BackgroundLimited study has shown whether NT-proBNP is related to the prognosis of children wth ventricular septal defect (VSD) surgery. The study was conducted to determine the predictive value of NT-proBNP on outcomes of children with VSD surgery.MethodsA total of 798 children with VSD surgery were enrolled, with NT-proBNP measured at preoperatively and 24-h postoperatively. The short- and mid-term clinical outcomes were recorded. Propensity scores (PS) was performed to acquire pre-op and post NT-proBNP 24-h PS-matched cohorts for comparisons between groups.ResultsIn the pre NT-proBNP PS-matched cohort, the higher NT pro-BNP group had longer hospitalization time and lower post-op 1-month EF value compared with low NT pro-BNP group (all P < 0.05), and there wasn't significant difference of mechanical ventilation time, cardiopulmonary bypass (CPB) time, intensive care unit (CCU) stay, and ejection fraction (EF) values of 3 month to 12 months after surgery (all P > 0.05). In the post NT-proBNP PS-matched cohort, there wasn't significant difference of mechanical ventilation time, CPB time, CCU stay, hospitalization time, and EF values of 1 month to 12 months after surgery between two groups (all P > 0.05).ConclusionsVSD children with higher pre NT-proBNP level had longer hospital stays after surgery than those with lower level. Pre NT-proBNP level had no effect on mechanical ventilation time, CPB time, ACC time and CCU stay and cardiac function after 3 months postoperatively. Post-op 24-h NT pro-BNP level wasn't associated with clinical outcomes.

BACKGROUND:We had reported that postoperative EEG background including sleep-wake cycle (SWC) and discharge (seizures, spikes/sharp waves) abnormalities were significantly correlated with adverse early outcomes in children after cardiac surgery. We aimed to analyze the relations between these EEG abnormalities and neurodevelopmental outcomes at about 2 years after cardiac surgery. METHODS:We enrolled 121 patients undergoing cardiac surgery at 3.3 months (0.03 ~ 28 months). EEG abnormalities described above during the first postoperative 48 h were evaluated. Griffiths Mental Development Scales-Chinese was used to evaluate the quotients of overall development and 5 subscales of the child's locomotor, language, personal-social, eye-hand coordination and performance skills at 16 ~ 31 months of age. RESULTS:EEG background abnormalities occurred in 59/121 (48.8%) patients and 33 (55.9%) unrecovered to normal by 48 h. Abnormal SWC occurred in 15 (12.4%) patients and 7 (5.8%) unrecovered to normal by 48 h. EEG seizures occurred in 11 (9.1%) patients with frontal lobe seizures in 4. Spikes/sharp waves occurred in 100 (82.6%). EEG background abnormalities, number of spikes/sharp waves and frontal lobe seizures were significantly associated with neurodevelopmental impairment at about 1 ~ 2 year after surgery (Ps ≤ 0.05). CONCLUSIONS:Most parameters of EEG abnormalities were significantly associated with neurodevelopmental impairment after cardiac surgery. IMPACT:Neurodevelopmental impairment in children with congenital heart disease remain poorly understood. Previous studies had reported that either EEG seizures or background abnormalities were associated with worse neurodevelopmental outcomes. Our present study showed that all the EEG background and discharge abnormalities including EEG background, seizures and spikes/sharp waves in the early postoperative period were significantly associated with neurodevelopmental impairment at about 1 ~ 2 years after cardiac surgery. Comprehensive evaluation of early postoperative EEG may provide further insights about postoperative brain injury, its relation with neurodevelopmental impairment, and guide to improve clinical management.

