Background Pulmonary arterial hypertension (PAH) is a progressive condition with a poorprognosis in children. Lung transplantation (Ltx) remains the ultimate option when patients are refractory toPAH-speciffc therapy....Background Pulmonary arterial hypertension (PAH) is a progressive condition with a poorprognosis in children. Lung transplantation (Ltx) remains the ultimate option when patients are refractory toPAH-speciffc therapy. Reverse Potts shunt (RPS) has been introduced to treat suprasystemic PAH. This studyaims to investigate the clinical outcomes of suprasystemic PAH in children. Methods Embase, Pubmed,and the Cochrane Library databases were searched for related studies that reported the clinical outcomes ofsuprasystemic PAH following RPS in children. To investigate the clinical outcomes of RPS, meta-analyses ofthe early and overall mortalities were performed. Results Nine studies were included in this study. Theestimated early mortality was 14.4% (95% CI, 7.1% to 23.1%), and the overall mortality/Ltx was 23.2% (95%CI, 14.4% to 32.9%). The estimated 1-year survival was 86.3% (95% CI, 75.9% to 88.7%). A qualitative reviewshowed that the median value of 5-year survival free from Ltx of patients undergoing RPS was 68.6% (range:65% to 92.3%). Compared to Ltx, RPS did not signiffcantly increase the early mortality (OR, 2.48, 95% CI0.75 to 8.24, p = 0.14). RPS also signiffcantly improved the New York Heart Association/World HealthOrganization functional class, reduced the BNP/NT-pro BNP levels, decreased the PAH-speciffc therapy,and increased the six-minute-walking distance. Conclusions RPS may serve as an alternative treatmentfor suprasystemic drug-refractory PAH. Further large-scale and prospective cohort studies are needed tovalidate these ffndings.展开更多
To the Editor:Owing to the heterogeneity of congenital heart disease-associated pulmonary hypertension(CHDPH)disease and the development of the pulmonary vascular system in pediatric patients,the management of CHD-PH ...To the Editor:Owing to the heterogeneity of congenital heart disease-associated pulmonary hypertension(CHDPH)disease and the development of the pulmonary vascular system in pediatric patients,the management of CHD-PH in children is signicantly different from that in adults.[1]Early identication and intervention of CHD-PH are of great signicance for disease management in children with CHD,and non-invasive,accurate clinical tools are urgently needed to predict PH risk in children with CHD.However,the applicability of the current risk-scoring system to pediatric CHD-PH remains unclear.Furthermore,there are presently no user-friendly,non-invasive online tools available for predicting CHD-PH,and there is a scarcity of related studies in Asian populations.This study aimed to develop and validate the prediction tools for estimating the risk of pulmonary hypertension and clinical deterioration in children with congenital heart disease.展开更多
The second edition of the expert consensus on pediatric nutrition was formed based on a global update of pedia-tric nutrition guidelines or consensus worldwide,the management of congenital heart disease,and the result...The second edition of the expert consensus on pediatric nutrition was formed based on a global update of pedia-tric nutrition guidelines or consensus worldwide,the management of congenital heart disease,and the results of multi-center clinical nutrition research for congenital heart disease following thefirst Chinese consensus edition of 2016.The consensus was also shaped by the results of three discussion sessions and two questionnaires con-ducted by the 13-member collaboration group.This process was informed by both clinical guidelines and expert consensus.The quality of literature,both in English and Chinese,and the level of recommendations were evaluated using the Grading of Recommendations Assessment,Development,and Evaluations(GRADE)system.展开更多
Tetralogy of Fallot(TOF)is a congenital heart disease characterized by abnormal cardiomyocyte differentiation in the right ventricular outflow tract(RVOT),and HA117 is a novel long noncoding RNA(lncRNA)with anti-diffe...Tetralogy of Fallot(TOF)is a congenital heart disease characterized by abnormal cardiomyocyte differentiation in the right ventricular outflow tract(RVOT),and HA117 is a novel long noncoding RNA(lncRNA)with anti-differentiation roles.To investigate the potential association of HA117 with TOF,we collected 84 RVOT tissues from patients with TOF.We determined the expression of HA117 in RVOT samples from TOF patients and collected clinical data to conduct a cross-sectional and short-term follow-up study.McGoon ratio,Nakata index,and left ventricular end-diastolic volume index(LVEDVI)were negatively correlated with the expression of HA117 based on subgroup analysis,correlation analysis and logistic regression analysis.Additionally,cardiopulmonary bypass(CPB)time and ICU stay were longer in patients with higher expression of HA117 than in patients with lower expression of HA117.Furthermore,percentage improvement in SPO2 was significantly reduced in patients with increased HA117 expression at 6 months after surgery.Our results suggested that the increased expression of the novel lncRNA HA117 is a risk factor for unfavorable McGoon ratio,Nakata index and LVEDVI in TOF patients.Additionally,an increased expression of HA117 might lead to adverse short-term outcomes in TOF patients.展开更多
基金Chongqing Medical University Program for Youth Innovation in Future Medicine(W0204)Natural Science Foundation Project of Chongqing,Chongqing Science and Technology Commission(CSTB2023NSCQ-BHX0010)+1 种基金Chongqing Postdoctoral Research Project Special Support(2023CQBSHTB3074)Science and Technology Research Program of Chongqing Municipal Education Commission(KJQN202400421).
