Objective To summarize experiences in surgical treatments of 18 patients suffered from Ebstein's anomaly with intracardiac repair and bi-directional cavopulmonary shunt from January 1999 to June 2003. Methods Of 18 patients, 10 male (55.6%) and 8 female (44.4%), mean age was (18.47?13.85) years old (range from 9 months～54 years old),and mean weight of body (36.09?19.78) kg (range from 8.5～80.0 kg). All patients were in type II of Ebstein's anomaly according to WANG (Zenwei's) classification, while 15 (83.3%) were in type B, and 3 (16.7%) in type C according to Carpentier's classification. Danielson repair of Ebstein's malformation and other intracardiac repair were performed on all patients, followed by bi-directional cavopulmonary anastomosis, on pump with heart beating. Results There was no death, and no severe heart dysfunction and refractory low cardiac output in all patients postoperatively. No reoperation for residual or recurrent tricuspid incompetence was required in all patients. At follow-up of 13 (72.2%) patients ranging from 1 to 53 months, 12 patients were in New York Heart Association ((NYHA)) class I, 1 in class II. Four were with 1 grade, 2 with 2 grades tricuspid regurgitation. The patency of bi-directional cavopulmonary anastomoses was verified by echocardiography. Conclusion Ventricular unloading added to intracardiac repair appears to be effective to improve left and right ventricular function and tricuspid valve performance in Ebstein's anomaly with moderate or massive tricuspid dysfunction and physiological right ventricular outlet tract obstruction.

Objective To compare and analyze clinical and pathological data of autopsyconfirmed invasive fungal disease (IFD) in elderly patients in order to achieve a better understanding of the clinical and pathological characteristics of IFD.Methods A total of 18 cases of IFD were diagnosed by autopsy from 1984 to 2014 at Beijing Hospital.Clinical and pathological data of IFD,including risk factors,clinical manifestation,X-ray and pathological characteristics,were analyzed retrospectively.Results The 18 cases were all male wvith an average age of (83.7±7.2) years and each patient had at least one risk factor for IFD.Of them,14 patients (77.8％) suffered malignancies of various origins.With respect to the pathogens,Mucor (6 cases) was the most common one,followed by Aspergilla (4 cases),Mycotoruloides (4 cases) and Cryptococci (2 cases).The lung was the most frequently implicated organ wvith 13 cases (72.2％),followed by the gastrointestinal tract.Vascular erosion was an important pathological characteristic of fungal infection,whose presentations included vasculitis,hemorrhage and embolism in tissues and organs.14 patients died from fungal infection-related causes,of which.massive hemorrhage as a result of vascular erosion by fungal infection was responsible for four patients' deaths.Conclusions Malignancies are an important risk factor for invasive fungal disease in elderly patients.Vascular erosion is a significant character of fungal infection.

Objective To evaluate the efficiency of emergency surgery to consecutive 258 infants suffering with critical congenital heart diseases (CHD). Methods From January 2006 and February 2009,emergency surgery was performed within 24 h after definite diagnosis on 258 children with critical congenital heart diseases,including 150 males (58.1%) and 108 females (41.9%),with a mean age of (7.5?4.3) months (ranging from 6 d to 11 months),at a mean weight of (5.5?3.3) kg (ranging from 2.1 to 9.5 kg). Complications such as refractory pneumonia,heart failure,repeated episodes of hypoxic spells,kidney dysfunction,liver dysfunction,severe anaemia or growth retardation were found in 233 infants (90.2%). Totally 246 (95.3%) of them were operated on CPB with heart arrested,and 12 (4.7%) with patent ductus arteriosus and operated off pump. Results This group of patients had a stage-one corrective rate of 92.3%,and a total curative rate of 96.9%. In infants operated with heart arrested and with heart beating,the duration of cardiopulmonary bypass was 105.00?38.71 min and (71.89?27.78) min respectively,postoperative ventilation duration was (16.5?9.3) h,intensive care unit (ICU) staying duration was (98.5?33.6) h. After operation,low cardiac output syndrome occourred with a rate of 6.9% and severe complications of major organs with a rate of 12.9%. Of 8 (3.1%) dead infants,7 were diagnosed with complicated CHD. Five died of low cardiac output syndrome,1 of arrhythmia,1 of respiratory failure and 1 of arrhythmic heart. Conclusion Emergency surgical treatment should be performed as soon as definite diagnosis for critical infants with congenital heart disease. Heart failure,respiratory failure,liver dysfunction,severe anaemia and infection are not contraindications to surgical treatment. However,complex anomalies and younger age are considered to be the most risk factors for surgery of infants with CHD.

