In 1984, according to the criteria of the classifieation for congenital hand deformity which wasput out by the International Hand Surgery Committee, we had made an investigation for congenitalhand deformity among 318066 newborns in Shanghai. It was found that the inctdence of a congenitalhand malformation was 0. 0808 percent among the total newborns. The congenital malformation ofthe thumb was 37. 74 percent of all deformities of the hand. According to the statistical analysis, we ...
Objective To investigate the clinicopathologic features of congenital cystic adenomatoid malformation (CCAM) of lung in adults. Methods The clinical and pathological characteristics of two cases of CCAM of lung in adults from November 19, 2012 to February 12, 2014 were analyzed, and relevant literatures were reviewed. Results Both of the two patients were males who were 59 years and 60 years old respectively. Both of them presented with respiratory symptoms such as productive cough, hemoptysis and fever. The lesions in the two cases were about 4.0 cm×3.0 cm×1.5 cm and 5.0 cm×5.0 cm×3.0 cm in size respectively. Both had a cystic appearance and involved unilateral lobes of the lung. Histologically, normal pulmonary alveoli were replaced by different size of cysts composed of adenomatoid hyperplastic bronchioles. The inner cystic wall was lined by pseudostratified ciliated columnar epithelium, and the cystic wall contained smooth muscle and elastic tissue, but no cartilage. In one of our cases, mucous cells could be seen in part of the inner cystic wall, with focal atypical hyperplasia. Conclusions CCAM of the lung is a rare congenital developmental anomaly, which typically manifests in neonates and infants, but extremely rare in adults. The diagnosis of CCAM in adults depends on clinical features, imaging changes and histopathological characteristics.
Objective To compare the clinical results of different thoracic incision approaches for congenital heart disease in children. Methods Retrospective analyses of the clinical results of different thoracic incision approaches for 1 669 children with congenital heart disease was performed. All patients were divided into median sternotomy group(Med group), right thoracotomy group (Rat group),and lower partial median sternotomy group (Pt group) according to different thoracic incision. Two hundred and forty five children underwent atrial septal defect(ASD) repair, 1 005 children underwent ventricular septal defect(VSD) repair and 419 children underwent tetralogy of Fallot(TOF) correct repair from Jan. 1999 to Dec. 2001. Results In ASD repair the incidence of pulmonary complications after operation in the Rat group was significantly higher than that in Med group and Pt group ( P lt;0 05). The percentage of pulmonary hypertension before operation in Med group was significantly greater than th...更多at in the Rat group and Pt group ( P lt;0 05), but the incidence of pulmonary complications in Rat group was significantly higher than that in the Med group and Pt group in children with VSD( P lt;0 05). The concentration of hemoglobin , the oxygen saturation and the percentage of transannular patch in Med group were significantly higher, lower and greater respectively than those in the Pt group and in Rat group in children with TOF( P lt;0 05). Conclusion The approaches to be selected should guarantee to correct the cardiac anomaly satisfactorily, reduce the postoperative complications maximally and ensure success of their operations.
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.
ObjectiveTo evaluate clinical results of left ventricular retraining followed by double switch operation (DSO) for patients with congenitally corrected transposition of the great arteries (CCTGA) and a deconditioned morphologically left ventricle (mLV). MethodsClinical data of 14 patients with CCTGA and a deconditioned mLV who underwent surgical therapy in Fu Wai Hospital from May 2005 to May 2011 were retrospectively analyzed. There were 8 male and 6 female patients with their age of 2.5-72.0 (34.4±24.0) months and body weight of 5.1-23.0 (12.7±4.9) kg. Preoperative diagnosis was confirmed by echocardiography, angiography or cardiac catheterization. Major concomitant anomalies included tricuspid regurgitation (TR) in 13 patients, restrictive ventricular septal defect in 10 patients, atrial septal defect or patent foramen ovale in 7 patients, mild pulmonary valve stenosis in 4 patients, patent ductus arteriosus in 4 patients, and third-degree atrioventricular block in 1 patient. All the patients underwent first-stage morphologic left ventricular retraining under general anesthesia followed by second-stage atrial switch and arterial switch operations (DSO) under cardiopulmonary bypass with the interval of 0.67-34.0(10.23±9.47)months. ResultsAfter the first-stage morphologic left ventricular retraining, there was no postoperative complication or death. During follow-up, mLV end-diastolic diameter (mLVEDd) and posterior wall thickness