ObjectiveTo explore risk factors associated with mortality and restenosis after the surgery for congenital pulmonary venous stenosis (CPVS) combined with congenital heart disease.MethodsFrom May 2007 to August 2019, 58 patients received surgical relief of CPVS combined with congenital heart disease, including 24 males and 34 females, aged 17.2±26.3 months, weighing 8.8±8.2 kg. Endpoints were death and restenosis, and the risk factors were analyzed. A univariate and multivariate risk analyses were performed.ResultsPreoperative pulmonary venous stenosis severity score (PVSSS) was 4.5±2.7. Average pulmonary vein counts with CPVS was 1.9±1.0. There were 2 (3.4%) early deaths. The mean follow-up time was 2-145 (49.8±40.0) months. The 1-, 2-, 3- and 5-year overall survival rates were 86.7%, 81.3%, 78.5% and 73.6%, respectively, and the pulmonary venous restenosis-free rates were 79.6%, 68.5%, 68.5% and 68.5%, respectively. Preterm birth was an independent risk factor for mortality. The pulmonary venous peak flow rate ≥1.2 m/s at discharge was an independent risk factor for mortality and restenosis.ConclusionThe prognosis of CPVS is still poor. Postoperative residual stenosis at discharge is an independent risk factor for death and restenosis.
Objective To confirm the changes of pulmonary artery pressure, neo pulmonary artery stenosis and reoperation in children with unilateral absence of pulmonary artery (UAPA) undergoing pulmonary artery reconstruction. Methods The clinical data of the infants with UAPA undergoing pulmonary artery reconstruction in our hospital from February 19, 2019 to April 15, 2021 were analyzed. Changes in pulmonary artery pressure, neo pulmonary artery stenosis and reoperation were followed up. Results Finally 5 patients were collected, including 4 males and 1 female. The operation age ranged from 13 days to 2.7 years. Cardiac contrast-enhanced CT scans were performed in all children, and 2 patients underwent pulmonary vein wedge angiography to confirm the diagnosis and preoperative evaluation. Preoperative transthoracic echocardiography and intraoperative direct pulmonary arterial pressure measurement indicated that all 5 children had pulmonary hypertension, with a mean pulmonary arterial pressure of 31.3±16.0 mm Hg. Pulmonary arterial pressure decreased immediately after pulmonary artery reconstruction to 16.8±4.2 mm Hg. The mean follow-up time was 18.9±4.7 months. All 5 patients survived during the follow-up period, and 1 patient had neo pulmonary artery stenosis or even occlusion and was re-operated. Conclusion Pulmonary artery reconstruction can effectively alleviate the pulmonary hypertension in children with UAPA. The patency of the neo pulmonary artery should be closely followed up after surgery, and re-pulmonary angioplasty should be performed if necessary.
Objective To investigate the morphological anatomical abnormal ities of high congenital dislocation of hip in adults and provide anatomical basis for the total hip arthroplasty (THA). Methods From May 1997 to July 2008, 49 patients (57 hi ps) with high congenital dislocation of hip (Hartofilakidis type III) were treated. There were 6 males and 43 females with an average age of 29.4 years old (18-56 years old). The locations were left in 24 hi ps and right in 33 hi ps. The morphological parameters (including femoral length, isthmus, height of femoral head center, neck-shaft angle, medialhead offset, anteversion angle, canal flare index, anteroposterior diameter of the true acetabulum, posterior thickness of the true acetabulum, depth of the true acetabulum) of suffering hips (dislocation group, n=57) were measured by preoperative X-ray, CT and intraoperative cl inical observation and were compared with those of contralateral hips (control group, n=41). The intraoperative situations of hip were observed. Results The height of dislocation was (45.41 ± 2.15) mm. The length difference of both lower extremities was (40.41 ± 2.02) mm. In dislocation group, isthmus was shortened; height of femoral head center, neck-shaft angle and medial head offset were decreased; and anteversion angle was increased. CT showed that the canal flare index was larger than 4.7, femoral shape was funnel-shaped according to Noble classification. Anteroposterior diameter of the true acetabulum became smaller, posterior thickness of the true acetabulum became thicker, and depth of the true acetabulum was shallower. There were statistically significant differences in the morphological parameters of femur and acetabulum between two groups (P lt; 0.05). The intraoperative measurements showed that the anteroposterior diameter of acetabulum was (32.98 ± 1.02) mm and the depth of acetabulum was (14.21 ± 0.56) mm. There was no statistically significant difference between intraoperative measurements and preoperative measurements (P gt; 0.05). The acetabulum was full of fat and fibrous tissues. Running of the sciatic nerve in 40 cases were changed and it ran upward and laterally. Conclusion When high congenital dislocation of the hip in adults is treated with THA, anatomical variation must be fully taken into account. The acetabulum is expanded toward posterosuperior, excessive reamed should be avoided to prevent femoral fractures, and appropriate or tailor-made prosthesis was selected.
