ObjectiveTo summarize the cardiac-vascular abnormalities and clinical results in patients with Fontan operation for heterotaxy syndrome. MethodWe retrospectively analyzed the medical records of 81 patients who underwent the Fontan operation with heterotaxy syndrome between September 2008 and September 2013. There were 49 male and 32 female patients at age of 3.79 (range 2.07-13.02) years with preoperative room air saturation of 81% (range 63%-97%) and weight of 14.8 (10.0-36.0) kg. ResultsThere were 70 patients in the right atrial isomerism group and left in 11 patients. Dextrocardia was seen in 16 patients, and single atrium in 50 patients. Single ventricle was found in 44 patients, conotruncal defects in 40 patients, pulmonary stenosis in 72 patients, pulmonray atresia in 8 patients, common atrioventricular valve in 61 patients, and bilateral superior vena cava in 54 patients. The staged Fontan procedure was applied in 57 patients and one stage in 24 patients. Operation strategies were included intra/extracardiac conduit (n=17), extracardiac conduit (n=48), lateral tunnel (n=14) and direct cavopulmonray connection (n=2).There were 15 early deaths. Postoperative complications included low cardiac output in 15 patients, hepatic insufficiency in 35 patients, renal insufficiency in 55 patients required peritoneal dialysis and arrhythmia in 28 patients. The room air saturation was 89% (range 78%-98%) before discharge and time of follow-up was from 2 months to 6 years in 64 patients. Thromboembolic events were found in 2 patients who had accomplished conduit replacement operation. ConclusionCompared with reported literatures in western countries, heteraotaxia patients are mostly with right atrial isomerism. Fontan palliation is still the main treatment option and strict indication was needed for satisfactory clinical results.
Objective To discuss the effectiveness of intramedullary nail fixation with selective cable wiring in the treatment of ipsilateral femoral neck fracture and subtrochanteric fracture. Methods Between June 2012 and December 2015, a total of 19 patients with ipsilateral femoral neck fracture and subtrochanteric fracture underwent closed reduction of femoral neck fracture and intramedullary nail fixation combined with selective cable wiring. There were 5 males and 14 females with a median age of 52 years (range, 35-77 years). The cause of injury included traffic accident injury in 17 cases and falling injury in 2 cases. According to Garden classification for femoral neck fractures, 7 cases were rated as type Ⅱ, 8 as type Ⅲ, and 4 as type Ⅳ. Femoral subtrochanteric fractures were classified by Seinsheimer classification, with 9 cases as type Ⅱ, 5 as type Ⅲ, 3 as type Ⅳ, and 2 as type V. The interval from injury to operation ranged from 2 to 7 days with an average of 3.7 days. Results The operation time was 58-125 minutes (mean, 82.4 minutes) and the intraoperative blood loss was 225-725 mL (mean, 289.5 mL). All incisions achieved healing by first intention and no early complication such as infection was observed. All patients were followed up 12-18 months (mean, 13.9 months). At 1 month after operation, the tip apex distance was 9-23 mm (mean, 15.2 mm). All patients achieved bone union with the healing time of 18-42 weeks (mean, 27.4 weeks). One case of hip varus and femoral neck re-displacement (femoral neck shaft angle was 122°) occurred at 3 months after operation, which achieved bone union at 42 weeks after operation. Five patients complained of postoperative pain with the visual analogue scale (VAS) score of 1-3 (mean, 1.8), which did not influence normal life. A total of 16 patients recovered preoperative hip function. During follow-up, no fracture nonunion, femoral head necrosis, implant failure, screw cut-out, and loosening of cable wiring was observed. The Harris hip score (HSS) was 72-92 (mean, 82.8) at last follow-up and 15 patients (78.9%) achieved good hip function. Conclusion Intramedullary nail fixation combined with selective cable wiring was effective in the treatment of ipsilateral femoral neck fracture and subtrochanteric fracture.
