Comparison of Prone With Lithotomy Position in Removal of Posterior Myoma in Transvaginal Natural Orifice Endoscopic Surgery: A Prospective Cohort Study.

OBJECTIVE: Vaginal natural orifice transluminal endoscopic surgery (vNOTES) is considered to have the advantages of completely scarless, less postoperative pain, earlier flatus, and faster postoperative recovery. However, posterior myoma are relatively difficult to operate through vNOTES in the conventional lithotomy position. Thus, we innovated the application of prone position in the removal of posterior myoma in vNOTES. The aim of this study is the comparison of myomectomy outcomes of patient for single posterior myoma in prone and lithotomy position. DESIGN: A single-center, prospective study. SETTING: A university teaching hospital. PATIENTS: A total of 81 patients with posterior myoma who underwent myomectomy in vNOTES from January 2021 to December 2022. INTERVENTIONS: The patients underwent myomectomy in vNOTES in prone or lithotomy position. MEASUREMENTS AND MAIN RESULTS: Among the patients who underwent vNOTES myomectomy, 29 (35.8%) were in the lithotomy position group, and 52 (64.2%) in the prone position group. Of note, 4 (4.9%) patients underwent a conversion to LESS during the operation-3 in the lithotomy and 1 in the prone position group. And a patient in the lithotomy position group underwent resurgery for hemostasis due to postoperative pelvic bleeding. Compared with the lithotomy position, prone position significantly shortens the operation time (12.3, 95% CI: 6.811, 17.761. p = .009) without increasing the complications and postoperative discomfort of patients. CONCLUSION: Compared to the lithotomy position, the prone position provides greater convenience for operation and exhibits a lower rate of surgical conversion during the removal of single posterior myomas via vNOTES. Further, for patients selecting vNOTES, surgeons need to conduct sufficient preoperative evaluation, timely hemostasis during surgery, and timely surgical conversion if necessary to ensure patient safety.

Transvaginal natural orifice endoscopic surgery for tubal ectopic pregnancy: A more suitable surgical approach for enhanced recovery after surgery.

Objective: We aimed to determine the safety of Vaginal natural orifice transluminal endoscopic surgery (vNOTES) in terms of the Enhanced Recovery after Surgery (ERAS) concept for tubal pregnancy surgery and provide a detailed process of vNOTES for tubal pregnancy surgery, including experience and key points for surgeons performing this procedure. Methods: The Longitudinal Vaginal Natural Orifice Transluminal Endoscopic Surgery Study (LovNOTESS), which was conducted in Chengdu, China. A total of 219 patients who underwent tubal ectopic pregnancy surgery between September 2021 and March 2022. The patients underwent salpingectomy or salpingostomy using transumbilical laparoendoscopic single-site surgery (LESS) or vNOTES, according to their preferences. This study prospectively collected perioperative and one-year follow-up data on tubal pregnancy outcomes after vNOTES and compared them with those after LESS. Results: The vNOTES group showed a shorter surgical duration, hospitalization duration, and postoperative exhaust time and a lower analgesic medication usage rate, but it showed a higher surgical conversion rate. The vNOTES approach reduced the postoperative exhaust time by approximately 9 h (95% confidence interval [CI]: -11.93, -5.57 h, p < .001) and the risk of postoperative analgesic drug use by 77% (odds ratio, 0.23; 95% CI: 0.10, 0.61, p = .023). Conclusion: vNOTES can shorten the exhaust time and duration of hospitalization, reduce postoperative pain, and avoid surface surgical scars in tubal pregnancy surgeries, consistent with the ERAS concept. However, more comprehensive preoperative evaluation of patients who choose vNOTES is required to reduce the occurrence of intraoperative conversion.Trial registration: ChiCTR2100053483.

Different Nuss procedures and risk management for pectus excavatum after surgery for congenital heart disease.

