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Risk Stratification and Prognosis of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease
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作者 Wenjie Dong Zhibin Hong +6 位作者 Aqian Wang Kaiyu Jiang Hai Zhu Fu zhang Zhaoxia Guo Hongling Su Yunshan Cao 《Congenital Heart Disease》 SCIE 2024年第3期325-339,共15页
Background:Current guidelines for managing pulmonary arterial hypertension(PAH)recommend a risk strati-fication approach.However,the applicability and accuracy of these strategies for PAH associated with congenital he... Background:Current guidelines for managing pulmonary arterial hypertension(PAH)recommend a risk strati-fication approach.However,the applicability and accuracy of these strategies for PAH associated with congenital heart disease(PAH-CHD)require further validation.This study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year follow-up.Additionally,new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD patients.Methods:This retrospective study included 126 PAH-CHD patients.Clinical and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s exact tests.Indepen-dent risk factors were identified using ordered logistic regression,while Kaplan-Meier and Cox proportional hazards regression analyses evaluated their impact on all-cause mortality.A new stratification model for the PAH-CHD population was constructed based on these analyses.Results:Significant survival differences across stratified risk groups were observed(p<0.001),validating the effectiveness of the simplified risk stratification method in PAH-CHD patients.Prothrombin activity was a strong independent predictor of adverse outcomes of PAH-CHD patients(Hazard ratio 0.95,p<0.001,C-index 0.70).A model combining N-terminal pro-brain natriuretic peptide,prothrombin activity,albumin,and right atrial area achieved an area under the curve of 0.89 and a C-index of 0.85.Conclusions:The simplified risk stratification method is applicable to PAH-CHD patients.Prothrombin activity is a strong independent predictor of adverse outcomes.A comprehensive risk stratification approach,incorporating both established and novel biomarkers,enhances accessibility and offers predictive efficacy during follow-up for PAH-CHD patients,comparable to established models. 展开更多
关键词 pulmonary hypertension pulmonary arterial hypertension congenital heart disease risk stratification
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The Potential of Circular RNAs as Biomarkers in Pulmonary Arterial Hypertension Related to Congenital Heart Disease
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作者 Fajri Marindra Siregar Sofia Mubarika Haryana +2 位作者 Dyah Wulan Anggrahini Lucia Kris Dinarti Anggoro Budi Hartopo 《Congenital Heart Disease》 SCIE 2024年第4期375-388,共14页
A particular type of endogenous noncoding RNAs known as circular RNAs(circRNAs)has now become possible biomarkers for several diseases because of their stability and tissue-specific expression patterns.CircRNAs might p... A particular type of endogenous noncoding RNAs known as circular RNAs(circRNAs)has now become possible biomarkers for several diseases because of their stability and tissue-specific expression patterns.CircRNAs might play a role in various of biological processes.The identification of particular circRNAs dysregulated in pulmonary arterial hypertension(PAH)raises the possibility of these molecules serving as biomarkers for the disease’s early diagnosis and treatment.This review mainly summarizes the role and potential of circRNA as a future biomarker in PAH related to congenital heart disease.This study presented several potential circRNA targets as diagnostic biomarkers for PAH,discussed their biological functions,and addressed the challenges that need to be considered for their application in clinical settings. 展开更多
关键词 BIOMARKER circular RNA congenital heart disease pulmonary arterial hypertension
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Independent prognostic value of lipocalin-2 in congenital heart disease-associated pulmonary artery hypertension
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作者 Zhang-Ke Guo Ping-Gui Chen +6 位作者 Yao-Xuan Li Hong Jiao Xiao-Hui Kong Song Bai Xiao-Feng Li Ai-Jun Liu Guo-Liang Wang 《World Journal of Cardiology》 2024年第12期720-730,共11页