目的 探讨经胸超声心动图诊断儿童先天性左心耳瘤的图像特点.资料与方法 分析2017年7月—2021年4月广州市妇女儿童医疗中心经胸超声心动图诊断并手术或CT确诊的7例先天性左心耳瘤的病例资料及随访结果,总结其声像图特征.结果 7例均表现为左前外侧一囊性暗区,多位于心包腔内,壁光整,薄,内有血流与左心房相通,彩色多普勒提示交通口处血流呈双向.7例中,1例为单纯左心耳瘤,手术后预后良好,2例合并近闭合的室间隔缺损,长期随访中,1例合并左心室发育不良综合征放弃治疗后死亡;3例合并复杂性先天性心脏病,均行相应手术治疗,预后良好,所有病例均无明显心律失常,未见明显血栓回声.结论 经胸超声心动图可以早期发现并诊断儿童先天性左心耳瘤.

先天性冠状动脉瘘是指冠状动脉和心腔或大血管间存在异常交通，是一种少见疾病，瘘入右心室壁内极为罕见。本文报道1例6岁男性儿童右冠状动脉右心室壁内瘘形成巨大夹层瘤，准确诊断并成功手术治疗的病例。冠状动脉心室壁内瘘存在破裂风险，发现后应尽早手术；超声心动图及冠状动脉造影对冠状动脉瘘诊断具有重要意义。

Objective Balloon angioplasty (BA) has been the treatment of choice for pulmonary artery stenosis (PAS) in children. There remains, however, a significant proportion of resistant lesions. The ultra-high pressure (UHP) balloons might be effective in a subset of these lesions. In this study, we analyzed the safety and efficacy with short- to mid-term follow-up results of UHP BA for PAS in children with congenital heart defects (CHD) in our center. Methods This is a retrospective cohort study in a single tertiary heart center. Children diagnosed with PAS associated with CHD were referred for UHP BA. All data with these children were collected for analysis with updated follow-up. Results A total of 37 UHP BAs were performed consecutively in 28 children. The success rate was 78.4%. A significantly (P = 0.005) larger ratio of the balloon to the minimal luminal diameter at the stenotic waist (balloon/waist ratio) was present in the success group (median 3.00, 1.64–8.33) compared to that in the failure group (median 1.94, 1.41 ± 4.00). Stepwise logistic regression analysis further identified that the balloon/waist ratio and the presence of therapeutic tears were two independent predictors of procedural success. The receiver operating characteristic curve revealed a cut-off value of 2.57 for the balloon/waist ratio to best differentiate success from failure cases. Signs of therapeutic tears were present in eight cases, all of whom were in the success group. Perioperative acute adverse events were recorded in 16 patients, including 11 pulmonary artery injuries, three pulmonary hemorrhages, and two pulmonary artery aneurysms. During a median follow-up period of 10.4 (0.1–21.0) months, nine cases experienced restenosis at a median time of 40 (4–325) days after angioplasty. Conclusions The UHP BA is safe and effective for the primary treatment of PAS in infants and children with CHD. The success rate is high with a low incidence of severe complications. The predictors of success are a larger balloon/waist ratio and the presence of therapeutic tears. The occurrence of restenosis during follow-up, however, remains a problem. A larger number of cases and longer periods of follow-up are needed for further study.

目的:为提高体静脉连接异常的疾病认知,评估体静脉连接异常超声心动图的诊断价值.方法:回顾分析2012年7月—2021年6月广州市妇女儿童医疗中心收治的42例经手术、心血管造影或心脏CT明确诊断为体静脉连接异常患儿的超声心动图特征.所有患儿均进行经胸超声心动图(TTE)诊断,评估其诊断体静脉连接异常的诊断价值.结果:上腔静脉连接异常29例(69.05%),其中左上腔静脉连接异常11例:直接回流入左房9例,经无顶冠状静脉窦回流入左房2例;右上腔静脉连接异常18例:右上腔静脉缺如并左上腔静脉15例,右上腔静脉直接回流入左房3例.下腔静脉连接异常13例(30.95%),其中下腔静脉离断11例,下腔静脉回流入左房1例,双下腔静脉1例.超声心动图准确诊断40例(95.24%),漏诊2例(4.76%).结论:体静脉连接异常的存在与否对外科有明显的影响,应提高其疾病认知,超声心动图因其方便、无放射性等优点,且诊断准确率高,应成为首选的检查.