ObjectiveTo explore the anesthesia management experience in the interventional treatment of pediatric congenital heart diseases (CHD) percutaneously guided by transthoracic echocardiography (TTE) on a mobile operating platform. Methods From March to July 2023, a total of 13 patients from remote areas underwent interventional treatment for CHD on the mobile operating platform of Fuwai Yunnan Cardiovascular Hospital. Patients who received non-tracheal intubation general anesthesia were retrospectively included. ResultsEight children who had difficulty cooperating with the surgery (due to young age, emotional tension, crying) received monitored anesthesia care with local anesthesia supplemented by sedative and analgesic drugs while maintaining spontaneous breathing under the monitoring and management of an anesthesiologist (i.e., non-tracheal intubation general anesthesia). Among them, there were 5 males and 3 females, with an age of (6.95±3.29) years and a body weight of (19.50±6.04) kg. Through transthoracic echocardiography, they were diagnosed with atrial septal defect (6 patients), residual shunt after patent ductus arteriosus ligation (1 patient), and severe pulmonary valve stenosis (1 patient). The surgery proceeded smoothly, with satisfactory anesthesia and surgical effects, complete analgesia, and satisfactory postoperative recovery. There was 1 patient of body movement and 1 patient of respiratory depression during the operation, and both patients completed the surgery successfully after treatment. All children had no serious surgery- and anesthesia-related complications. The anesthesia time was 40.5 (34.5, 47.5) min, the surgery time was 39.0 (33.0, 45.5) min, and the recovery time was 43.0 (28.0, 52.5) min Conclusion Interventional surgery for CHD guided by TTE at a mobile platform is a minimally invasive approach without radiation damage. Non-tracheal intubation general anesthesia with spontaneous breathing can be safely and effectively implemented in children who cannot cooperate.
Objective To examine the influence of hormonal fluctuations on the perioperative outcomes of patients undergoing congenital heart surgery. Methods We conducted a retrospective analysis of clinical data from fertile women diagnosed with congenital heart disease at the Guangdong Provincial People's Hospital, between January 1, 2015, and July 30, 2019. Initially, patients were categorized into groups based on serum progesterone levels: a low progesterone group (n=31) and a high progesterone group (n=153). Furthermore, based on serum estrogen levels, they were divided into a low estrogen group (n=10), a medium estrogen group (n=32), and a high estrogen group (n=118) for comparative analysis. A control group (n=24) consisted of patients who received progesterone injections before their menstrual period. Results We finally included 184 patients. The patients’ average age was 27.6±5.7 years, with 142 (77.17%) presenting with complex congenital heart conditions. There were statistically significant differences in total postoperative standard thoracic drainage volume and postoperative albumin level between the high and low progesterone groups (P<0.05), while other perioperative outcome indicators showed no statistical differences (P>0.05). Among the different serum estrogen level groups, there were statistically significant differences in postoperative blood urea nitrogen levels, total postoperative standard thoracic drainage volume, and hospital stay (P<0.05), while other perioperative outcome indicators showed no statistical differences (P>0.05). ConclusionConsidering the overall clinical significance, the physiological changes in sex hormone levels appear to have a negligible effect on the perioperative outcomes of fertile women with congenital heart disease.