PURPOSE: In Nuss procedure for pectus excavatum (PE) after surgery for congenital heart disease (CHD), retrosternal adhesion may increase the risk of cardiac injury. This study aimed to explore different Nuss procedures and their implications for reducing the incidence of serious complications and improving surgical safety. METHODS: We retrospectively reviewed 49 cases (29 male and 20 female) of Nuss procedures for PE after surgery for CHD that were performed between April 2003 and December 2016. The median age was 5.8 years (range, 3.0-17.9), and the median Haller index was 4.6 (3.2-17.7). All patients were evaluated on their cardiac function and severity of PE by echocardiography and computed tomography scan, respectively. Three surgical procedures were used. Perioperative conditions were analyzed, including CHD type, interval between two operations, blood loss, operation time, hospital stay, complications, and postoperative results. RESULTS: All 49 cases were completed successfully. Fourteen cases (28.6%) involved the standard three-incision thoracoscopic Nuss procedure, 30 cases (61.2%) involved the Nuss procedure assisted by a median sternum incision, and 5 cases (10.2%) involved the Nuss procedure with sternal suspension. The median interval between the CHD surgery and Nuss procedure was 4.0 years (0.5-12.0). The median blood loss was 2.0 mL (1.0-150.0 mL). The median operation time was 45.0 min (27.0-230.0), and the median hospital stay was 6.0 days (5.0-9.0). Three patients (6.1%) experienced severe surgical complications: 2 experienced a rupture of the right atrium and 1 had pericardial injury. Patients were followed up for 7-120 months after surgery. The postoperative results were excellent in 46 cases (93.9%) and good in 3 (6.1%). Twenty-four of the 49 patients have had their bars removed. The median time for bar removal was 36.0 months (24.0-47.0). The outcome after bar removal surgery was excellent in 20 cases and good in 4. CONCLUSIONS: Patients may develop PE or worsening of preexisting PE after open heart surgery for CHD. Surgery for PE can still be performed by the standard Nuss technique without increasing the risk of cardiac injury for the patients that have had interventional cardiology procedures for CHD previously. However, the risk of cardiac injury during the Nuss procedure dramatically increases due to retrosternal adhesions that develop after open heart surgery for CHD. In our experience, the Nuss procedure is safe and feasible after open heart surgery for CHD when performed by an experienced pectus surgeon using an individualized surgical plan for each patient. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level IV.

AxonTracer: a novel ImageJ plugin for automated quantification of axon regeneration in spinal cord tissue.

BACKGROUND: Quantification of axon regeneration in spinal cord tissue sections is a fundamental step to adequately determine if an applied treatment leads to an anatomical benefit following spinal cord injury. Recent advances have led to the development of therapies that can promote regeneration of thousands of injured axons in vivo. Axon labeling methods and in the application of regeneration-enabling stem cell grafts have increased the number of detectable regenerating axons by orders of magnitudes. Manual axon tracing in such cases is challenging and laborious, and as such there is a great need for automated algorithms that can perform accurate tracing and quantification in axon-dense tissue sections. RESULTS: We developed "AxonTracer", a fully automated software algorithm that traces and quantifies regenerating axons in spinal cord tissue sections. AxonTracer is an open source plugin for the freely available image-processing program ImageJ. The plugin identifies transplanted cells grafts or other regions of interest (ROIs) based on immunohistological staining and quantifies regenerating axons within the ROIs. Individual images or groups of images (batch mode) can be analyzed sequentially. In batch mode, a unique algorithm identifies a reference image for normalization, as well as a suitable image for defining detection parameters. An interactive user interface allows for adjustment of parameters defining ROI size, axon detection sensitivity and debris cleanup. Automated quantification of regenerating axons by AxonTracer correlates strongly with semi-manual quantification by the widely-used ImageJ plugin NeuronJ. However, quantification with AxonTracer is automated and reduces the need for user input compared to alternative methods. CONCLUSIONS: AxonTracer is a freely available open-source tool for automated analysis of regenerating axons in the injured nervous system. An interactive user interface provides detection-parameter adjustment, and usage does not require prior image analysis experience. Raw data as well as normalized results are stored in spreadsheet format and axon tracings are superimposed on raw images allowing for subjective visual verification. This software allows for automated, unbiased analysis of hundreds of axon-dense images, thus providing a useful tool in enabling in vivo screens of axon regeneration following spinal cord injury.