Objective: Neurodevelopmental impairment has been realized as the most common complication in children with congenital heart disease undergoing cardiac surgery during the past 30 years. But little attention has been paid to this problem in China. The potential risk factors for adverse outcomes include demographic, perioperative, and socioeconomic factors, which are vastly different in China compared with the developed countries in previous reports. Methods: Four hundred twenty-six patients (aged 35.9 +/- 18.6 months) at about 1to 3-year follow-up after cardiac surgery were prospectively enrolled from March 2019 to February 2022. Griffiths fi ths Mental Development Scales-Chinese was used to evaluate the quotients of overall development and 5 subscales of the child's ' s loco- motor, language, personal-social, eye-hand coordination, and performance skills. Demographic, perioperative, socioeconomic, and feeding type during the fi rst year of life (breastfeeding, mixed, or never breastfeeding) were examined to identify the risk factors for adverse neurodevelopmental outcomes. Results: Mean scores were 90.0 +/- 15.5 for development quotient, 92.3 +/- 19.4 for locomotor, 89.6 +/- 19.2 for personal-social, 85.5 +/- 21.7 for language, 90.3 +/- 17.2 for eye-hand coordination, and 92 +/- 17.1 for performance subscales. For the entire cohort, the impairment in at least 1 subscale was found in 76.1% % of the cohort (>1 > 1 SD below population mean) with 50.1% % being severe (>2 > 2 SDs below the mean). The significant fi cant risk factors included prolonged hospital stay, peak level of postoperative C-reactive protein, socioeconomic status, and never breastfeeding or mixed feeding. Conclusions: Neurodevelopmental impairment is substantial in terms of incidence and severity in children with congenital heart disease undergoing cardiac surgery in China. Risk factors contributing to the adverse outcomes included prolonged hospital stay, early postoperative inflammatory fl ammatory response, socioeconomic status, and never breastfeeding or mixed feeding. There is an urgent need for standardized follow-up and neurodevelopmental assessment in this special group of children in China. (JTCVS Open 2023;14:462-71)

目的 分析右上腔静脉回流入左房的超声心动图的图像特征,评价其诊断价值.方法 3例经心外科手术明确诊断为右上腔静脉回流入左房的患儿为研究对象,对其超声心动图检查结果进行回顾性分析.结果 超声心动图诊断的右上腔静脉回流入左房多为孤立性畸形,主要表现为上腔静脉骑跨于房间隔之上,二维超声显示其近乎完全与左房连接,彩色多普勒显示其内血流绝大部分进入左房,其中3例均合并卵圆孔未闭,2例合并右上肺静脉的异位引流,1例合并上腔静脉型的房间隔缺损.结论 超声心动图是诊断右上腔静脉回流入左房的首选方法,临床上应提高对该病的认知.