Objective To investigate the CT features of hepatic focal nodular hyperplasia (FNH) in children.Methods Thirteen patients with FNH,which was confirmed by postoperative pathology,were enrolled retrospectively.Plain and contrast CT were performed on all patients before operation.The CT imaging features of FNH including size,shape,density,style of contrast were analyzed retrospectively and compared with pathology.Results There were 13 patients with 16 lesions,8 lesions were found in the right lobe,5 lesions in the left lobe and 3 lesions involving both lobes.The tumor size ranged from 5.5 cm to 11.5 cm (media size 7.5 cm) in diameter.Histologically,2 cases were typical type,11 cases were atypical type.The lesions were regular morphology in 12 cases and 1 case with capsule.On plain CT,the lesions were isodensity (n=1) or slightly low-density (n=12).In 2 typical type lesions,there were slit-like,stellate-shaped low density central scars.Arterial phase demonstrated that 12 cases were significantly enhanced and 1 case showed mild enhancement.The central scar was not enhanced.In 12 cases,thickened and torturous arteries were seen.The enhancement was reduced at the portal venous phase in all the lesions,with 10 cases showing slightly higher density,2cases isodensity and 1 case low-density.Two cases showed mild enhancement of the central scar.The enhancement of the solid portion in all lesions decreased at the delay phase,with 12 cases showing isodensity and 1 case slightly low density.Two cases with central scar showed delayed enhancement with slightly higher density.Conclusion The CT features of FNH in children are diversified but distinctive which are related with postoperative pathological findings.Combining with clinical symptoms and CT features can be helpful for the early diagnosis of FNH in children.

Objective　To investigate the relationship betwee n tricuspid insufficiency (TI) and the function of the right ventricle, degree o f pulmonary vascular pathological change in patients with rheumatic heart diseas e so as to provide the indication of operation for treating TI. Methods 　41 cases of rheumatic heart disease with TI accompanying with bicuspid pathological changes were reviewed. Correlation analysis was done between the s everity of TI and the right ventricular size(RVS), pulmonary artery pressure(PAP ), pulmonary artery resistance(PAR) etc. Results　It was found t hat the size of the right ventricle, PAP, PAR were positively correlated to th e degree of TI. Tricuspid annuloplasty should be carried out in patients with RV D>40 mm, PAR >48 kPa*s/L, PAP>8 kPa. Conclusion　TI resulted ma inly from insufficient function of the right ventricle and marked patholog ical changes of the pulmonary blood vessels. Doppler echocardiography evaluation for the function of right ventricle and pathological condition of pulmonary blo od vessels might be of significant in deciding whether tricuspid annuloplasty sh ould be performed simutaneously in patients of bicuspid valve replacement.

Objective　To introduce the technique of mitral v alve replacements in beating heart, and review the clinical experience in 234 ca ses of operation. Methods　A total of 234 patients of mitral val ve replacement in beating heart with mild hypothermic extracorporeal circulation (30～32 ℃) were reviewed. Results　 The procedures underwe nt fluently and only 2(0.85%) died early postoperatively. No low cardiac output , arrhythmia and cer ebral embolism complications was found. Conclusion　Results sugg ested that mitral valve replacement in beating heart is a safe and available method and is good in extenuating myocardial and pulmonary i njury from ischemia-reperfusion and deep hypothermia.

Objective　To compare the effects of mild hypothe rmic cardiopulmonary bypass (CPB) and moderate hypothermic cardiopulmonary bypas s in pediatric cardiac surgery. Methods　A total of 118 cas es of less than 3 years of age that had undergone open heart surgery were review ed, in which 46 patients received moderate hypothermic CPB(group 1) and 72 patie nts received mild hypothermic CPB(group 2). The CPB duration, incidence of low c ardiac output and postoperative concentration of CK-MK, etc, were compared with each other betwee n the two groups. Results　Duration of bypass and postoperative mechanical respiratory assistance of group 2 was shorter than that of group 1 ( P<0.05). Transfusion requirements, incidence of low cardiac output syndrome, concentration of CK-ME and percentage of metabolic acidosis were lower in grou p 2 than in group 1 (P<0.05), while the index of oxygenation was higher in g roup 2(P<0.05). Conclusion　The mild hypothermic CPB is saf er and more effective and therefore is superior to moderate hypothermic CPB in p ediatric cardiac surgery.