BACKGROUND Timely and accurate evaluation of the patient's pulmonary arterial pressure(PAP)is of great significance for the treatment of congenital heart disease.Currently,there is no non-invasive gold standard me... BACKGROUND Timely and accurate evaluation of the patient's pulmonary arterial pressure(PAP)is of great significance for the treatment of congenital heart disease.Currently,there is no non-invasive gold standard method for evaluating PAP.AIM To assess the prognostic value of lipocalin-2(LCN2)in relation to PAP in patients with congenital heart disease associated with pulmonary artery hypertension.METHODS We conducted a retrospective analysis of 69 pediatric patients diagnosed with ventricular septal defects.The patients’clinical and laboratory data were collected.The serum LCN2 concentrations were compared between the pulmonary arterial hypertension(PAH)group and the nonPAH group.The correlation of LCN2 concentration with PAH classification was evaluated using binary logistic regression analysis.The receiver operating characteristic(ROC)curve was used to evaluate the diagnostic potential of LCN2 for PAH.RESULTS Serum LCN2 concentration significantly correlated with patients’mean PAP(r=0.544,P<0.001),but not correlated with creatinine(P=0.446)or blood urea nitrogen(P=0.747).LCN2 levels were significantly correlated with PAH in both univariate[odds ratio(OR)1.107,95%CI:1.033-1.185,P=0.004)]and multivariate regression analysis(OR 1.150,95%CI:1.027-1.288,P=0.015).ROC curve analysis revealed an area under the curve of 0.783 for LCN2.At the cutoff value of 19.42 ng/mL,the sensitivity and specificity of LCN2 for diagnosing PAH is 90.19%and 55.56%,respectively.LCN2 concentration also significantly correlated with the post-repair mean PAP in patients with congenital heart disease(r=0.532,P=0.009).CONCLUSION LCN2 is emerging as a candidate biomarker for assessing PAP in patients with congenital heart disease.Its high sensitivity in diagnosing PAH makes it a valuable tool in patient management. 展开更多
关键词 congenital heart disease pulmonary arterial hypertension Lipocalin-2 ENDOTHELIN-1 BIOMARKER
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Transgelin as a potential target in the reversibility of pulmonary arterial hypertension second to congenital heart disease 被引量:8
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作者 Li Huang Li Li +6 位作者 Tao Yang Wen Li Li Song Xianmin Meng Qing Gu Changming Xiong Jianguo He 《中国循环杂志》 CSCD 北大核心 2018年第S01期165-166,共2页
Background The reversibility of pulmonary arterial hypertension(PAH)in congenital heart disease(CHD)is of great importance for the operability of CHD.Proteomics analysis found that transgelin was significantly upregul... Background The reversibility of pulmonary arterial hypertension(PAH)in congenital heart disease(CHD)is of great importance for the operability of CHD.Proteomics analysis found that transgelin was significantly upregulated in the lung tissue of CHD-PAH patients,especially in the irreversible group.However,how exactly it participated in CHD-PAH development is unknown. 展开更多
关键词 pulmonary arterial hypertension congenital heart disease CHD-PAH
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Correlation between endothelia cells activation and imbalance of cytokines in pulmonary hypertension of congenital heart disease 被引量:1
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作者 师桃 吕毅 +1 位作者 耿希刚 李兆志 《Journal of Pharmaceutical Analysis》 SCIE CAS 2007年第2期208-211,共4页
Objective To explore the correlation between endothelia cells activation and cytokines (ET-1, NO) levels in patients with pulmonary hypertension (PH), and to discuss their roles in the development of PH. Methods Twent... Objective To explore the correlation between endothelia cells activation and cytokines (ET-1, NO) levels in patients with pulmonary hypertension (PH), and to discuss their roles in the development of PH. Methods Twenty patients with simple ventricular septal defect (VSD) were chosen as controls, and 30 patients with PH were studied. Plasma levels of ET-1 and NO were measured by radioimmunoassay or colorimetric method. Before cardiopulmonary bypass was established, the specimens from right lung were fixed with formaldehyde solution, embedded with paraffin and stained by SP immunohistochemistry. Intercellular adhesion molecule-1 (ICAM-1) expression was measured through the determination of the light density with computer imaging technology. Results Compared with that of the patients with simple VSD, the light density of ICAM-1 and plasma level of ET-1 increased in patients with PH; but plasma level of NO decreased (P<0.05). Positive correlation was observed between ICAM-1 and ET-1/NO (P<0.05). Conclusion Endothelia cells activation and imbalance of ET-1/NO might play an important role in the development of PH. 展开更多