ObjectiveTo observe the optical coherence tomography (OCT) features of X-linked juvenile retinoschisis (XLRS) and its relationship and visual acuity. Methods Twenty-three XLRS patients (38 eyes) were enrolled in this study.All patients underwent best corrected visual acuity (BCVA) and frequency-domain optical coherence tomography (SD-OCT) examination. The international standard visual acuity chart was used for BCVA test and the results were then converted into logarithm of the minimum angle of resolution (logMAR) visual acuity. The mean logMAR BCVA of all patients was 0.41±0.28. Topcon 3D-OCT 1000 and Topcon DRI-OCT were applied to detect the retinal layers of retinoschisis. Based on the OCT features, retinoschisis was classified into 3 types, including splitting between outer nuclear layer (ONL) and outer plexiform layer (OPL), inner nuclear layer morphology (INL) splitting, splitting between ganglion cell layer (GCL) and nerve fiber layer (NFL). Based on the OCT features of fovea, there were photoreceptor inner segment (IS)/outer segment (OS) presence group and IS/OS absence group. ResultsThere were 17 eyes (44.7%) with splitting between ONL/OPL, 38 eyes (100%) with INL splitting, 13 eyes (34.2%) with splitting between GCL/NFL. INL splitting can coexist with splitting in other retinal layers. There were 25 eyes (65.8%) with splitting in 2 or more retinal layers. The mean logMAR BCVA were 0.32±0.17 and 0.44±0.22 respectively in the IS/OS absence group and presence group, the difference was statistically significant (t=6.531, P=0.008).The mean fovea thickness (FT) were(517.10±96.92)and(523.08±72.84) μm respectively in the IS/OS absence group and presence group, the difference was not statistically significant (t=2.282, P=0.061). There was no correlation between logMAR BCVA and FT (r=0.200, P=0.929). ConclusionsIn patients with XLRS, the foveomacular schisis involved in multiple retinal layers and most frequently in the INL. The defect of IS/OS is the important reason of the low visual acuity.
ObjectiveTo evaluate the mid-term results of surgical treatment for functional single ventricle associated with total anomalous pulmonary venous connection.MethodsWe reviewed the clinical data and follow-up results of 12 patients in our hospital who underwent both single ventricle series palliation and total anomalous pulmonary venous connection correction from 2008 to 2018. There were 6 males and 6 females at age of 2.3 (1-21) years. Univariable and multivariable Cox proportional hazard regression methods were performed. The Kaplan-Meier method was used to estimate the survival rate.ResultsAll patients were successfully removed from extracorporeal circulation. The cardiopulmonary bypass time was 113.8±42.5 min. The myocardial block time was 57.7±31.7 min. There were 3 in-hospital deaths and 4 late deaths. The causes of death in hospital were pulmonary hypertension, pulmonary hemorrhage, and hypoxemia. During follow-up, the cause of death was heart failure. The survival rate at 1 year and 3 years was 58.3% (95%CI 27% to 80%) and 40% (95%CI 13% to 65%), respectively. Median follow-up was 48.3 months (range: 1 to 118 months).ConclusionFunctional single ventricle combined with complete pulmonary venous drainage is a serious clinical disease, with poor therapeutic effect and high overall mortality.
