Unilateral absence of a pulmonary artery (UAPA) is a rare congenital malformation resulting from the failed development or premature involution of the sixth aortic arch during embryogenesis, leading to a failure to establish a connection with the main pulmonary artery. Currently, there is a notable lack of consensus regarding the surgical management of UAPA in China. Drawing upon the latest clinical research, this consensus aims to summarize surgical approaches and techniques to improve the clinical management of UAPA patients and serve as a scientific reference for physicians specializing in pediatric cardiology and structural heart disease. This consensus aims to promote the standardization of UAPA diagnosis and treatment, thereby facilitating improved patient outcomes and long-term management, and stimulating the continuous development and innovation of surgical treatment for this condition in China.

Objective To investigate the efficacy of leaflet augmentation technique to repair the recurrent mitral valve (MV) regurgitation after mitral repair in children. Methods A retrospective analysis was conducted on the clinical data of children who underwent redo MV repair for recurrent regurgitation after initial MV repair, using a leaflet augmentation technique combined with a standardized repair strategy at Fuwai Hospital, Chinese Academy of Medical Sciences, from 2018 to 2022. The pathological features of the MV, key intraoperative procedures, and short- to mid-term follow-up outcomes were analyzed. Results A total of 24 patients (12 male, 12 female) were included, with a median age of 37.6 (range, 16.5–120.0) months. The mean interval from the initial surgery was (24.9±17.0) months. All children had severe mitral regurgitation preoperatively. The cardiopulmonary bypass time was (150.1±49.5) min, and the aortic cross-clamp time was (94.0±24.2) min. There were no early postoperative deaths. During a mean follow-up of (20.3±9.1) months, 3 (12.5%) patients developed moderate or severe mitral regurgitation (2 severe, 1 moderate). One (4.2%) patient died during follow-up, and one (4.2%) patient underwent a second MV reoperation. The left ventricular end-diastolic diameter was significantly reduced postoperatively compared to preoperatively [ (43.5±8.6) mm vs. (35.8±7.8)mm, P<0.001]. Conclusion The leaflet augmentation technique combined with a standardized repair strategy can achieve satisfactory short- to mid-term outcomes for the redo mitral repair after previous MV repair. It can be considered a safe and feasible technical option for cases with complex valvular lesions and severe pathological changes.

Objective To investigate the correlation between serum leptin level and body mass index(BMI)in in-fants with cyanosis congenital heart disease,and the relationship between leptin and Ob gene receptor(Ob-R)and hypoxia-inducible factor 1α(HIF-1α)in myocardium.Methods A total of 52 children under 6 months of age with congenital heart disease who underwent surgical treatment in the Department of Congenital Heart Surgery,Fuwai Hospital from January 2019 to October 2020 were included in this study.According to the arterial partial pressure of oxygen(PaO2)of 90 mmHg,they were divided into cyanotic group(n=30)and acyanotic group(n=22).Their height and weight were collected to calculate BMI.The serum leptin level was measured by ELISA.The ex-pressions of HIF-1α and Ob-R in myocardial tissue were detected by RT-PCR and Western blot.In animal mod-el,SD rats were divided into normoxia group and hypoxia intervention group,which were subjected to continuous hypoxia(10％ O2)for 4 weeks.The hypoxia intervention group received intraperitoneal injection of HIF-1α in-hibitor digoxin(2 mg/kg)daily from the 14 th to 21st day of hypoxia,respectively.The body weight of rats was recorded,and the expressions of HIF-1α and Ob-R were detected by RT-qPCR and Western blot.Results Com-pared with the acyanosis group,the cyanosis group had a significantly lower BMI(P<0.05)and a lower leptin/BMI ratio(leptin/BMI)(P<0.05).Spearman correlation analysis confirmed that serum leptin in the circulatory system was positively correlated with BMI(P<0.05).In the cyanosis group,the expression of Ob-R increased with the upregulation of HIF-1α,showing a positive correlation.In animal model,with the down-regulation of HIF-1α expression in digoxin injection,the Ob-R level was significantly lower than that in the control group(P<0.05),the trend of weight loss was significantly inhibited(P<0.05).The right ventricular hypertrophy in-dex was significantly lower than that in the control group(P<0.05).Conclusions HIF-1α regulates the expres-sion of Ob-R in myocardial tissue,and the mechanism of its association with leptin and Ob-R may help to find new therapeutic target for improving the prognosis of infants with congenital heart disease.