Force required for correcting the deformity of pectus carinatum and related multivariate analysis.

OBJECTIVE: To measure the force required for correcting pectus carinatum to the desired position and investigate the correlations of the required force with patients' gender, age, deformity type, severity and body mass index (BMI). METHODS: A total of 125 patients with pectus carinatum were enrolled in the study from August 2013 to August 2016. Their gender, age, deformity type, severity and BMI were recorded. A chest wall compressor was used to measure the force required for correcting the chest wall deformity. Multivariate linear regression was used for data analysis. RESULTS: Among the 125 patients, 112 were males and 13 were females. Their mean age was 13.7±1.5 years old, mean Haller index was 2.1±0.2, and mean BMI was 17.4±1.8 kg/m2. Multivariate linear regression analysis showed that the desirable force for correcting chest wall deformity was not correlated with gender and deformity type, but positively correlated with age and BMI and negatively correlated with Haller index. CONCLUSIONS: The desirable force measured for correcting chest wall deformities of patients with pectus carinatum positively correlates with age and BMI and negatively correlates with Haller index. The study provides valuable information for future improvement of implanted bar, bar fixation technique, and personalized surgery. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level 3-4.

Analysis of MTR and MTRR Gene Polymorphisms in Chinese Patients With Ventricular Septal Defect.

BACKGROUND: Congenital heart defects (CHDs) are the most common birth defects and ventricular septal defects (VSDs) are one of the most common types of CHDs. Genes involved in homocysteine/folate metabolism may play important roles in CHDs. Methionine synthase and methionine synthase reductase (MTRR) are key regulatory enzymes involved in the metabolic pathway of homocysteine. METHODS: We investigated whether a polymorphism (A2756G) of the methionine synthase and 2 polymorphisms (A66G and C524T) of the MTRR gene are associated with VSDs. A total of 183 children with VSDs and 201 healthy children were studied. RESULTS: The polymorphisms were detected by polymerase chain reaction amplification and sequencing of the amplified product. Significant differences in the distributions of the A66G and C524T alleles were observed between VSD cases and controls, and a slightly increased risk of VSDs was associated with either of the 66AG, 524CT, and 524TT genotypes [odds ratios (OR)=1.796, 1.909, and 2.088, respectively]. The genotype frequency of 66AG in VSDs patients was significantly different from those of controls (ORs=3.147). In addition, the combined 66AG/524CT and 66GG/524TT in VSDs had ORs 2.937 and 5.344, respectively. CONCLUSIONS: MTRR A66G and C524T polymorphisms are associated with increased risk of VSDs.

Design and preparation of polyurethane-collagen/heparin-conjugated polycaprolactone double-layer bionic small-diameter vascular graft and its preliminary animal tests.

