ObjectiveTo observe the outcome of scleral buckle and vitrectomy for familial exudative vitreoretinopathy (FEVR) associated rhegmatogenous retinal detachment (RRD) with different stages. MethodsTwenty eyes in 19 patients were included in this study. All the eyes were staged according to the staging system of FEVR. There are 7 eyes at stage 3A, 4 eyes at stage 4A, 6 eyes at stage 4B, and 3 eyes at stage 5. According to classification of retinal detachment (RD) with proliferative vitreoretinopathy (PVR), PVR B was in 5 eyes, PVR C1 in 2 eyes, PVR C2 in 3 eyes, PVR C3 in 7 eyes, PVR D1 in 3eyes. Retinal holes responsible for the RD could be found in every case. Scleral buckle or vitrectomy were chosen according to FEVR staging, PVR classification, location of retinal breaks, extent of RD.Ten eyes (stage 3A in 7 eyes, stage 4A in 3 eyes;PVR B in 5 eyes, PVR C1 in 2 eyes, PVR C2 in 3 eyes) were undergone scleral buckle, the mean preoperative minimum resolution angle in logarithmic (logMAR) best corrected visual acuity (BCVA) is 0.60±0.32.Ten eyes (stage 4A in 1 eyes,stage 4B in 6 eyes,stage 5 in 3 eyes;PVR C2 in 1 eyes,PVR C3 in 6 eyes,PVR D1 in 3 eyes) were undergone vitrectomy, the mean preoperative logMAR BCVA is 1.81±0.53. The mean follow up was(20.20±7.25) months, range 3 to 30 months. Surgical outcome were estimated by the average number of operation, reattachment of retina and BCVA. ResultsFinal retinal attachment was obtained in 100% of all 20 eyes. The mean postoperative logMAR BCVA of scleral buckle group (0.34±0.32) is improved than preoperative BCVA, the difference wan statistically significant (t=2.932, P=0.017). The mean postoperative logMAR BCVA of vitrectomy group (1.42±0.64) is not changed compare with preoperative BCVA (t=1.812,P=0.103).The mean number of operation of scleral buckle group (1.10±0.32) is less than vitrectomy group's (2.20±0.42),the difference wan statistically significant (t=6.588, P=0.000). ConclusionsAmong the patients whose FEVR staging is less than 4A and PVR classification is less than C3,epiretinal membranes or subretinal membranes appears mild, and scleral buckle can achieve high success rate with less number of operations,and the BCVA is improved in most of the cases. For the patients whose FEVR staging is more than 4B and PVR classification is more than C3, proliferative vitreoretinopathy seems to be serious, retina can be effectively reattached via vitrectomy, however, the number of operations required is multiple, and the BCVA is probably unimproved after operation.
ObjectiveTo share the experience of treating special cardiac malformations by applying minimally invasive techniques.MethodsEight children with special cardiac malformations admitted to our hospital from July 2014 to September 2020 were recruited, including 3 males and 5 females, aged 0.8-1.2 (1.1±0.4) years, and weighted 7.8-11.5 (9.6±2.9) kg. There were 2 patients of huge muscular ventricular septal defect (VSD), 3 perimembranous cribriform VSD, 1 right coronary-right atrial fistula, 1 right coronary-right ventricular fistula, and 1 young, low-weight child with large aortopulmonary. All were treated with minimally invasive techniques using transesophageal echocardiography (TEE) as a guiding tool. All children received intraoperative TEE immediately to evaluate the curative effect of the surgery, and all went to outpatient clinic for reexamination of echocardiography, electrocardiogram and chest X-ray after discharge.ResultsEight children underwent minimally invasive surgery successfully without any incision infection, intracardiac infection, arrhythmia or pericardial effusion. None of the 8 children were lost to follow-up, and the results of all reexaminations were satisfactory.ConclusionThe application of minimally invasive techniques is a bold and innovative attempt for the treatment of a few special types of cardiac malformations. It has significant advantages in reducing trauma and medical costs in some suitable patients, and has certain clinical reference values.