Objective:To summarize the diagnostic and surgical experience of children with scimitar syndrome and to explore the immediate/mid-term outcomes of these operations.Methods:Retrospective analysis was performed for 14 clinical records of children with scimitar syndrome from January 2010 to December 2017. There were 7 boys and 7 girls with a mean age of (18.23±28.55 months)(23 days to 9 years). The complications included other cardiac abnormalities ( n=10), mesocardia ( n=8), right pulmonary hypoplasia ( n=7) and collaterall circulation ( n=8). Follow-ups were conducted for analyzing the immediate/mid-term outcomes of survival and stenosis of corrected scimitar vein (SV). And postoperative survival curves were plotted. Results:SVs were dissected from inferior caval vein (IVC) and anastomosed to left artrium ( n=9); SVs (2 SVs) were resected together with partial IVC wall and anastomosed to left artrium ( n=1); SVs (2 SVs) were resected respectively and anastomosed side-to-side and then anastomosed to left artrium ( n=1); SVs were anastomosed to right atrium and interatrium septum be rebuilt ( n=2) and SV was connected to artificial conduit and then connected to left atrium ( n=1) owing to that SV was too far to left atrium. In the same term of operation, complicated cardiac abnormality was cured. And 2/14 cases died immediately postoperatively with a mortality rate of 14.29%; 1/12 survivors died of pneumonia and respiratory failure at 14 months postoperatively and 1/3/5-year postoperative cumulative survival rates were 86%, 73% and 73% respectively. One corrected SV of 12 survivors was stenosed during discharge and 3 corrected SVs of 11 survivors became stenotic during follow-ups. Conclusions:Scimitar syndrome may be complicated with other cardiac abnormality, mesocardia and pulmonary hypoplasia, etc. Most SVs of scimitar syndrome may be cured by anastomosing SV to left artrium. SV should be anastomosed to right atrium instead of left atrium and interatrium septum. Collateral circulation of scimitar syndrome may be plugged during cardiac interventions and even resected intraoperatively.

目的 探讨左室收缩功能正常的左冠状动脉异常起源于肺动脉(ALCAPA)患儿的超声心动图特点.方法 回顾性分析2010年1月至2019年12月广州市妇女儿童医疗中心收治并经手术证实的13例左心室射血分数(LVEF)≥55％的ALCAPA患儿的超声心动图特征和临床资料.结果 13例ALCAPA患儿均可见右冠状动脉增宽,其他超声征象包括左冠状动脉血流逆灌12例、左冠状动脉直接开口于肺动脉9例、肺动脉内逆灌血流7例、心肌侧枝血流8例、左室明显增大8例、二尖瓣功能不全7例、左室心内膜纤维化5例.<1岁ALCAPA患儿的心肌侧枝血流信号不明显,左心室大小正常或轻度增大且二尖瓣功能良好;≥1岁患儿可见丰富侧枝血管、左室明显扩大以及二尖瓣功能不全.4例误诊的ALCAPA左冠状动脉均起源于肺动脉右后方.结论 超声心动图可准确诊断左室收缩功能正常的ALCAPA,如果超声科医生在怀疑该诊断时能全面探查冠状动脉的起源和血流方向,可减少漏诊和误诊.

Objective To assess the application value of juxtaposition of the great vessels in diagnosis of right atrial isom-erism( RAI) by echocardiography.Methods We retrospectively analyzed 73 patients with right atrial isomerism in Guangzhou Women and Children's Medical Center from 2010-2017.All these patients were performed by echocardiography and compu-ted tomography(CT).We used the results of CT as the golden standard.Results 73 cases of RAI were diagnosed by CT.60 cases of RAI were diagnosed by echocardiography.Juxtaposition of the descending aorta(DAO) and inferior vena(IVC) was demonstrated in all these 60 patients.29 of 60 were on the left side of the spine(39.73％).31 of 60 were on the right side of spine(42.47％).In the other 13 patients of echocardiography missed diagnosis, the arrangement of the great vessels was nor-mal in 10 cases, and inverse in 3 cases.Conclusion Juxtaposition of the great vessels is one of the most important findings in patients with RAI.It is quick and simple and has a higher application value in diagnosis of RAI.