BACKGROUND: People recently realized that it is important for artificial vascular biodegradable graft to bionically mimic the functions of the native vessel. In order to overcome the high risk of thrombosis and keep the patency in the clinical small-diameter vascular graft (SDVG) transplantation, a double-layer bionic scaffold, which can offer anticoagulation and mechanical strength simultaneously, was designed and fabricated via electrospinning technique. METHODS: Heparin-conjugated polycaprolactone (hPCL) and polyurethane (PU)-collagen type I composite was used as the inner and outer layers, respectively. The porosity and the burst pressure of SDVG were evaluated. Its biocompatibility was demonstrated by the 3-(4,5-dimethyl-2-thiazol)-2,5-diphenyl-2H tetrazolium bromide (MTT) test in vitro and subcutaneous implants in vivo respectively. The grafts of diameter 2.5 mm and length 4.0 cm were implanted to replace the femoral artery in Beagle dog model. Then, angiography was performed in the Beagle dogs to investigate the patency and aneurysm of grafts at 2, 4, and 8 weeks post-transplantation. After angiography, the patent grafts were explanted for histological analysis. RESULTS: The double-layer bionic SDVG meet the clinical mechanical demand. Its good biocompatibility was proven by cytotoxicity experiment (the cell's relative growth rates (RGR) of PU-collagen outer layer were 102.8%, 109.2% and 103.5%, while the RGR of hPCL inner layer were 99.0%, 100.0% and 98.0%, on days 1, 3, and 5, respectively) and the subdermal implants experiment in the Beagle dog. Arteriography showed that all the implanted SDVGs were patent without any aneurismal dilatation or obvious anastomotic stenosis at the 2nd, 4th, and 8th week after the operation, except one SDVG that failed at the 2nd week. Histological analysis and SEM showed that the inner layer was covered by new endothelial-like cells. CONCLUSION: The double-layer bionic SDVG is a promising candidate as a replacement of native small-diameter vascular graft.

Association of NFATc1 gene polymorphism with ventricular septal defect in the Chinese Han population.

BACKGROUND: Congenital heart disease (CHD) is a diverse group of diseases determined by genetic and environmental factors. Considerable research has been done on genes associated with the development of the heart. Recently, focus is on the role of transcription factor NFATc1 in the development of proper valve and septa. As part of a larger study, high density single nucleotide polymorphism (SNP) scanning was used to explore the relationship between NFATc1 gene polymorphism and susceptibility to ventricular septal defect (VSD) in the Chinese Han population. METHODS: One hundred and ninety-two pediatric patients with congenital VSD and 192 matching healthy control subjects were studied. The haplotype reconstructions were calculated by PHASE2.0 software. Haploview software was used to perform linkage disequilibrium assessment and define haplotype blocks. The algorithm used for defining the blocks was the confidence interval method. RESULTS: The NFATc1 gene region can be divided into 11 haplotype blocks. Strong linkage disequilibrium existed within blocks 6, 8, 9, and 11. Three SNPs (rs7240256, rs11665469, and rs754505) within the NFATc1 gene had significant correlation with VSD by single marker association analysis. In addition, two haplotypes correlated with VSD. CONCLUSIONS: NFATc1 is associated with the occurrence of VSD and it may be a predisposing gene to CHD in Han Chinese. This finding has set a direction for further genetic and functional studies.

Surgical treatment for vascular anomalies and tracheoesophageal compression.

BACKGROUND: Vascular rings are uncommon anomalies in which preferred strategies for diagnosis and management may vary among institutions. In this study, we reported our approach and a review of our 5-year experience. METHODS: From May 2006 to April 2011, 45 children (31 boys) with vascular rings underwent surgical repair at Beijing Children's Hospital. Nineteen patients (26%) had associated heart anomalies. RESULTS: There were two hospital deaths. At follow-up, 11 patients still had intermittent respiratory symptoms, but these symptoms had no effect on growth or physical activities. No patients required reoperation. CONCLUSIONS: The rates of misdiagnosis and missed diagnosis of vascular rings are higher than those of other congenital heart diseases. A high index of clinical suspicion coupled with the use of computed tomography enables early diagnosis. Surgical repair can be performed successfully, although a number of patients will have persistent symptoms.

Novel mutations of NODAL gene in Chinese patients with congenital heart disease.

Congenital heart disease (CHD) is one of most common birth defects threatening newborns' health. Over the past few decades, a variety of CHD-causing gene mutations have been identified, but the pathogenic mechanism of congenital heart disease is yet not very clear. The aim of this study was to identify potential pathologic mutations in the NODAL gene and to gain insight into the etiology of CHD. By using amplification with polymerase chain reaction and sequence analysis of NODAL in 800 patients with nonsyndromic CHD and 250 healthy controls, we identified 3 nonsynonymous variants. One of them was first identified in the present study. These variants were not observed in 250 controls. To our knowledge, this is the first study to suggest that NODAL may be involved in the etiology of nonsyndromic CHD in a Chinese population.

