very little immunoreactive fibre in the CCC. Conclusion The abnormal distribution of P75 NGFR in the aganglionic choledochal suggests that abnormal P75 NGFR is related to the occurrance of the CCC.
【Abstract】Objective To investigate the operative methods and the factors affecting the prognosis of congenital intestinal atresia. Methods The clinic data of 40 cases of congenital intestinal atresia was reviewed, including duodenal atresia (6)， jejunal atresia (12)， ileum atresia (20) and colonic atresia (2). The types of atrasia were septal type (10)，blindend type (26), and multisegmental type (4). All had been diagnosed before operation, 4 patients refused the treatment and 26 cases accepted the operation. Results Twenty-six cases of the 36 which accepted operation were cured and survived，the rate of postoperative survival were 72%，postoperative follow-up of 20 patients for 1-21 years had well-pleasing curative effect.Ten cases died postoperatively. Conclusion Operation is the only treatment of this disease，the mode of operation should be selected depending on the site and the type of atresia. The curative effect and the prognosis of this disease are affected by multiple factors.
In order to observe the absorption of the fat solube vitamine afer operative treatment of the congenital biliary dilation(CBD),the plasma fat soluble vitamin A,D and E were determined in 57 cases of CBD at the postoperative stage and 51 cases of normal children as control.The normal values of vitamin A,D and E was 576.25±170.93ng/ml,13.21±2.20ng/ml and 7.34±1.96ng/ml respectively in control group versus 501.59±120.64ng/ml, 11.66±1.81ng/ml and 6.16±1.18ng/ml respectively in the postoperative group of CBD.The differences were significant for a long period of about 10 years,and gradually disappear after that to approach or near the normal level.Therefor,the radical operation of CBD may affect the absorption of the fat soluble vitamins.
Objective To explore the efficacy of humidified high flow nasal cannula ( HHFNC) for respiratory failure after ventilator weaning in post-operative newborns and infants with congenital heart disease. Methods FromJanuary 2010 to August 2010, 33 newborns and infants [ ( 7. 8 ±8. 4) months, range 3 days to 36 months; weight ( 6. 6 ±3. 6) kg, range 2. 2 to 19. 6 kg] were treated with HHFNC ( 22 cases) and routine oxygen therapy ( 11 cases) for respiratory failure following ventilator weaning after operation of congenital heart disease. Symptoms, blood oxygen saturation ( SpO2 ) , partial pressure of oxygen( PaO2 ) , partial pressure of carbondioxide ( PaCO2 ) , incidence rate of re-intubation, duration of ICU, and hospital stay were assessed and compared between the HHFNC group and the routine oxygen therapy group.Results There were no statistical significance in the duration of ICU, hospital stay, duration of mechanical ventilation, or infection rate between the HHFNC group and the routine oxygen therapy group ( P gt; 0. 05) . But the incidence rate of re-intubation was lower in the HHFNC group than that in the routine oxygen therapy group. Meanwhile SpO2 and PaO2 increased and PaCO2 decreased significantly in the HHFNC group ( P lt;0. 05) . Conclusion HHFNC shows a clinical improvement rapidly and efficiently in preventing respiratory failure after ventilator weaning in post-operative newborns and infants with congenital heart disease.
Objective To investigate clinical outcomes of one and a half ventricle repair for acute right ventricular dysfunction after biventricular repair. Methods Clinical data of 5 pediatric patients with congenital heart diseases who underwent emergency bidirectional Glenn shunt without cardiopulmonary bypass for acute right ventricular dysfunction after biventricular repair，converting the operation into one and a half ventricle repair，from February 2007 to June 2012 in Qingdao Women and Children Hospital were retrospectively analyzed. There were 4 male patients and 1 female patient with their age of 7-18 months and body weight of 6-13 kg. Preoperative diagnosis included pulmonary atresia with intact ventricular septum （PA/IVS） in 1 patient，tricuspid stenosis （TS） with ventricular septal defect （VSD） and atrial septal defect （ASD） in 2 patients，and tricuspid stenosis with tetralogy of Fallot （TOF） in 2 patients. Postoperative care focused on cardiopulmonary support and control of pulmonary artery pressure. Results Mean pulmonary artery pressure （mPAP） was 12-18 mm Hg at 72 hours after emergency bidirectional Glenn shunt. Mechanical ventilation time was 3-182 hours and ICU stay was 2-13 days. Postoperatively 1 patient died of pulmonary hypertension crisis. The other 4 patients were discharged with their transcutaneous oxygen saturation in the resting state of 93%-99%，which was improved in different degrees compared with preoperative value. These 4 patients were followed up from 6 months to 4 years. Three patients were in NYHA classⅠand 1 patient was in NYHA class Ⅱ during follow-up. Echocardiography showed smooth vena cava to pulmonary artery anastomosis without thrombosis formation. Conclusion One and a half ventricle repair can be used as an adjunct surgical strategy for acute right ventricular dysfunction after biventricular repair.