BACKGROUND:Kawasaki disease (KD) is a systemic form of self-limited vasculitis in children less than five years old, and the main complication is coronary artery injury. However, the etiology of KD remains unclear. The IL-1B polymorphisms rs16944 GG and rs1143627 AA and their diplotype GA/GA have been associated with significantly increased risk of intravenous immunoglobulin (IVIG) resistance in a Taiwanese population, but the relationship between rs16944 A/G and rs1143627 G/A and coronary artery lesions (CALs) in patients with KD has not been investigated. The present study is aimed at investigating whether the rs16944 A/G and rs1143627 G/A polymorphisms in IL-1B were associated with KD susceptibility and CALs in a southern Chinese population.METHODS AND RESULTS:We recruited 719 patients with KD and 1401 healthy children. Multiplex PCR was used to assess the genotypes of single nucleotide polymorphisms (SNPs), including two SNPs of IL-1B, rs16944 A/G and rs1143627 G/A. According to the results, no significant association was observed between the IL-1B (rs16944 and rs1143627) polymorphisms and KD risk in the patients compared with the healthy controls in our southern Chinese population. However, in further stratified analysis, we found that children younger than 12 months with the rs16944 GG and rs1143627 AA genotypes of IL-1B had a higher risk of CALs than those with the AA/AG genotypes of rs16944 and GG/AG genotypes of rs1143627 (OR = 2.28, 95% CI = 1.32-3.95, P = 0.0032, adjusted OR = 2.33, 95% CI = 1.34-4.04, P = 0.0027).CONCLUSIONS:Our results indicated that there was no association between the rs16944 A/G and rs1143627 G/A gene polymorphisms and KD susceptibility. However, the rs16944 GG and rs1143627 AA genotypes of IL-1B may significantly impact the risk of CAL formation in children younger than 12 months, which may contribute to the pathogenesis of KD. These findings need further validation in multicenter studies with larger sample sizes.

Objective To summarize the outcomes and experience of arterial switch operation (ASO) in the past 10 years in our center.Methods From September 2008 to July 2017,238 patients underwent ASO at Guangzhou Women and Children Medical Center for repair of transposition of the great arteries and Taussig-Bing anomaly.There were 193 male and 45 female.Median age at operation was 2 months (2 days to 10 years) and mean body weight was(4.2 ± 1.7) kg (1.8-20.6 kg).There were TGA and VSD 91 cases,TGA/IVS 110 cases,and Taussig-Bing anomaly 37 cases.Among them 24 patients had an aortic arch anomaly.Intramural coronary artery was found in 8 patients.Results All patients successfully completed the operation,one-stage ASO was perfonned in 232 patients.Two-stage ASO was performed in 6 patients.The mortality was 14.2％.The follow-up duration was 1-10 years (median time,46 mouths).There were 3 died.Two suffered sudden death,and another one arrhythmia.10-year survival rate was 92.8％.Conclusion The outcomes of ASO were satisfactory.The long-term reoperation rate was rare.

1病例资料 女,3月龄,因"咳嗽伴发热1周"于2017年8月至广州医科大学附属广州市妇女儿童医疗中心(我院)就诊.出生时体检发现心脏杂音,当地医院诊断为"左冠状动脉-右室瘘、动脉导管未闭、卵圆孔未闭",未行处理.患儿平素无反复呼吸道感染;吃奶时有气促,有间断,无呛咳;大小便未见异常. 患儿系G1P1,足月剖宫产,无窒息抢救史,出生体重3000g.父母均体健,否认近亲结婚,否认遗传病史.家族成员无类似病史.

Objective To explore the diagnostic value of transthoracic echocardiography (TTE) for anomalous origin of right pulmonary artery (AORPA).Methods Echocardiographic data of 26 patients with AORPA proved by surgical operation were analyzed retrospectively.Results TTE showed that the pulmonary trunk and the left pulmonary artery were displayed normally,and the right pulmonary artery originated from the ascending aorta.AORPA in all 26 patients were diagnosed,and the coincidence rate was 100％.Among 26 patients,9 associated with Berry's syndrome,1 combined with coarctation of aorta,22 combined with patent ductus arteriosus,23 combined with atrial septal defect or patent foramen ovale,25 were found with severe pulmonary artery hypertension,and the diagnostic accuracy rate of TTE was 100％ (26/26),100％ (26/26),96.15％ (25/26),92.31％ (24/26) and 100％ (26/26),respectively.Conclusion TTE can early and accurately diagnose AORPA,also has high accuracy in diagnosis of other concomitant malformations.Multi-section scan can reduce misdiagnosis.TTE can be taken as the preferred inspection method for diagnosing AORPA.