关键词 congenital heart disease (CHD) pulmonary hypertension (PH) endothelia cells activation intercellular adhesion molecule-1 (ICAM-1) nitric oxide (NO) endothlin-1 (ET-1)
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Pulmonary Arterial Hypertension Medical Management of the Adult Patient with Congenital Heart Disease 被引量:1
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作者 Ali Ataya Julian Chung +1 位作者 Jessica Cope Hassan Alnuaimat 《Cardiovascular Innovations and Applications》 2018年第B05期1-8,共8页
Congenital heart disease(CHD)-associated pulmonary arterial hypertension(PAH)includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation.CHD-associated PAH has an estim... Congenital heart disease(CHD)-associated pulmonary arterial hypertension(PAH)includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation.CHD-associated PAH has an estimated prevalence of 5– 10% in adult patients,with an increasing number of patients surviving to adulthood because of advances in the surgical management and the development of pulmonary arterial hypertension(PAH)-targeted pharmacotherapy.Although limited data exist,targeted PAH pharmacotherapy has proven to be benefi cial in patients with CHD-associated PAH,with observed improvement in functional class,increase in exercise capacity,and improvement in quality of life and cardiopulmonary hemodynamics.Additionally,there has been increasing interest in the“treat-to-close”strategy.PAH-targeted pharmacotherapy may be used to optimize cardiopulmonary hemodynamics so as to improve patients’operability in repairing the cardiac defect.Although there have been signifi cant advances in the management of this disease state in the past 2 decades,mortality remains high,and ongoing clinical trials are needed to better understand the treat-to-close strategy. 展开更多
关键词 pulmonary ARTERIAL hypertension congenital heart disease EISENMENGER syndrome ARTERIAL SEPTAL DEFECT ventricular SEPTAL DEFECT
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Selexipag as Add-on Therapy for Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease:A Single-Center Retrospective Study 被引量:1
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作者 Se Yong Jung Doyoung Jung +4 位作者 Ah Young Kim Jae Hee Seol Jung Min Park Jo Won Jung Jae Young Choi 《Congenital Heart Disease》 SCIE 2021年第3期233-244,共12页
Purpose:This study examined the efficacy and safety of selexipag in treating pulmonary arterial hypertension(PAH)associated with congenital heart disease(CHD).Materials and Methods:We conducted a retrospective study o... Purpose:This study examined the efficacy and safety of selexipag in treating pulmonary arterial hypertension(PAH)associated with congenital heart disease(CHD).Materials and Methods:We conducted a retrospective study of patients with CHD-associated PAH,treated with selexipag since December 2017.Thirteen adult patients(mean age,45.4 years;women,77%)were treated with selexipag as add-on therapy.Baseline characteristics,World Health Organization functional class,6-minute walking distance(6MWD)test results,N-terminal pro-B-type natriuretic peptide levels,echocardiographic data,and incidence of side effects were assessed.Results:The majority of patients(12/13,92.3%)experienced more than one treatment-associated complication;one patient dropped out of the study due to intolerable myalgia.The results of 6MWD test(from 299.2±56.2 m to 363.8±86.5 m,p=0.039)and tricuspid regurgitation(TR)pressure gradient(from 84.7±20.5 mmHg to 61.6±24.0 mmHg,p=0.018)improved and remained improved after selexipag treatment in 12 patients.Based on the results of a non-invasive risk assessment,8(66.7%)patients showed improvement,3(25.0%)showed no interval change,and the status of one patient(8.3%)deteriorated.Moreover,compared to patients treated with a low dosage,patients treated with a medium-to-high dosage showed a greater increase in 6MWD results(88.3±26.4 m vs.55.3±27.6 m,p=0.043)and a greater reduction in the TR pressure gradient(-33.7±10.9 mmHg vs.-12.5±12.0 mmHg,p=0.015).Conclusion:Selexipag is an efficient pulmonary vasodilator as add-on therapy in treating CHD-associated PAH. 展开更多