ObjectiveTo observe the characteristics of images of optic coherence tomography (OCT) for the macular region of congenital retinoschisis and investigate its significance.MethodsThe data of OCT of 11 patients (20 eyes) with congenital macular retinoschisis diagnosed by direct or preplacedmirror ophthalmoscopy, fundus fluorescein angiography (FFA), and electroretinography (ERG) were retrospectively analyzed.ResultsThe images of OCT showed split in the mid portion of sensory retina at the macula in all eyes. The retina of fovea centralis was divided by the split into two slightly thickening layers. In addition, in a few number of cases, the parafoveal sensory retina which became much thickening with 2 splits, was divided into 3 layers.ConclusionThe characteristic of images of OCT in macular congenital retinoschisis is the split cavity at the middle layer of the retina, and OCT has a high sensitivity in the diagnosis of congenital retinoschisis. (Chin J Ocul Fundus Dis, 2005,21:93-96)
Objective To analyze the clinical efficacy and mid-term outcomes of reimplantation of anomalous origin of left coronary artery from the pulmonary artery (ALCAPA), and to evaluate whether concomitant management of mitral regurgitation (MR) during ALCAPA repair is needed. Methods Between March 2005 and March 2015, 52 consecutive patients (20 males and 32 females with a median age of 10 months ranging 2-193 months) underwent reimplantation of ALCAPA at Department of Cardiac Surgery, Guangdong Cardiovascular Institute. There were 14 males and 21 females with a mean age of 35.4±42.8 months not receiving mitral valvuloplasty (a Non-MVP group), and 6 males and 11 females with a mean age of 13.5±11.0 months receiving mitral valvuloplasty (a MVP group). In order to facilitate the analysis, degree of MR was graded by number: 0.0=none, 1.0=trivial, 2.0=mild, 2.5=mild-moderate, 3.0=moderate, 3.5=moderate-severe and 4.0=severe. Results The left ventricular fractional shortening (LVFS) and left ventricular end diastolic diameter (LVEDD) demonstrated significant improvement between preoperation and discharge (28.6%±9.6% vs. 32.1%±10.1%, P=0.023; 38.4±5.6 mm vs. 30.5±5.7 mm, P<0.001), and there was also significant improvement in the mean MR grade between preoperation and discharge (2.9±1.2,vs. 2.4±1.2, P=0.001). There were 4 in-hospital deaths (7.7%). The median follow-up was 21.0 months (ranging 1.5-111.0 months). Three patients (5.8%) were lost to follow-up, 1 patient required reoperation for mitral valve replacement and there was no death during follow-up. Significant improvement was seen in LVFS between discharge and final follow-up (32.1%±10.1% vs. 38.0%±6.0%, P=0.001); however, there was no significant difference in the degree of MR between discharge and final follow-up (2.4±1.2 vs. 2.3±1.2, P=0.541). There was no significant difference in cardiopulmonary bypass time, aortic cross-clamping time, mechanical ventilation time or hospital stay between the two groups. Conclusion Creation of a dual coronary system with reimplantation of the left coronary artery results in complete recovery of left ventricular function. However, concomitant management of MR during ALCAPA repair remains controversial. Concomitant mitral valve repair for ALCAPA patients with moderate-severe and severe MR is helpful to early function recovery of mitral valve.
Abstract: The cyanotic congenital heart defect remains a focal point to study in congenital heart diseases. A successfully developed model of cyanotic congenital heart defect can contribute to a profound advancement of clinical diagnosis and treatment. Various kinds of animal models simulating cyanotic heart diseases have been created and improved step by step , such as experimental pulmonary arteriovenous fistulas, inferior vena cava-left atrium shunt, pulmonary artery-left atrium shunt and breeding animals in mionect ic environment. As an important means, they are used to investigate the animal’s pathophysilolgocal characteristics in cyanotic and hypoxic state. However, it need a further exploration since these models are not fully perfect yet.
Morning glory syndrome (MGS) is a congenital optic disc anomaly. The characteristic ophthalmoscopic findings consist of a generally enlarged, funnel-shaped and excavated optic disc, surrounded by an elevated annulus of chorioretinal pigment disturbance, with a central glial tuft, multiple narrow branches of retina vessels radiating from the disc. There are peripheral non-perfusion retinal areas in most cases. The pathogenesis of MGS remains unclear. MGS might be associated with many ocular and systemic abnormalities, involving facial, central nervous, cerebrovascular and endocrine systems. Persistent hyperplastic primary vitreous and retinal detachments (RD) are the most common ocular complications of MGS. The mechanism RD in MGS is unclear. Vitrectomy with long-acting gas or silicone tamponade and photocoagulation around the breaks or the enlarged disc might be efficient for rhegmatogenous RD of MGS. Early diagnosis is crucial for recognition and treatment of the ocular and systemic complications, and maintenance of the visual function.