Introduction Kawasaki disease is a kind of systemic vasculitis that mainly damages moderate and small-sized blood vessels, and is a leading cause of coronary artery lesions (CAL). Antiplatelet therapy is a routine component of Kawasaki disease treatment strategies. So it is important to evaluate the antiplatelet effect of aspirin because of the individual biological variability of antiplatelet effect of aspirin. The immature platelet fraction (IPF) has attracted particular attention as it may influence the antiplatelet effect of aspirin. This study investigated the prognostic factors for evaluating the degree of vasculitis and the effect of antiplatelet therapy in children with Kawasaki disease. Materials and methods Blood samples were collected from 44 patients with Kawasaki disease before aspirin treatment and 7 to 10 days after treatment. The IPF counts, percentage of the IPF, and highly fluorescent IPF were detected by a Sysmex XE-5000 instrument. The levels of 11-dehydrothromboxane B2 (11-DH-TXB2), soluble CD40 ligand (sCD40L), and soluble P-selectin (sP-selectin) were measured by ELISA. The correlation between the measured factors and the degree of coronary artery damage in Kawasaki disease was analyzed. Results We found that 11-DH-TXB2, sP-selectin, and sCD40L levels were much more elevated in the CAL group than in the non-coronary artery lesions (NCAL) group before aspirin treatment. The concentrations of 11-DH-TXB2, sCD40L, sP-selectin, and IPF were reduced after aspirin treatment in the NCAL group but not the CAL group. This is related to the degree of coronary artery damage in Kawasaki disease patients. Additionally, 11-DH-TXB2, sCD40L, sP-selectin, and IPF were positively correlated with the degree of coronary artery damage in Kawasaki disease patients. Conclusion The current study suggests that the presence of high plasma concentrations of 11-DH-TXB2, sCD40L, sP-selectin, and IPF can be considered a risk factor and experimental biomarker for CAL in Kawasaki disease patients.

Objective To evaluate and analyze echocardiography(ECG) in diagnosis of congenital vascular rings (CVR) to elevate accuracy of diagnosis.Methods 103 cases of children with CVR confirmed by surgery were analyzed retrospectively.We reviewed the ECG and CTA characteristics of this disease with the surgical results as the gold standard.Results Among the 103 CVR patients,61 cases were pulmonary artery sling (PAS),ECG sensitivity was 96.7 ％ (59/61),missed diagnosis rate was 3.3 ％ (2/61).21 cases were double aortic arch(DAA),ECG sensitivity was 57.1％ (12/21),missed diagnosis rate was 42.9 ％ (9/21).19 cases were right aortic arch with an aberrant left subclavian artery(RAA-ALSA),ECG sensitivity was 15.8％(3/19),missed diagnosis rate was 84.2％ (16/19).2 cases were left aortic arch with aberrant right subclavian artery(LAA-ARSA).All the CVR cases were diagnosed by CTA.77 CVR combined with intracardiac malformation and all diagnosed byECG and the missed diagnosis rate was 11.7 ％ for CTA.Conclusions Missed diagnosis are prone to take place in DAA and an aberrant subclavian artery than in PAS with ECG.CTA could show clearly the relationship of vascular rings and trachea.So integration of the two diagnosis methods could hand out more information to operation plan.

