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 summarize and explore the individualized surgical treatment strategy and prognosis of anomalous aortic origin of coronary artery (AAOCA). Methods The clinical data of children with AAOCA admitted to Shanghai Children's Medical Center from March 2018 to August 2021 were retrospectively analyzed. Results A total of 17 children were enrolled, including 13 males and 4 females, with a median age of 88 (44, 138) months and a median weight of 25 (18, 29) kg. All patients received operations. The methods of coronary artery management included coronary artery decapitation in 9 patients, coronary artery transplantation in 5 patients and coronary artery perforation in 3 patients. One patient with severe cardiac insufficiency (left ventricular ejection fraction 15%) received mechanical circulatory assistance after the operation for 12 days. No death occurred in the early postoperative period, the average ICU stay time was 4.3±3.0 d, and the total hospital stay was 14.4±6.1 d. All the children received regular anticoagulation therapy for 3 months after discharge. The median follow-up time was 15 (13, 24) months. All patients received regular anticoagulation therapy for 3 months after discharge. No clinical symptoms such as chest pain and syncope occurred again. The cardiac function grade was significantly improved compared with that before operation. Imaging examination showed that the coronary artery blood flow on the operation side was unobstructed, and no restenosis occurred. Conclusion AAOCA is easy to induce myocardial ischemia and even sudden cardiac death. Once diagnosed, operation should be carried out as soon as possible. According to the anatomic characteristics of coronary artery, the early effect of individualized surgery is satisfactory, and the symptoms of the children are significantly improved and the cardiac function recovers well in the mid-term follow-up.

Objective:To analyze the coronary angiographic (CAG) characteristics of coronary artery lesion (CAL) in children with Kawasaki disease (KD), and to clarify the necessity of CAG in the diagnosis and treatment of KD combined with CAL in children.Methods:It was a retrospective study to analyze the clinical data, electrocardiogram, echocardiography, time and findings of CAG in children with KD and CAL who underwent CAG in Shanghai Children′s Medical Center of Shanghai Jiao Tong University School of Medicine from January 2013 to August 2022.The distribution, type, severity, and prognosis of CAL were analyzed.Results:A total of 117 children with KD and CAL were included in the analysis.The onset age of KD was from 2 months to 12.8 years old, and the age of performing CAG was from 8 months to 18.1 years old.A total of 234 coronary artery lesions were detected in 117 cases.Among them, CAL in the right coronary artery (RCA), left anterior descending branch (LAD), left main coronary artery and left circumflex artery were detected in 96 branches(41.1%), 78 branches(33.3%), 44 branches(18.8%), and 16 branches(6.8%), respectively.Unilateral coronary artery involvement was detected in 43 cases (36.8%), of which LAD was the dominant; while bilateral involvement was detected in 74 cases (63.2%), among which, LAD and RCA were the most involved arteries.Stratified by the degree of coronary involvement, large coronary aneurysms and severe coronary stenosis were most frequently occurred in the RCA and LAD.In contrast, 10 cases (13.6%), 20 cases (24.3%), 55 cases (45.8%) and 37 cases (67.3%) of intraluminal lesions were found in small, medium and large coronary aneurysms, and stenosis or occlusion, respectively.The incidence of intraluminal lesions tended to be higher in the site of severe lesions.CAG showed stenosis or occlusion in a total of 55 cases, and collateral circulation at varying degrees was found in cases of severe stenosis or occlusion.Conclusions:CAL in children with KD are complex and varied.Although clinical symptoms, routine electrocardiogram and cardiac ultrasound may indicate severe CAL.Their applications are limited by the diagnosis of the type (especially stenosis), degree, and extent of CAL, as well as the detection of extracoronary lesions.CAG is of great significance to identify vascular lesions and guide clinical management of KD combined with CAL in children.

Aging poses a major risk factor for cardiovascular diseases, the leading cause of death in the aged population. However, the cell type-specific changes underlying cardiac aging are far from being clear. Here, we performed single-nucleus RNA-sequencing analysis of left ventricles from young and aged cynomolgus monkeys to define cell composition changes and transcriptomic alterations across different cell types associated with age. We found that aged cardiomyocytes underwent a dramatic loss in cell numbers and profound fluctuations in transcriptional profiles. Via transcription regulatory network analysis, we identified FOXP1, a core transcription factor in organ development, as a key downregulated factor in aged cardiomyocytes, concomitant with the dysregulation of FOXP1 target genes associated with heart function and cardiac diseases. Consistently, the deficiency of FOXP1 led to hypertrophic and senescent phenotypes in human embryonic stem cell-derived cardiomyocytes. Altogether, our findings depict the cellular and molecular landscape of ventricular aging at the single-cell resolution, and identify drivers for primate cardiac aging and potential targets for intervention against cardiac aging and associated diseases.
Aged ; Animals ; Humans ; Aging/genetics* ; Forkhead Transcription Factors/metabolism* ; Myocytes, Cardiac/metabolism* ; Primates/metabolism* ; Repressor Proteins/metabolism* ; Transcriptome ; Macaca fascicularis/metabolism*