Objective To explore the treatment method of congenital heart disease (CHD) with pulmonary artery hypertension (PAH) in infants with Down syndrome (DS). Methods The clinical data of 60 CHD patients with PAH from March 2015 to August 2016 in our hospital were retrospectively analyzed. There were 30 infants with DS classified as a DS group (trial group, 17 males and 13 females with a mean age of 1.15±0.25 years) and the other 30 patients without DS were classified as a control group (20 males and 10 females with a mean age of 1.24±0.30 years). All the patients underwent surgical treatment and fasudil combined with sildenafil were used to prevent pulmonary hypertension crisis postoperatively. Results There was no significant difference in cardiopulmonary bypass time, aortic cross-clamping time, modified ultrafiltration time and the incidence of postoperative respiratory complications between the two groups. The pulmonary systolic blood pressure significantly decreased at 24 h after operation in the two groups (both P<0.05). The arterial oxygen pressure and oxygenation index of the trial group were lower than those of the control group at 6 h after operation (both P<0.05). The mechanical ventilation time and intensive care time of the trial group were significantly longer than those of the control group (P=0.007 and P=0.000, respectively). There were no reoperations or early death. Conclusion The effects of surgical repair of CHD with PAH in infants with DS are satisfactory by grasping the indication, protecting lung function and controlling PAH in the early postoperative period, although there is a high incidence of pulmonary complications.
Objective To investigate the effectiveness of Ilizarov technique in treatment of rigid congenital clubfoot in older children. Methods A clinical data of 55 patients (69 feet) with rigid congenital clubfoot (Diméglio grade Ⅳ) between September 2005 and September 2015 were retrospectively analyzed. There were 33 boys and 22 girls with an average age of 11 years (range, 6-14 years). There were 41 cases of unilateral clubfoot and 14 cases of bilateral clubfeet. The degree of foot deformity was rated as fair in 24 feet and as poor in 45 feet according to International Clubfoot Study Group (ICFSG) score before operation. Sixty-nine feet were treated by Ilizarov external fixator combined with limited soft tissue release, and 21 feet were combined with individualized V-shaped osteotomies. Results All patients were followed up 2-10 years ( mean, 4.2 years). The incisions healed well after operation. Five cases of needle tract infection occurred and were cured by local dressing change. The effectiveness was excellent in 39 feet, good in 21 feet, and fair in 9 feet according to ICFSG score, with the excellent and good rate of 87.0%. During the follow-up, X-ray films showed that no blood supply disorder or necrosis of talus, scaphoid, cuneiform, metatarsal, calcaneus, and cuboid occurred. No osteomyelitis occurred. Conclusion Ilizarov technique combined with limited soft tissue release or V-shaped osteotomy for the older children with rigid congenital clubfeet is safe and can obtain significant orthopedic effectiveness.
Artificial intelligence belongs to the field of computer science. In the past few decades, artificial intelligence has shown broad application prospects in the medical field. With the development of computer technology in recent years, doctors and computer scientists have just begun to discover its potential for clinical application, especially in the field of congenital heart disease. Artificial intelligence now has been successfully applied to the prediction, intelligent diagnosis, medical image segmentation and recognition, clinical decision support of congenital heart disease. This article reviews the application of artificial intelligence in congenital cardiology.
ObjectiveTo evaluate the long-term clinical effect and risk factors of tricuspid valve replacement (TVR) as a relief treatment for adult patients with congenitally corrected transposition of the great artery (CCTGA).Method We retrospectively analyzed the clinical data of 47 adult patients with CCTGA who underwent tricuspid valve replacement in Fuwai Hospital between 2000 and 2017 year. There were 27 males and 20 females with operation age of 14–62 (38.8±13.5) years. Preoperative echocardiography showed moderate or more tricuspid regurgitation in all patients. The basic data of patients before and during operation were recorded. Survival was followed up by telephone and ultrasound report.ResultsThe average follow-up time was 6.5±3.7 years. The 1-year, 5-year and 10-year survival rate or the incidence of heart transplant-free was 94.6%, 90.5% and 61.7%, respectively. During the follow-up period, the long-term right ventricular ejection fraction of most patients (>90%) was still greater than or equal to 40%. Increased preoperative right ventricular end diastolic diameter (RVEDD) was a risk factor for death or heart transplantation (risk ratio 1∶11, P=0.04). The survival rate of patients with RVEDD (>60 mm) before operation was significantly reduced (P=0.032).ConclusionTVP is a feasible treatment for adult patients with CCTGA. The increase of preoperative RVEDD is a risk factor for long-term mortality.