Purpose Unilateral absence of pulmonary artery is a rare congenital pulmonary arterydysplasia whose untypical symptom often leads to missed diagnosis or misdiagnosis.Diagnostic and follow-up values of ultrasound cardiogram to pediatric congenital unilateral absence of pulmonary artery (UAPA) are explored in this paper.Materials and Methods Ultrasound cardiogram images of 35 UAPA patients confirmed by surgery or CT angiography in Guangzhou Women and Children's Medical Center from May 2009 to July 2016 were analyzed retrospectively,and images characteristics of the correctly diagnosed and missed diagnosis or misdiagnosed ultrasound cardiogram were analyzed.Results Among these 35 UAPA patients,21 cases were on the right side,14 cases on the left side,16 cases single UAPA and 19 cases combined with other intracardiac malformations.27 cases were initially diagnosed by ultrasound cardiogram,7 cases were missed diagnosed and 1 case misdiagnosed,with 77.1％ diagnosis accuracy.UAPA ultrasound cardiogram showed that normal bifurcation structure of pulmonary artery disappears.Main pulmonary artery continued to be one-side pulmonary artery,running to left or right and forming left branch or right branch of pulmonary artery.Pulmonary artery of the one-side absence of pulmonary segment supplied blood through aorta or other branches or vessels on the other side.After receiving unilateral pulmonary artery reconstruction surgery,ultrasound cardiogram of 8 patients of one month indicated that average flow rate of close-end anastomosis was (1.22± 0.17) m/s and far-end (2.17± 0.56) m/s.Conclusion Ultrasound cardiogram is efficient for UAPA preoperative diagnosis.Meanwhile,it is convenient,noninvasive and efficient in assessing anastomosis and pressure of pulmonary artery after surgery.

Objective To evaluate the difference of tracheobronchial stenosis and airway malformation between double aortic arches and pulmonary artery sling in children. Methods Clinical feature and imaging data of spiral CT were retrospectively analyzed in children with double aortic arches or pulmonary artery sling who was hospitalized from July 2010 to July 2015 . Results There were 16 children ( 11 males and 5 females) with double aortic arches whose median age at onset was 3 . 5 months old. There were 47 children ( 28 males and 19 females) with pulmonary artery sling whose median age at onset was 4-month-old. In these 16 cases of double aortic arches, 14 cases were coupled with tracheobronchial stenosis. Two cases had more than one segment involved and they were segments II or III. Twelve cases had only one segment involved. Different degrees of tracheobronchial stenosis occurred in 47 cases of pulmonary artery sling, among whom 27 cases had more than one segment involved and the common segment was II and III ( 19 cases). Two cases had four segments involved and 20 cases had only one segment involved. There was statistical signiifcance in the number of involved segments between children with double aortic arches and pulmonary artery sling (χ2=13 . 588 , P=0 . 001 ). In 16 cases of double aortic arches, one case was combined with tracheal bronchus, and 1 case was combined with pulmonary hypoplasia. In 47 cases of pulmonary artery sling, 8 cases were combined with bridging bronchus, 3 cases combined with tracheal bronchus, 3 cases combined with pulmonary hypoplasia, 5 cases combined with bronchial deifciency or tracheal diverticula, and 2 cases combined with bronchopulmonary foregut malformation. There was statistical signiifcance in the number of cases combined with airway malformation between children with double aortic arches or pulmonary artery sling (χ2=5 . 333 , P=0 . 021 ). Conclusions The tracheobronchial stenosis and pulmonary airway abnormalities are more prominent in children with pulmonary artery sling than those in children with double aortic arch.

Objective To discuss the value of color Doppler echocardiography in diagnosis of scimitar syndrome .Methods The echocardiographic results of 6 patients with a diagnosis of scimitar syndrome were reviewed retrospectively .Their sonographic and hemodynamic characteristics were also analyzed connected with the reports in the literature .Results Three cases had dextrocardia and the others had mesocardia .All cases got right ventricular dimension enlargement .Total or partial of right pulmonary venous connection to the inferior vena cava were 3 cases respectively .All cases had right pulmonary artery hypoplasia .All of 6 cases echocardiographic results were in accordance with the findings by CT angiography and 4 cases were confirmed by operation .Conclusions The sonographic features of scimitar syndrome were obvious ,and echocardiography was contribute to early diagnosis of scimitar syndrome .