Objective    To compare and investigate the efficacy and differences of modified B-T shunt, central shunt and right ventricle-pulmonary artery (RV-PA) connection in the treatment of pulmonary atresia with ventricular septal defect (PA/VSD). Methods    A total of 124 children with PA/VSD underwent initial palliative repair in Shanghai Children's Medical Center from September 2014 to August 2019, including 63 males and 61 females, aged 7 days to 15 years. They were divided into in a modified B-T shunt group (55 patients), a central shunt group (22 patients) and a RV-PA connection group (47 patients). The clinical data of these children were retrospectively analyzed. Results    There were 9 early deaths after palliation, with an early mortality rate of 7.3%. The mean follow-up time was 26.5±20.3 months, with 5 patients lost to follow-up, 5 deaths during the follow-up period, and 105 survivors. The 1-year and 5-year survival rates were both 89.7%. The monthly increased Nakata index was 5.2 (–0.2, 12.3) mm2/m2, 9.2 (0.1, 23.6) mm2/m2, 6.3 (1.8, 23.3) mm2/m2 in the modified B-T shunt group, the central shunt group, and the RV-PA connection group, respectively, with no statistical difference among the three groups. The 1-year survival rate was 85.3%, 78.4%, 95.2%, and the 5-year (4-year in the central shunt group) survival rate was 85.3%, 58.8%, 95.2% in the three groups, respectively, with a statistical difference among them (P<0.05). The complete repair rate was 36.5%, 19.0% and 67.4% in the three groups, respectively, with a statistical difference among the three groups (P<0.001). Conclusion    All these three palliative surgical approaches can effectively promote pulmonary vascular development. But compared with systemic-pulmonary shunt, RV-PA connection has a lower perioperative mortality rate and can achieve a higher complete repair rate at a later stage, which is beneficial for long-term prognosis.

Objective:To optimize the strategy of Nuss procedure with open approach via median sternotomy for pectus excavatum(PE) with congenital heart disease(CHD).Methods:25 PE patients with CHD treated in our center from January 2017 to November 2021 were reviewed and divided into two groups. The CHD surgery and open Nuss procedure with median sternotomy were performed concomitantly in 9 cases(group A), whose height of(121.7±28.0)cm, weight of(22.2±14.0)kg, age of(7.65±4.08) years old, and Haller index of 3.99±1.37. 16 cases underwent open Nuss procedure via median re-sternotomy after congenital heart surgery(group B), whose height of(130.9±27.2)cm, weight of(26.5±14.3)kg, age of(8.82±4.09) years old, and Haller index of 4.18 ± 0.97. The cardiac anatomy, cardiac function and the severity of PE of all patients were evaluated by echocardiography and computed tomography preoperatively. The preoperative gender, appearance of PE, types of associated CHD, retrosternal adhesion and the interval between open Nuss procedure and CHD surgery were collected. The details of operation, hospital stay, intraoperative adverse events, postoperative complications and follow-up were collected.Results:All the operations were accomplished successfully in 25 children. No intraoperative complications occurred. One had a postoperative wound infection in group A. The operation time of group A was significantly longer than that of group B[(3.78±1.54) h vs.(2.19±0.94) h, P<0.05]. There was no significant difference in the mechanical ventilation time between the two groups[(22.50±45.64) h vs.(4.18±1.41) h, P=0.263]. The ICU stay of group A was significantly longer than that of group B[(4.00±6.42) days vs.(1.13±0.34) days, P<0.05]. There was no significant difference in the length of hospital stay between the two groups[(16.00±15.18) days vs.(9.19±2.31) days, P=0.419]. The Nuss bar was removed in 14 cases of the two groups, with a interval of(27.9±11.25) months after open Nuss procedure, the postoperative Haller Index was 2.48±0.49. There was no difference of LVEF in postoperative and preoperative echocardiography(0.663±0.028 vs. 0.659±0.038, P=0.533). The FVC and FEV1 were significantly improved compared with before operation[(87.2±3.9)% vs.(84.1±8.2)%]. The outcome was good. Conclusion:For PE patients with CHD, one-stage or staged individualized Nuss procedure with open approach via median sternotomy can be performed after careful preoperative evaluation. A skillful median re-sternotomy and widely dissecting retrosternal adhesion between sternum and anterior wall of heart are necessary to avoid serious intraoperative complications such as massive heart bleeding and ensure good sternal elevation.