ObjectiveTo evaluate the effects of anatomic correction for congenitally corrected transposition of the great arteries (ccTGA) and 10-year follow-up.MethodsFrom January 2008 to December 2018, 48 patients with ccTGA who underwent anatomic correction were reviewed. There were 29 males and 19 females with age of 39.2 (3-91) months. The cohort was divided into two groups: a biventricular anatomic correction group (39 patients) and a 1.5 ventricular anatomic correction group (9 patients). They were followed for in-hospital mortality, late mortality, long-term survival, freedom from reoperation, and heart function.ResultsThere were 3 early deaths and 2 early re-intervention in the biventricular anatomic correction group, but no death and only one re-intervention in the 1.5 ventricular anatomic correction group. Compared with the biventricular anatomic correction group, the operation time, tracheal intubation and ICU time were significantly reduced or shortened in the 1.5 ventricular anatomic correction group (P<0.05). The patients were followed up for 0.5-10.4 years. Four patients were lost. Two patients died in the biventricular anatomic correction group, and two patients received re-intervention. The 1-year, 5-year and 10-year survival rate was 88.2%, 84.0%, and 84.0%, respectively. There was no death or intervention in the 1.5 ventricular anatomic correction group. The quality of life of the other patients in the medium-term follow-up was satisfactory. Only 2 patients were classified as grade Ⅲ in cardiac function, and the other patients were classified as grade Ⅰ-Ⅱ.ConclusionAccording to the different anatomic characteristics of ccTGA, the individualized strategy of anatomic correction can achieve satisfactory surgical results, and the medium-term quality of life was good. Especially, 1.5 ventricular anatomic correction may obtain better therapeutic effects because of its lower operative mortality and less postoperative complications.
ObjectiveTo summarize and explore the individualized surgical treatment strategy and prognosis of anomalous aortic origin of coronary artery (AAOCA). MethodsThe clinical data of children with AAOCA admitted to Shanghai Children's Medical Center from March 2018 to August 2021 were retrospectively analyzed. ResultsA total of 17 children were enrolled, including 13 males and 4 females, with a median age of 88 (44, 138) months and a median weight of 25 (18, 29) kg. All patients received operations. The methods of coronary artery management included coronary artery decapitation in 9 patients, coronary artery transplantation in 5 patients and coronary artery perforation in 3 patients. One patient with severe cardiac insufficiency (left ventricular ejection fraction 15%) received mechanical circulatory assistance after the operation for 12 days. No death occurred in the early postoperative period, the average ICU stay time was 4.3±3.0 d, and the total hospital stay was 14.4±6.1 d. All the children received regular anticoagulation therapy for 3 months after discharge. The median follow-up time was 15 (13, 24) months. All patients received regular anticoagulation therapy for 3 months after discharge. No clinical symptoms such as chest pain and syncope occurred again. The cardiac function grade was significantly improved compared with that before operation. Imaging examination showed that the coronary artery blood flow on the operation side was unobstructed, and no restenosis occurred. ConclusionAAOCA is easy to induce myocardial ischemia and even sudden cardiac death. Once diagnosed, operation should be carried out as soon as possible. According to the anatomic characteristics of coronary artery, the early effect of individualized surgery is satisfactory, and the symptoms of the children are significantly improved and the cardiac function recovers well in the mid-term follow-up.
ObjectiveTo investigate the clinical, radiographic characteristics and differential diagnosis of pulmonary Langerhans cell histiocytosis (PLCH) mimicking metastasis of cancer in radiography. MethodsClinical data of 2 patients with PLCH manifesting as metastatic cancer on HRCT and PET/CT were retrospectively analyzed. Patients reported as PLCH on WanFang Database, China Knowledge Resource Integrated Database and Pubmed were reviewed to screen misdiagnosis literature and further analyzed the clinical and radiographic characteristics. ResultsTwo cases both presented with cough and sputum. 18F-FDG PET/CT showed increased 18F-FDG up-take in both nodules in the lungs. One patient presented with multiple nodules, diffuse multiple cystic changes in lungs and osteoclasia in the right 4th rib on HRCT who was diagnosed by a video-assisted thoracoscopic biopsy of rib biopsy. The other patient presented with diffuse multiple nodules on HRCT who was diagnosed by a video-assisted thoracoscopic biopsy of lung biopsy. The pathological characteristics of both biopsy specimen demonstrated infiltration by Langerhans cells (LC) and eosinophils. The LC were positive for CD1a. Literature review found seven PLCH cases who were misdignosed as depression, eosinophilic pneumonia, interstitial lung disease involvement of autoimmune disorders and malignant tumor. ConclusionWhen clinician faced with a patient suspected as metastatic cancer by HRCT and PET/CT, it is reasonable to consider PLCH as a differential diagnosis and obtain the pathological information as soon as possible so that better prognosis can be achieved through early intervention.