文摘Background Pulmonary arterial hypertension (PAH) is a progressive condition with a poorprognosis in children. Lung transplantation (Ltx) remains the ultimate option when patients are refractory toPAH-speciffc therapy. Reverse Potts shunt (RPS) has been introduced to treat suprasystemic PAH. This studyaims to investigate the clinical outcomes of suprasystemic PAH in children. Methods Embase, Pubmed,and the Cochrane Library databases were searched for related studies that reported the clinical outcomes ofsuprasystemic PAH following RPS in children. To investigate the clinical outcomes of RPS, meta-analyses ofthe early and overall mortalities were performed. Results Nine studies were included in this study. Theestimated early mortality was 14.4% (95% CI, 7.1% to 23.1%), and the overall mortality/Ltx was 23.2% (95%CI, 14.4% to 32.9%). The estimated 1-year survival was 86.3% (95% CI, 75.9% to 88.7%). A qualitative reviewshowed that the median value of 5-year survival free from Ltx of patients undergoing RPS was 68.6% (range:65% to 92.3%). Compared to Ltx, RPS did not signiffcantly increase the early mortality (OR, 2.48, 95% CI0.75 to 8.24, p = 0.14). RPS also signiffcantly improved the New York Heart Association/World HealthOrganization functional class, reduced the BNP/NT-pro BNP levels, decreased the PAH-speciffc therapy,and increased the six-minute-walking distance. Conclusions RPS may serve as an alternative treatmentfor suprasystemic drug-refractory PAH. Further large-scale and prospective cohort studies are needed tovalidate these ffndings.
基金National Natural Science Foundation of China(Nos.82000034,82120108001,and 82170069)Chongqing Postdoctoral International Exchange Training Program(No.2021JLPY001)+2 种基金National Clinical Medical Research Center(No.NCRC-2022-GP-08)Guangdong Department of Science and Technology(No.2022A0505030017)Basic Science and Application of Guangzhou Science and Technology Plan(No.202201010069)
文摘To the Editor:Owing to the heterogeneity of congenital heart disease-associated pulmonary hypertension(CHDPH)disease and the development of the pulmonary vascular system in pediatric patients,the management of CHD-PH in children is signicantly different from that in adults.[1]Early identication and intervention of CHD-PH are of great signicance for disease management in children with CHD,and non-invasive,accurate clinical tools are urgently needed to predict PH risk in children with CHD.However,the applicability of the current risk-scoring system to pediatric CHD-PH remains unclear.Furthermore,there are presently no user-friendly,non-invasive online tools available for predicting CHD-PH,and there is a scarcity of related studies in Asian populations.This study aimed to develop and validate the prediction tools for estimating the risk of pulmonary hypertension and clinical deterioration in children with congenital heart disease.
基金supported by the National Natural Science Foundation of China(81970265,82270310)a Sub-Project of the National Key R&D Program“The recognition and Identification of Genetic Pathogenic Genes for Structural Birth Defects”(2021YFC2701002)+1 种基金Nanjing Science and Technology Development Project(2019060007)Jiangsu Provincial Key Research and Development Program(BE2023662).
文摘The second edition of the expert consensus on pediatric nutrition was formed based on a global update of pedia-tric nutrition guidelines or consensus worldwide,the management of congenital heart disease,and the results of multi-center clinical nutrition research for congenital heart disease following thefirst Chinese consensus edition of 2016.The consensus was also shaped by the results of three discussion sessions and two questionnaires con-ducted by the 13-member collaboration group.This process was informed by both clinical guidelines and expert consensus.The quality of literature,both in English and Chinese,and the level of recommendations were evaluated using the Grading of Recommendations Assessment,Development,and Evaluations(GRADE)system.
基金The study was funded by the National Natural Science Foundation of China(http://www.nsfc.gov.cn/)(NSFC 81370474 and NSFC 81400577).
文摘Tetralogy of Fallot(TOF)is a congenital heart disease characterized by abnormal cardiomyocyte differentiation in the right ventricular outflow tract(RVOT),and HA117 is a novel long noncoding RNA(lncRNA)with anti-differentiation roles.To investigate the potential association of HA117 with TOF,we collected 84 RVOT tissues from patients with TOF.We determined the expression of HA117 in RVOT samples from TOF patients and collected clinical data to conduct a cross-sectional and short-term follow-up study.McGoon ratio,Nakata index,and left ventricular end-diastolic volume index(LVEDVI)were negatively correlated with the expression of HA117 based on subgroup analysis,correlation analysis and logistic regression analysis.Additionally,cardiopulmonary bypass(CPB)time and ICU stay were longer in patients with higher expression of HA117 than in patients with lower expression of HA117.Furthermore,percentage improvement in SPO2 was significantly reduced in patients with increased HA117 expression at 6 months after surgery.Our results suggested that the increased expression of the novel lncRNA HA117 is a risk factor for unfavorable McGoon ratio,Nakata index and LVEDVI in TOF patients.Additionally,an increased expression of HA117 might lead to adverse short-term outcomes in TOF patients.