Two novel HAND1 mutations in Chinese patients with ventricular septal defect.

BACKGROUND: The HAND1 gene encodes a basic helix-loop-helix (bHLH) transcription factor which plays an essential role in the development of heart. Mutations in HAND1 have been identified in congenital heart disease (CHD) patients with hypoplastic hearts and septal defects. The spectrum of CHD relating to HAND1 mutations needs further study. METHODS AND RESULTS: We screened HAND1 coding regions for mutations in 498 Chinese patients with CHD and 250 control subjects. We identified two novel non-synonymous mutations, c.217G>A (p.Gly73Ser) and c.456G>T (p.Lys152Asn), in the patients with ventricular septal defect (VSD). The two mutations were located in HAND1 evolutionarily conserved residues and enhanced the capability of HAND1 to form homodimers. CONCLUSIONS: This is the first report of mutations in the HAND1 gene in Chinese patients with VSD and provides new insight into the etiology of VSD.

Two novel mutations of the IRX4 gene in patients with congenital heart disease.

IRX4 was the first identified cardiac transcription factor that is restricted to the ventricles at all stages of heart development. Irx4-deficient mice show ventricular dysfunction and develop cardiomyopathy. To study the potential impact of sequence variations in IRX4 on congenital heart disease (CHD) in humans, we examined the coding region of IRX4 in a cohort of 698 Chinese people with congenital heart disease and 250 healthy individuals as the controls. We found two potential disease-causing mutations, p. Asn85Tyr and p. Glu92Gly. A mammalian two-hybrid assay showed that both of the mutations significantly affected the interaction between IRX4 and RXRA. It demonstrated that IRX4 had a potential causative impact on the development of congenital heart disease, particularly ventricular septal defect.

Transcription factor HAND2 mutations in sporadic Chinese patients with congenital heart disease.

BACKGROUND: The basic helix-loop-helix transcription factor HAND2 plays an essential role in cardiac morphogenesis. However, the prevalence of HAND2 mutations in congenial heart disease (CHD) and the correlation between the HAND2 genotype and CHD phenotype have not been studied extensively. METHODS: We amplified the exons and the flanking intron sequences of the HAND2 gene in 131 patients diagnosed with congenital defects of the right ventricle, outflow tract, aortic artery or cardiac cushion and confirmed the mutations by sequencing. RESULTS: Seven mutations including three missense mutations (P11R, S36N and V83L), one isonymous mutation (H14H) and three mutations in untranslated region (241A > G, 604C > T and 3237T > A) were identified in 12 out of the 131 patients. Both nonisonymous mutations are located in the transcriptional activation domain on the N-terminus. Only one mutation (S36N) was identified in 250 normal healthy controls. The distribution of 3637T > A is the unique one which was different between the 2 groups. CONCLUSIONS: HAND2 may be a potential candidate gene of stenosis of the right ventricle, outflow tract. Further study of those with a family history of HAND2 mutations will help convincingly relate their genotype to the pathogenesis of CHD.

Screening NKX2.5 mutation in a sample of 230 Han Chinese children with congenital heart diseases.

Congenital heart disease (CHD) is the most common developmental anomaly, affecting approximately 1% of all newborns. Genetic factors play an important role in CHD's development. Germline mutations in NK2 transcription factor related, locus 5 (NKX2.5) have been identified as the factors responsible for various forms of CHD. In this study, we investigated mutations of the NKX2.5 gene's coding region in 230 nonsyndromic CHD patients belonging to the Chinese Han nationality by PCR, denaturing high-performance liquid chromatography, and sequencing. Pathogenic mutations were not found among the patients. Two known single-nucleotide polymorphisms (rs2277923 and rs3729753) were detected, but the differences in the allele and genotype frequencies were insignificant between CHD and the controls (p > 0.05). The data we gathered suggest that NKX2.5 mutations are highly rare in CHD patients of the Chinese Han nationality. Therefore, NKX2.5 mutation investigation should be limited within a number of familial and special phenotype of CHD in Chinese patients.