ObjectiveTo evaluate the effect of off-pump pulmonary valvulotomy for the patients with pulmonary atresia with intact ventricular septum (PA/IVS).MethodsThe clinical data of 61 PA/IVS patients who underwent off-pump right ventricular decompression surgery in our hospital from January 2013 to September 2019 were retrospectively analyzed, including 37 males and 24 females, with an average age of 29.7 (2.0-86.0) d and weight of 4.1 (2.5-6.9) kg. Thirty-nine patients received off-pump pulmonary valvulotomy (an open-view valvulotomy group) and 22 patients received balloon valvuloplasty through the right ventricle pulmonary valve (a hybrid therapy group). The postoperative mortality, early re-intervention, and completion of final operation of the two groups were compared.ResultsThere were 2 deaths in the study with a mortality rate of 3.3% (2/61), and the mortality rate of the two groups was not significantly different (2.6% vs.4.5%, P=0.68). The rate of early re-intervention in the two groups was 5.3% and 19.0%, respectively (P=0.09). There was no statistical difference in intubation time (56.0±25.9 h vs. 62.0±28.9 h, P=0.41), ICU retention time (4.7±2.9 d vs. 5.5±2.2 d, P=0.23) and postoperative hospital stay time (3.9±0.9 d vs. 4.3±1.1 d, P=0.38) between the two groups. The follow-up time was 45.3 (4.0-84.0) months. There were 5 patients lost to follow-up. During the follow-up period, in the open-view valvulotomy group, 17 patients did not need further operation, 13 patients completed the final operation. In the hybrid therapy group, 7 patients did not need further operation, 8 patients completed the final operation. Heart function classification of all patients was in New York Heart Association class Ⅰ-Ⅱ.ConclusionCompared with the hybrid therapy, off-pump pulmonary valvulotomy for PA/IVS also has the advantages of simple operation, short operation time and high survival rate, and it may be easier to be promoted in clinical application because of its more economic benefits and relatively lower re-intervention rate.
ObjectiveTo evaluate the surgical strategy and follow-up for pulmonary atresia with intact ventricular septum (PA/IVS) in our heart center.MethodsFrom January 2008 to December 2018, 151 patients with PA/IVS were divided into two groups: a one-stage surgery group (26 patients), including 17 males and 9 females at an average age of 14.7±13.2 months, and a staged surgery group (125 patients) including 72 males and 53 females at an average age of 6.4±6.3 months. The clinical effectiveness of the two groups were analyzed.ResultsAll patients were followed up for 1-11 years. Eighteen patients died and 19 patients were lost to follow-up. The 1-year, 5-year and 10-year survival rate was 90.2%, 87.0%, and 85.2%, respectively. Two patients died in the one-stage surgery group. Twelve patients died after initial surgery, and 4 patients died after final operation in the staged surgery group. The Z value of tricuspid valve (P=0.013) and severe right ventricular dysplasia (P=0.025) were the risk factors of postoperative death in the patients with PA/IVS. Furthermore, 58 patients completed final operation, and the total number of the final operation (including one-stage radical surgery) accounted for 55.6% (84/151). Five patients accepted the re-operation intervention in the medium-term follow-up. The rest of the patients recovered well. Only 2 patients were classified as grade Ⅲ in cardiac function, and the rest patients were classified as gradeⅠ-Ⅱ.ConclusionAccording to the degree of right ventricular hypoplasia, the age at operation and the presence or absence of coronary artery malformation, the individualized surgical strategy could significantly improve the success rate of PA/IVS, and early completion of right ventricular decompression operation is conducive to improve the chance for biventricular repair.
ObjectiveTo compare and investigate the efficacy and differences of modified B-T shunt, central shunt and right ventricle-pulmonary artery (RV-PA) connection in the treatment of pulmonary atresia with ventricular septal defect (PA/VSD).MethodsA total of 124 children with PA/VSD underwent initial palliative repair in Shanghai Children's Medical Center from September 2014 to August 2019, including 63 males and 61 females, aged 7 days to 15 years. They were divided into in a modified B-T shunt group (55 patients), a central shunt group (22 patients) and a RV-PA connection group (47 patients). The clinical data of these children were retrospectively analyzed.ResultsThere were 9 early deaths after palliation, with an early mortality rate of 7.3%. The mean follow-up time was 26.5±20.3 months, with 5 patients lost to follow-up, 5 deaths during the follow-up period, and 105 survivors. The 1-year and 5-year survival rates were both 89.7%. The monthly increased Nakata index was 5.2 (–0.2, 12.3) mm2/m2, 9.2 (0.1, 23.6) mm2/m2, 6.3 (1.8, 23.3) mm2/m2 in the modified B-T shunt group, the central shunt group, and the RV-PA connection group, respectively, with no statistical difference among the three groups. The 1-year survival rate was 85.3%, 78.4%, 95.2%, and the 5-year (4-year in the central shunt group) survival rate was 85.3%, 58.8%, 95.2% in the three groups, respectively, with a statistical difference among them (P<0.05). The complete repair rate was 36.5%, 19.0% and 67.4% in the three groups, respectively, with a statistical difference among the three groups (P<0.001).ConclusionAll these three palliative surgical approaches can effectively promote pulmonary vascular development. But compared with systemic-pulmonary shunt, RV-PA connection has a lower perioperative mortality rate and can achieve a higher complete repair rate at a later stage, which is beneficial for long-term prognosis.