ObjectiveTo analyze the early outcomes of 203 neonates with low birth weight (<2 500 g) undergoing cardiac surgery, and to analyze the causes of death during hospitalization.MethodsFrom June 2003 to June 2017, medical records of 203 neonates with low birth weight undergoing congenital heart surgery in Guangdong General Hospital were reviewed retrospectively. There were 124 males and 79 females, including 151 premature infants. The average birth weight was 1 719±515 g, the average age at operation was 32.7±20.2 d and the average weight at operation was 1 994±486 g. The causes of death during hospitalization (including neonates given up on treatments) were analyzed.ResultsTotally 103 patients had pneumonia, 98 patients needed mechanical ventilation to support breathing and 26 patients needed emergency operation before operation. All patients undergoing congenital heart surgery were treated with general anesthesia with tracheal intubation, including 107 patients under non cardiopulmonary bypass (CPB) and 96 patients under CPB with a mean CPB time of 96.5±71.7 min and a mean aorta cross-clamp time of 51.8±45.5 min. The average postoperative mechanical ventilation time was 9.1±21.5 d and the average postoperative length of stay was 26.7±19.3 d. The major postoperative complications included pneumonia, anemia, atelectasis, septicemia, intrapleural hemorrhage, diaphragm paralysis and cardiac dysfunction. Twenty-nine patients died during hospitalization and the overall mortality rate was 14.3%. Four patients died in the operation room, 14 patients died 72 hours after operation and 2 patients were given up. The main causes of hospitalized death were low cardiac output syndrome, severe infection, disseminated intravascular coagulation disorder, acute renal failure and pulmonary hypertension crisis.ConclusionOverall, early cardiac surgery for low birth weight neonates is safe and effective. The difficulty of the cardiac surgery is the key to the prognosis. Strengthening perioperative management can improve the quality of operation and reduce the risk of mortality and morbidity during hospitalization.
Objective To explore the dominant views with positive results when performing echocardiography on common congenital heart diseases (CHD) in children using the "Seven-Step Screening Method for Pediatric Echocardiography". MethodsThe echocardiographic data of children with atrial septal defect, patent foramen ovale, ventricular septal defect, and patent ductus arteriosus were collected from September 2021 to February 2022. The disease type distribution, view distribution, and the dominant view distribution were analyzed. Dominant view refered to the view with a high ratio of positive result images per view to the total image in each disease. Results A total of 8 353 images of 1 633 children with common CHD were collected. There were 813 males and 820 females at age of 0-7 years. Including 3 613 images in 701 patients with atrial septal defect, 1 178 images in 206 patients with patent foramen ovale, 2 857 images in 576 patients with ventricular septal defect, and 705 images in 150 patients with patent ductus arteriosus. The dominant views of atrial septal defect were subxiphoid 2-chamber view (92.96%), subxiphoid 4-chamber view (85.61%), parasternal 4-chamber view (62.07%), and parasternal short-axis view (38.50%). The dominant views of patent foramen ovale were subxiphoid 2-chamber view (82.69%) and subxiphoid 4-chamber view (65.41%). The dominant views of ventricular septal defect were parasternal 5-chamber view (79.73%), parasternal short-axis view (79.41%), parasternal 4-chamber view (58.18%), and parasternal long-axis view (51.11%). The dominant view of patent ductus arteriosus were parasternal short-axis view (98.80%). Conclusions The analysis of the lesion key areas of common CHD showed that there were 4 dominant views for atrial septal defect and ventricular septal defect, 2 for patent foramen ovale, and only 1 for patent ductus arteriosus. Clarifying the dominant views of common CHD were conducive to rapid and accurate diagnosis of diseases, clinical, teaching and scientific research.
Cochlear implant (CI) is the only method for efficacious treatment of congenital severe deafness at present. However, for children with congenital severe deafness after CI, the mechanism of the structural and functional changes of their cerebral cortex is not clear. This study was based on the cross-modal reorganization of deaf patients. Event related potential (ERP) and source localization technique were used to visualize the change of cortical activity in children with congenital severe deafness during 1-year period (0, 1, 3, 6, 9 and 12 months after CI). We aimed to investigate the association between hearing restoration and cross-modal reorganization in children with congenital severe deafness after CI. The results showed that the cross-modal reorganization exists in children with congenital severe deafness. During hearing restoration, the function of the cross-modal reorganization reversed to the normal state. The method and conclusions of this study may be of significance in guiding the training and evaluation of hearing rehabilitation after CI in patients.