Artificial chord is a mature mitral valve repair technique, especially in adult mitral valve repair. It is still challenging to repair mitral valve in children with artificial chords because the quality of mitral valve is soft and immature. There are some differences in the methods of suture, the choice of suture size and the number of artificial chords. Although the artificial chords could not grow naturally, we found through the long-term research that most children did not have mitral valve restriction or even chords rupture due to itself can compensate through the growth of the flap and papillary muscle. This article summarizes the recent research progress on the treatment of mitral valve insufficiency in children with artificial chords, providing reference for clinical treatment.

Objectives:This study aims to investigate optimal surgical management strategies for pediatric patients diagnosed with volume-overloaded mitral regurgitation. Methods:A comprehensive retrospective analysis was conducted on a cohort of 110 pediatric patients who underwent primary mitral valve repair for volume-overloaded mitral regurgitation at Fuwai Hospital between April 2020 and March 2022.The cohort,with an average age of(14.5±15.1)months and 38.2%males,was divided into standardized group for patients receiving 3-step standardized mitral valvuloplasty(n=69)and annuloplasty group for patients undergoing annuloplasty only(n=41).After propensity score matching,a total of 38 pairs of patients were included,comparing the primary endpoint(functional mitral failure and postoperative heart failure)between the two groups. Results:Over a median follow-up of 26.3(19.8,32.9)months,and with a median echocardiographic follow-up of 11.9(7.5,14.8)months,no death was recorded.Among the cases,one patient(0.8%)necessitated unplanned reoperation;and seven patients(6.4%)experienced a recurrence of moderate-severe mitral regurgitation as observed through echocardiography beyond 6 months post-surgery.Additionally,nine patients developed heart failure at one month post-discharge.Above events were similar between the two groups.Following propensity score matching,patients in the standardized group demonstrated significantly longer cardiopulmonary bypass and aortic cross-clamp times compared to the annuloplasty group(both P＜0.05),other outcomes were similar between the two groups.Subgroup analysis based on age indicated that infants(＜1 year old)in the standardized group exhibited a significantly lower incidence of major endpoint events compared to the annuloplasty group.Additionally,postoperative echocardiography in annuloplasty group indicated that Z score of left ventricular end diastolic diameter was still greater than 2 during the latest follow-up. Conclusions:Patients with volume-overloaded mitral regurgitation in the standardized group exhibited comparable perioperative recovery and postoperative outcomes as in the annuloplasty group.For pediatric patients suffering from volume-overloaded mitral regurgitation,particularly those under one year of age,the standardized surgical approach exhibited reduced rates of heart failure and major endpoint events,and this strategy is more suitable for this patient group.

Total anomalous pulmonary venous connection (TAPVC) is a relatively rare but complex congenital heart disease characterized by the anomalous drainage of the pulmonary veins. Rather than connecting to the left atrium, the pulmonary veins drain either directly or indirectly into the systemic venous circulation or the right atrium via abnormal pathways. While there is broad consensus on the diagnostic criteria for TAPVC, significant debate persists regarding the optimal timing of surgical intervention, preferred surgical techniques, and postoperative management including re-intervention strategies. This article formulates a Chinese consensus based on evidence-based data from the literature and opinions from domestic experts, with the goal of further standardizing the surgical treatment of TAPVC in China.

The consensus was authored by National Society of Congenital Heart Diseases. After employing the Delphi process and incorporating literature reviews and expert discussions, seven recommendations were ultimately formulated. The consensus provides a detailed elaboration on the pathoanatomy, pathophysiology, clinical manifestations, diagnostic methods, and surgical treatment approaches for aortic valve diseases in children. It emphasizes that the treatment of aortic valve diseases in children should take into account the needs of growth and development, and recommends surgical strategies for different age groups and types of lesions, including valve plasty, Ross procedure, valve replacement, and balloon dilation. Specifically, aortic valve plasty is recommended for neonates and infants, while surgical options for older children are more diversified. The consensus only discusses isolated aortic valve disease and does not cover cases complicated with other heart malformations