of mLV were significantly larger than preoperative parameters (P < 0.05). The interventricular septum moved partially towards morphologically right ventricle (mRV). TR degree was significantly decreased, the pressure gradient across the pulmonary artery band was significantly increased (P < 0.05), and left ventricular ejection fraction (LVEF) was not statistically different from preoperative LVEF. And mLV/mRV pressure ratio was significantly increased (P < 0.05). After the second-stage DSO, 2 patients died with the in-hospital mortality of 14.3% (2/14). The causes of death included serious arrhythmia, circulatory collapse and sudden death. Early postoperative complications included pulmonary infection in 6 patients, atrial arrhythmias in 2 patients, pleural effusion in 2 patients, pneumothorax in 1 patient, diaphragmatic paralysis cured by diaphragm placation in 1 patient, respiratory tract hemorrhage in 1 patient, mild aortic insufficiency in 1 patient, peritoneal dialysis for 1 patient, extracorporeal membrane oxygenation for 1 patient, and tracheal intubation for a second time for 1 patient. All the 12 patients who were discharged alive were followed up for 2 to 8 years. One patient died during follow-up with the late mortality of 8.33% (1/12), and the cause of death was serious arrhythmia and circulatory collapse. Eight patients were in New York Heart Association (NYHA) classⅠ, and 3 patients were in NYHA class Ⅱ. Major late complications included left ventricular dysfunction in 3 patients, moderate aortic valve regurgitation in 3 patients, and moderate mitral valve regurgitation in 1 patient. ConclusionShort-term clinical results of left ventricular retraining followed by DSO for patients with CCTGA and a deconditioned mLV are satisfactory, and its middleand long-term results need further follow-up. But postoperative left ventricular dysfunction and new-onset aortic valve regurgitation deserve more attention.
While the Fontan operation has improved the survival of a generation of children born with a functional single ventricle, it does not recreate a normal circulation. However, significant challenges remain. Early stage mortality risk seems stubbornly high. The risk of late cowplications seriously affect the longterm survival of children. As new techniques and therapies exist, more single ventricle patients survive till adulthood. Therefore, the limits of Fontan procedures is more and more evident. In recent years, the study of mechanical cavopulmonary assisting device, which addresses the limitations of Fontan circulation, has been developed and provided a more stable and effective biventricular of blood flow in the total cavopulmonary connection in existence. This would benefit not only the treatment of late Fontan complications, but also facilitating early surgical repair, which is promising.
ObjectiveTo investigate the efficacy and safety of hybrid perventricular device closure (PVDC) for the treatment of muscular ventricular septal defect (MVSD) in pediatric patients. MethodsWe retrospectively analyzed clinical data of 35 pediatric patients with MVSD who received hybrid PVDC in West China Hospital between 2009 and 2012. There were 16 males and 19 females with their median age of 3.9 years (range, 1 month to 7 years). There were 10 patients with single MVSD, 12 patients with multiple MVSD, 6 patients with other congenital heart defects, and 7 patients with post-surgical residual MVSD. ResultsAmong the 35 patients, immediate complete closure was achieved in 28 (80.0%) patients. Residual shunt was noted in 7 (20.0%) patients. Median device size was 5 mm. Cardiopulmonary bypass was avoided in 19 patients. Two (5.7%) patients died postoperatively due to postoperative heart failure resulted from complex congenital heart defect. All survival patients were followed up for a mean duration of 6 months, and there was no late death. Thirty-one (93.9%) patients had good positioning of the closure devices but no residual shunt. Small residual shunt at the apex was noted in 2 (6.1%) patients. ConclusionHybrid PVDC is an effective treatment for MVSD with relatively high successful rate but low mortality and morbidity, and its short-term results are satisfactory.
From 1978 to Dec. 1991, 50 cases of dilatation of the extrahepatic biliary duct in children were treated. They were classified as: cystic dilatation in 34 cases, arid fusiform dilatation in 16 cases. Types of reconstruction of the extrahepatic biliary duct included: excision of cystic dilatation and Rorx-en-Y hepatoductojejunostomy in 25 cases, and interposition of jejunum and hepatoductoduodenostomy in 9 eases. for those cases having fusiform dilatation, interposition of jejunum and hepatoductoduodenostomy，cases and Rorx-en-Y-hepatoductojejunostmy 5 cases.The follow-up period averajed 6.5 years. Forty nine patients were recoverwd from the teatment and 1 patient died.
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 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.