Objective　To compare the clinical efficiency of intracardiac procedures on traditional cardioplegic arrested-heart and on-pu mp beating-heart for congenital heart disease (CHD) with severe pulmonary hyper tension. Methods　Among all 153 cases, 95 cases underwent operat ions on cardioplegic arrested-heart, while 58 on-pump beating-heart. In arres ted-heart group, 79 cases with ventricular septal defect (VSD), 13 with atria l septal defect (ASD) and 3 with patent ductus arteriosus (PDA) were examined whi le in beating-heart group, 43 cases with VSD, 10 with ASD, and 5 with PDA were examined. Results　There were 12 cases of operative death (12.6%) and 8 of tracheotomy (8.4%) in heart arrested group. No operative death and tracheotomy in beating-heart group. 141 patients were followed up for 3 months to 10 year s with good recovery. There were 2 cases of right heart function failure six yea rs later in arrested-heart group. Conclusion　Results sugges t that on-pump beating-heart technique is superior to traditional cardiopl egic arrested-heart for CHD with severe pulmonary hypertesion. The cause might be t hat on-pump beating-heart intracardiac operation is more effective in cardio pulmon ary protection.

Objective To explore the clinicopathological features of IgA nephrolpathy associated with malignant hypertension (IgAN-MHT) and to analyze their correlation with renal vascular lesions. Methods Twenty-nine patients of IgAN-MHT were screened from 2000 biopsy-proven eases with primary IgA nephropathy (IgAN) in our department from April 1997 to May 2007. Data of clinicopathology and follow-up of these 29 patients were collected. Semi- quantitative analysis was performed to evaluate the pathological changes. Inner lumen, outer lumen, intimal thickness, tunica media-to-internal lumen ratio of 436 arterioles, 124 interlobular arteries and 5 arcuate arteries were measured. The primary endpeint was the composite of a doubling of serum creatinine level and ESRD. Correlations of renal vascular lesions with clinical manifestation, pathological change and prognosis were examined by Spearman and Cox methods. Results 1.5% of all the IgAN patients presented malignant hypertension. The common clinical features were renal failure (100%), hyperurieacidemia (62.7%) and hypertriglyceridemia (51.7%). The average amount of urine protein excretion was 2.8 g/d. The common pathological changes were moderate mesangial proliferation, severe global sclerosis, severe interstitial inflammation and severe interstitial- tubular fibrosis. The small arteries (arcuate arteries and interlobular arteries) and arterioles (afferent arterioles) were both involved in IgAN-MHT. The characteristic lesions of intrarenal arteries included vascular occlusion, media thickening, proliferative endarteritis (onionskin lesion, musculomucoid intimal hyperplasia), hyaline arteriosclerosis, but mainly vascular occlusion (86.2%). The arteriole lesion was negatively correlated with age and total protein level; vascular occlusion was positively correlated with uric acid level. The average foUow-up period was 21.1 months. Forteen patients reached the endpoint. The arteriole lesion was the main independent risk factor for the progression of IgAN-MHT (RR=10.21, 95%CI=1.16～89.67). Conclusions The main clinical feature of IgAN-MHT is renal failure. The main histological feature of intrarenal vascular lesions is occludes arterioles. Arteriole lesion is the main independent risk factor for the progression of IgAN-MHT.

Objective To investigate the diagnostic value of EUS with miniprobe on upper gastrointestinal tract mesenchymal tumor(GIMT).Methods The EUS features of 38 patients with GIMT who underwent EUS with miniprobe were studied retrospectively,and the results were compared with the postoperative pathological findings.Results Among 38 GIMT cases detected by EUS,there were 25 cases of gastrointestinal stromal tumor,11 cases of leiomyoma,and 2 cases of leiomyosarcoma.Postoperative histopathological and immunohistochemical examinations confirmed 28 cases of stromal tumor.In which 6 cases were high-risk GIST,8 cases were leiomyoma,1 case was leiomyosarcoma,and 1 case was neurofibroma.The accuracy of diagnosis with EUS was 89%.Conclusion EUS is an accurate method in diagnosis of submucosal tumors,which can make better differentiation diagnosis between GIMT and other submucosal tumors.