With the research and summary of the cases, the surgical indications, surgical procedures, and reference values for material selection for the surgical treatment of neonatal complex congenital heart disease are further refined, and the diagnosis and treatment system is further standardized and completed. In order to increase the success rate of surgery, reduce postoperative mortality and postoperative complications, many researchers have proposed improved surgical procedures, some of which have become mature technologies. In addition, in recent years, the surgical age of newborns has gradually advanced and the surgical weight has gradually decreased. In particular, the increasing application of Hybrid therapy has enabled some critically ill, premature, and low birth weight children to survive smoothly to accept surgical correction. This article will review the recent advances in the surgical treatment of neonatal complicated congenital heart disease.

Tetralogy of Fallot (TOF) is one the most common cyanotic congenital heart diseases. With the advancement of surgical techniques, most patients can achieve long term survival. However, they often encounter right ventricular dysfunction in the late course. Echocardiogram, one of the major techniques used in the postoperative follow-up, is assuming a growing role in the assessment of right ventricular function. Hereby, we reviewed the echocardiographic indices for right ventricular systolic function commonly used in the follow-up of TOF patients, so as to provide new insights into their treatment.

Objective:To study the surgical results of Yasui procedure for interruption of aortic arch and ventricular septal defect(IAA/VSD) associated with aortic stenosis(AS) or aortic atresia(AA).Methods:A retrospective study was performed on all 4 children with IAA/VSD/AS(AA) that underwent a Yasui procedure from Dec 2014 to Dec 2019. Three patients had AS, and 1 patient had AA. The age was from 10 days to 25 months and the weight was from 2.7 kg to 10 kg. The type of IAA was type A in 1 and type B in 3. The diameter and Z value of the aortic annulus were 1.9-4.3 mm and -6.7--3.6. The diameter and Z value of the ascending aorta were 2.6-5.8 mm and -5.4--2.6. The mean Z value of LVEDD and LVEDS was 3.6±2.6 and 3.6±2.9 resparately.Results:All 4 patients survived after the Yasui procedure. Three patients underwent primary repair and 1 patient underwent staged repair. CPB time was 128-283 min and aortic cross-clamping time was 98-171 min. Ventilation time was 93-296 h, and ICU and hospital length of stay was 7-25 days and 18-39 days. Follow-up was complete in all patients from 8 to 92 months, and there was no late death. All the patients were in good biventricular function with NYHA grade Ⅰ in 3 and NYHA grade Ⅱ in 2 patients. No residual left ventricular outflow tract obstruction was detected.Conclusion:The Yasui procedure can be an option for patients with IAA/VSD/AS(AA) and good developed left ventricle and can achieve good early surgical results.

Objective:To explore and summarize the surgical strategies of biventricular conversion from single-ventricle palliation for the complex congenital heart defects in this study.Methods:From April 2017 to December 2019, a total of 14 cases with complex complex congenital heart defects who had planned to undergo single-ventricle palliation were finally accepted biventricular conversion. According to the development and anatomical characteristics of the ventricles, they were divided into two groups: ventricular well development group(9 cases), all of which showed complex intracardiac defects, and the ventricles well developed, including 4 cases of double outlet right ventricle with remote ventricular septal defect, 2 cases of transposition of great arteries combined with ventricular septal defect and left ventricular outflow tract obstruction, one case of congenitally corrected transposition of the great arteries combined with double outlet right ventricle and interrupted aortic arch, one case of double outlet right ventricle with subpulmonary ventricular septal defect and atrioventricular connection inconsistency, one case of pulmonary atresia with ventricular septal defect. Ventricular unbalanced development group(5 cases), all of which were functional single ventricle, including 3 cases of heterotaxy and 2 cases of unbalanced complete atrioventricular septal defect. The operation time, postoperative complications, death, cardiac function and reoperation of the two groups were compared.Results:There was one early death, with a mortality rate of 7.1%. In the ventricular well development group, the average waiting time for biventricular conversion was(56.4 ± 38.9) months. One case died after the operation. The early complications included 2 case of arrhythmias, 3 cases of pleural effusions and 2 cases of pulmonary infections. In the ventricular unbalanced development group, the average waiting time for biventricular conversion was(20.4 ± 15.8) months, and the average of left ventricular end-diastolic volume was(33.6 ± 2.1) ml/m 2. The early complications included 2 cases of low cardiac outputs, one case of arrhythmia and one case of renal failure. There was no significant difference in operation time, intubation time and ICU time between the two groups. The patients were followed up for 4 months to 3 years, and there was no death. In the ventricular well development group, one case was reoperated due to the obstruction of the anastomosis between the superior vena cava and the right atrial appendage. In the ventricular unbalanced development group, one case was implanted with a permanent pacemaker due to the degree Ⅲ atrioventricular block. The NYHA grade of the whole group was Ⅰ-Ⅱ. Conclusion:Whether the complex congenital heart defects with the ventricles well developed or unbalanced, the comprehensive evaluation before the planned single-ventricle palliation will be helpful to find the appropriate cases for biventricular conversion. According to its anatomical characteristics performed the corresponding biventricular conversion can obtain a satisfied clinical outcomes, although the long-term results still need further follow-up.