GATA4 and NKX2.5 gene analysis in Chinese Uygur patients with congenital heart disease.

BACKGROUND: Congenital heart disease (CHD) is the most common developmental anomaly in newborns. The germline mutations in GATA4 and NKX2.5 genes have been identified as responsible for CHD. The frequency of GATA4 and NKX2.5 mutations in Chinese Uygur patients with CHD and the correlation between their genotype and CHD phenotype are unknown. METHODS: We examined the coding region of GATA4 and NKX2.5 genes in 62 Chinese Uygur patients with CHD and 117 Chinese Uygur individuals as the controls by denaturing high performance liquid chromatography (DHPLC) and sequencing. RESULTS: Two heterozygous missense mutations of c.1220C > A and c.1273G > A in GATA4 gene, which cause the amino acid residue changes of P407Q and D425N in GATA4, were found in a patient with tetralogy of Fallot and a patient with ventricular septal defect, respectively. The two patients did not have atrioventricular conduct defects or non-cardiac abnormalities. The two mutations are expected to affect the protein function. There were no reported NKX2.5 mutations in the patients. CONCLUSION: Our results provided the primary data on CHD phenotype associated with GATA4 mutation in the Chinese Uygur population.

Association of TBX5 gene polymorphism with ventricular septal defect in the Chinese Han population.

BACKGROUND: Congenital heart disease is a diverse group of diseases determined by genetic and environmental factors. Considerable research has been done on genes associated with development of the heart. A recent focus is the role of transcription factor TBX5 in the development of atria, left ventricle and conduction system. As part of a larger study, high density, single nucleotide polymorphism (SNP) scanning was used to explore the relationship between TBX5 gene polymorphism and susceptibility to ventricular septal defect not associated with forelimb malformation in the Chinese Han population. METHODS: One hundred and ninety two paediatric patients with congenital ventricular septal defect and 192 matched healthy control subjects were studied. The haplotype reconstructions were calculated by PHASE2.0 software. Haploview software was used to perform linkage disequilibrium assessment and defining of haplotype blocks. The algorithm used for defining of blocks was the confidence interval method. RESULTS: The TBX5 gene region can be divided into 3 haplotype blocks of 27, 15 and 2 SNPs. Strong linkage disequilibrium exists within each block. SNP rs11067075 within the TBX5 gene had significant correlation with ventricular septal defect (P = 0.0037) by single marker association analysis. In addition, a 20 kb haplotype composed of 27 SNPs correlated with ventricular septal defect (P = 0.05, multiple loci regression analyses based on reconstructed haplotype blocks). CONCLUSIONS: TBX5 is associated with the occurrence of ventricular septal defect and may be a predisposing gene to congenital heart disease in Han Chinese. This finding has set a direction for further genetic and functional studies.

T-box transcription factor TBX20 mutations in Chinese patients with congenital heart disease.

Despite animal studies having demonstrated that Tbx20 is essential for heart development, few studies have been conducted about TBX20 and congenital heart disease (CHD) in humans. Recently two TBX20 mutations have been associated with human heart defects in two Caucasian families, but TBX20 mutations underlying the more common isolated forms of CHD are still unknown. To explore this question and to analyze the association between TBX20 and susceptibility to CHD 203 Chinese patients with a variety of predominantly sporadic CHD and 300 control subjects were investigated for TBX20 mutations. The exon 2-6 contributing to the T-box DNA-binding domain and their flanking intron sequences were amplified by polymerase chain reaction (PCR) and then were sequenced after purification. Three non-synonymous mutations (A63T, I121F, and T262M) were identified in 3 patients, which were not seen in 300 controls. I121F and T262M mutations occurred within the highly conserved T-box DNA-binding domain. Two synonymous sequence variants (N222N, T262T) and one intervening variant (IVS2-5insCT) were observed in 3 patients but not in the controls. In addition, eight SNPs were observed both in patients and controls and four (S167S, P177P, A181A, and I219I) of them are novel. These data indicate that the frequency of TBX20 missense mutations occurred in Chinese CHD children is low, but they probably contribute to the risk of atrial septal defect (ASD), total anomalous pulmonary venous connection (TAPVC) and tetralogy of Fallot (TOF) in a small subset of Chinese. The findings provide the first insight into TBX20 mutations for TOF and TAPVC. Functional study involved in the new sequence variants should be subject of further investigation.