Objective To summarize diagnosis and treatment outcomes of coronary arterial fistulas （CAFs） so as to improve surgical results. Methods Clinical data of 23 patients with CAFs who underwent surgical repair in Xinhua Hospital，School of Medicine，Shanghai Jiaotong University from July 2006 to January 2013 were retrospectively analyzed. There were 15 male and 8 female patients with their age of 35.4±8.7 years. CAFs originated from left anterior descendingartery in 7 patients，right coronary artery in 13 patients，dual coronary arteries in 1 patient，left main coronary artery in 1 patient，and obtuse marginal artery in 1 patient. CAFs drained into pulmonary artery in 16 patients，right ventricle in 5patients，and right atrium in 2 patients. Twelve patients received surgical repair under off-pump condition with beating heart，and the other 11 patients underwent surgery under cardiopulmonary bypass （CPB）. Surgical techniques included extracardiacgasket interrupted suture ligation，intracardiac mattress suture ligation，intracardiac gasket interrupted suture for fistula repair，and a combination of intracardiac suture ligation and extracardiac repair. Results There was no in-hospital death or seriouspostoperative complication in this group. For the 11 patients undergoing surgical repair under CPB，average CPB time was 78.6±7.4 minutes，and average aortic cross-clamping time was 39.0±5.9 minutes. Postoperatively，patients’ heart functionrecovered well，their symptoms disappeared，and there was no myocardial ischemia or residual fistula. Twelve patients werefollowed up at the outpatient department and via telephone for 3 months to 6 years after discharge. During follow-up，theirheart function all recovered well，and echocardiogram detected no abnormality. There was no late death，myocardial ischemiaor residual fistula. Conclusion All CAFs patients need early surgical repair once their diagnosis is clear. Appropriate surgical strategies should be chosen according to the characteristics of pathological anatomy and associated intracardiaclesions. Surgical outcomes are satisfactory.
Objective To investigate diagnostic and treatment strategies of patients with congenital heart diseases and right aortic arch. Methods Clinical data of 27 children who underwent surgical correction for congenital heartdiseases and right aortic arch in Children’s Heart Center of Beijing Children’s Hospital from January 1，2012 to April 1，2013 were retrospectively analyzed. There were 20 male and 7 female patients with their age of 10.96±12.08 months and body weight of 7.70±3.13 kg. All the patients had right aortic arch，including 14 children with tetralogy of Fallot，9 childrenwith ventricular septal defect，1 child with patent ductus arteriosus，1 child with pulmonary artery sling and 2 children with isolated double aortic arch. Thirteen children were found to have a vascular ring including aberrant left subclavian artery and double aortic arch. All the patients received surgical correction for their intracardiac anomalies and concomitant transposition of the left subclavian artery for aberrant left subclavian artery or division of the nondominal arch for double aortic arch. Results Three children died perioperatively including 1 child who was unable to be weaned from cardiopulmonary bypass，1 child without vascular ring who died 7 days postoperatively，and 1 child with double aortic arch who died of acuterespiratory distress syndrome. There was another child who gave up further postoperative treatment. Twenty-three children were followed up for 3-17 months after discharge. Echocardiography showed satisfactory correction of their intracardiac anomalies. All the patients alive recovered well with good pulses in arteries of both arms. Conclusion Careful strategiesare needed for surgical correction of patients with congenital heart diseases and concomitant right aortic arch. Comprehensivepreoperative evaluation including contrast-enhanced CT and magnetic resonance imaging is helpful for clinical decision making in diagnosis and treatment. Clinical outcomes of 1-stage surgical correction of vascular ring and other concomitant intracardiac anomalies are satisfactory with good short-term results.
Abstract： Objective To summarize surgical results of secondary subaortic stenosis （SSS） after congenital heart disease （CHD） operations，and analyze the pathogenesis of SSS. Methods We retrospectively analyzed clinical data of 19 patients who underwent surgical repair for SSS in Guangdong General Hospital from 1st June 2008 to 31st December 2012. There were 10 males and 9 females. CHD types included double outlet right ventricle，ventricular septal defect and partial atrioventricular septal defect，et al. The median age of the patients when they received their first CHD operations was 4 months （15 days to 5 year and 11 months）. The median age of the patients when they received SSS repair was 5 years and 3 months （1 year to 15 years and 3 months）. The median time interval from CHD operation to SSS repair was 4 years and 10 months （8 months to 13 years and 11 months）. Results All the patients successfully received their SSS repair. There was no surgical death in this study. Median cardiopulmonary bypass time was 79 （39 to 172） minutes，and median aortic cross-clamp time was 42 （22 to 124） minutes. Median postoperative hospital stay was 7 （5 to 9） days. Postoperatively， 1 patient required permanent pacemaker implantation. All the patients were followed up after discharge for a median durationof 1 year and 10 months （5 months to 4 years and 4 months） . During follow-up， none of the patients had any clinical symptom，their heart function was normal，and there was no late death. One patient received another subaortic stenosis repair for gradually aggravating left ventricular outflow tract stenosis. Conclusions SSS is very rare after CHD operations. The pathogenesis of SSS is perhaps related to abnormal blood flows in the left ventricular outflow tract after CHD operations. The pathogenesis time of SSS and types of CHD leading to SSS cannot be predicted. Subaortic stenosis repair is a simple and safe procedure for SSS，but postoperatively left ventricular outflow tract stenosis may appear and aggravate again.
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.