[Purpose]To analyze the compliance rates and influencing factors for gastroscopy and colonoscopy in gastrointestinal cancer screening,and to provide evidence and management recom-mendations for gastrointestinal cancer screening programs.[Methods]The study was based on the joint gastrointestinal cancer screening program conducted in Fenghua District of Ningbo City in 2023.The target population underwent risk assessments,following which high-risk individuals were mobilized for gastroscopy and colonoscopy screenings.The x2 test was used to compare the positive rates of gastrointestinal cancer risk assessments and the compliance rates of clinical screenings among populations with different characteristics.Multivariable Logistic regression models were applied to analyze the factors associated with compliance rates of clinical gastroscopy and colonoscopy screenings among high-risk individuals.[Results]A total of 48 587 individuals were included in the analysis,with an average age of(63.95±7.48)years old.Among them,39.45％(19 166 individuals)were male.The positive rates of risk assessment for upper gastrointestinal cancer and colorectal cancer were 39.00％(18 949 individuals)and 16.02％(7 782 individuals),respectively.The compliance rates for gastroscopy and colonoscopy were 59.51％(11 227/18 949)and 50.85％(3 957/7 782),respectively.Multivariable Logistic regression analysis revealed that unmarried in-dividuals and those with an assessment interval of more than 14 d had lower compliance with gas-troscopy(both P＜0.05).High-risk individuals for upper gastrointestinal cancer with BMI≥24 kg/m2,gastrointestinal symptoms,history of upper gastrointestinal diseases,family history of cancers,history of gastroscopy,lower intake of fresh vegetables and fruits,and higher intake of processed meats had higher compliance with gastroscopy(all P＜0.05).High educational levels and an assess-ment interval of more than 14 d were associated with lower compliance with colonoscopy(both P＜0.05).Among individuals at high risk for colorectal cancer,those with gastrointestinal symptoms,history of upper gastrointestinal diseases,higher intake of processed meats and fried or grilled foods,and a positive risk assessment for upper gastrointestinal cancer had higher compliance with colonoscopy(all P＜0.05).[Conclusion]Participants in the joint gastrointestinal cancer screening program exhibit high compliance with both gastroscopy and colonoscopy.Compliance with gas-troscopy and colonoscopy is associated with individual lifestyle,health conditions,disease history,medical history,and family history of cancers.

Two pediatric heart failure patients were treated with pulmonary artery banding(PAB)at Fuwai Hospital,from December 2021 to January 2022.In the first case,an 8-month-old patient presented with left ventricular non-compaction cardiomyopathy(LVNC),left ventricular systolic dysfunction,ventricular septal defect,and atrial septal defect.The second case was a 4-month-old patient with LVNC,left ventricular systolic dysfunction,and coarctation of the aorta.After PAB,the left ventricular function and shape of both patients were significantly improved,without serious surgery-related complications.In these individual cases of pediatric heart failure,pulmonary artery banding exhibited a more satisfactory efficacy and safety compared to pharmacological treatment,especially for those with unsatisfactory medication results.Future clinical data are needed to promote the rational and broader application of this therapeutic option for indicated patients.

BACKGROUND: The purpose of this study was to investigate the specific effects of signal transducer and activator of transcription 4 (STAT4)-induced long intergenic nonprotein coding RNA 1278 (LINC01278) on the growth of non-small cell lung cancer (NSCLC) cells involved in the microRNA (miR)-877-5p/activated transcription factor 4 (ATF4) axis. METHODS: NSCLC tumor tissue and adjacent normal tissue were collected. Human normal lung epithelial cell BEAS-2B and human NSCLC cell lines (H1299, H1975, A549, H2228) were collected. The expression levels of STAT4, LINC01278, miR-877-5p, and ATF4 were detected. A549 cells were screened for subsequent experiments. The proliferation ability of cells was detected by colony formation experiment. Cell apoptosis was tested by flow cytometry. Scratch test and transwell assay were used to detect the migration and invasion ability of cells. Biological function of LINC01278 in NSCLC was confirmed by xenograft experiments. RESULTS: Low expression miR-877-5p and high expression of STAT4, LINC01278 and ATF4 were detected in NSCLC.Silenced LINC01278 in A549 cell depressed cell proliferation, migration and invasion, but facilitated cell apoptosis.LINC01278 was positively correlated with STAT4 and could directly bind to miR-877-5p. Upregulating miR-877-5p suppressed NSCLC cell progression, while downregulating miR-877-5p had the opposite effect. Upregulating miR-877-5p abrogated the effects of silenced LINC01278 on NSCLC cell progression. MiR-877-5p targeted ATF4. ATF4 upregulation could partly restore the carcinogenic effect of LINC01278 in vitro and in vivo. CONCLUSION Our data supports that STAT4-induced upregulation of LINC01278 promotes NSCLC progression by modulating the miR-877-5p/ATF4 axis, suggesting a novel direction for NSCLC treatment.