GATA4 mutations in 486 Chinese patients with congenital heart disease.

Recent studies have reported germline mutations in GATA4 gene in some types of congenital heart disease (CHD). However, the prevalence of GATA4 mutations in CHD and the correlation between the GATA4 genotype and CHD phenotype have not been extensively studied. We screened germline mutations in the coding exons and the flanking intron sequences of the GATA4 gene in 486 CHD patients by denaturing high-performance liquid chromatography (DHPLC), and confirmed the mutations by sequencing. Nine distinct mutations including one small deletion mutation (46delS), two small insertion mutations (118-119insA and 125-126insAA), and six non-synonymous mutations (A6V, P163S, E359K, P407Q, S429T and A442V) were identified in 12 of the 486 patients (nine with ventricular septal defect, two with Tetralogy of Fallot, and one with endocardial cushion defect). Of them, two patients carrying E359K mutation were from two generations in one family with ventricular septal defect (VSD). Interestingly, a nucleotide insertion of c.1146+25insA in exon 6 was detected in five VSD patients, but not in 486 normal healthy controls. Our findings are useful in understanding the prevalence of GATA4 mutations and the correlation between the GATA4 genotype and the CHD phenotype in Chinese patients.

Congenital vascular rings: a rare cause of respiratory distress in infants and children.

BACKGROUND: Congenital vascular rings may often cause unexplained respiratory symptoms in infants and young children. Their diagnosis and treatment are often delayed. Few studies of vascular rings have been reported in China. The aim of this study was to describe the clinical presentation, diagnosis and surgical management of infants and children with congenital vascular rings. METHODS: Clinical histories, physical examinations, investigations, image studies and surgical interventions were retrospectively evaluated in 7 children (age range: 2 months-4 years, mean 7 months) with congenital vascular rings. Chest radiography was performed in all patients. Echocardiography and computed tomography (CT) with 3-dimensional (3D) reconstructions were performed in 6 patients. Esophagography, cardiac catheterization and angiography, and bronchoscopy were performed in 1, 1 and 4 children, respectively. RESULTS: Six of the 7 patients had respiratory symptoms, including recurrent cough, stridor and wheeze. Age at onset of symptoms ranged from 1 month to 11 months. Chest X-ray showed nothing important on the vascular rings, besides bronchitis and pneumonia. Contrast-enhanced CT diagnosed vascular rings in 6 patients. Four patients had double aortic arches, two had balanced arches and two were right arch dominant. One patient had a right aortic arch with left ligament and 1 patient had a pulmonary artery sling. Echocardiography failed to diagnose vascular rings in 2 patients. The esophagogram of 1 patient showed esophageal compression. Bronchoscopy of 4 patients showed compression of the distal trachea. Five of the 7 patients underwent surgical division of the vascular rings. Surgical observation confirmed the CT findings in each patient. CONCLUSIONS: Patients, especially infants or young children, with recurrent respiratory symptoms such as chronic cough, stridor and wheeze, should be examined for the possible presence of congenital vascular rings. Contrast-enhanced CT can clearly show the anatomy of vascular rings. As a noninvasive technique, echocardiography is helpful for diagnosis. Early surgical management in symptomatic patients is effective.