关键词 Selexipag congenital heart disease pulmonary arterial hypertension
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NOTCH3 Mutations and CADASIL Phenotype in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease 被引量:1
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作者 Rui Jiang Kaisheng Lai +4 位作者 Jianping Xu Xiang Feng Shaoye Wang Xiaojian Wang Zhe Liu 《Congenital Heart Disease》 SCIE 2022年第6期675-686,共12页
Background:The etiology of pulmonary arterial hypertension associated with congenital heart disease(PAHCHD)is complicated and the phenotype is heterogeneous.Genetic defects of NOTCH3 were associated withcerebral disea... Background:The etiology of pulmonary arterial hypertension associated with congenital heart disease(PAHCHD)is complicated and the phenotype is heterogeneous.Genetic defects of NOTCH3 were associated withcerebral disease and pulmonary hypertension.However,the relationship between NOTCH3 mutations and theclinical phenotype has not been reported in CHD-PAH.Methods:We eventually enrolled 142 PAH-CHD patientsfrom Fuwai Hospital.Whole exome sequencing(WES)was performed to screen the rare deleterious variants ofNOTCH3 gene.Results:This PAH-CHD cohort included 43(30.3%)men and 99(69.7%)women with the meanage 29.8±10.9 years old.The pathogenic or likely pathogenic mutations of NOTCH3 were identified in five cases.Patients 2,5,8 and 11 carried the same NOTCH3 mutation c.1630C>T(pArg544Cys),which is the hot-spotmutation for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy(CADASIL).Patient 3 carried the NOTCH3 mutation p.Arg75Gln that has also been reported to be associatedwith the CADASIL.Patients 2,5,8,11 took the examination of the cerebral magnetic resonance imaging(MRI)and confirmed the phenotype of CADASIL.Conclusions:We first reported the NOTCH3 rare mutationsand CADASIL phenotypes in CHD-PAH patients.The NOTCH3 rare variants were with a relatively high positiverate and CADASIL phenotypes were likely enriched in PAH-CHD patients.The preoperative neurological examinationmight be recommended for PAH-CHD patients to determine the surgical contraindications and reduceintraoperative neurological complications. 展开更多
关键词 pulmonary arterial hypertension congenital heart disease NOTCH3 Cerebral autosomal dominant arteriopathy with subcortical infarcts and lesions(CADASIL)
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Perioperative Nursing for Adult Congenital Heart Disease with Severe Pulmonary Arterial Hypertension 被引量:1
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作者 Shunling Li Surui Liang Weihua Xue 《International Journal of Clinical Medicine》 2020年第9期538-547,共10页
<strong>Objectives: </strong>To explore the main points of perioperative nursing for adult congenital heart disease with severe pulmonary arterial hypertension. <strong>Methods: </strong>A retr... <strong>Objectives: </strong>To explore the main points of perioperative nursing for adult congenital heart disease with severe pulmonary arterial hypertension. <strong>Methods: </strong>A retrospective study of 13 patients with congenital heart disease and severe pulmonary arterial hypertension who admitted to the perioperative period of care from January 2018 to December 2019. To prevent perioperative complications of the patients, the focus is on respiratory and circulatory system care, followed by blood coagulation monitoring, digestive system protection and psychological care. <strong>Results:</strong> All 13 patients passed the perioperative period and were discharged from ICU. <strong>Conclusion: </strong>Adult congenital heart disease with severe pulmonary arterial hypertension has high perioperative risk, respiratory and circulatory system care is the key. 展开更多
关键词 Adult congenital heart Disease (ACHD) pulmonary Arterial hypertension (PAH) Perioperative Nursing
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CORRELATION OF vWF: Ag LEVELS WITH PULMONARY HYPERTENSION IN CONGENITAL HEART DEFECTS
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作者 李筠 周爱卿 《Medical Bulletin of Shanghai Jiaotong University》 CAS 1995年第2期53-56,共4页
vWF: Ag is an important factor reflecting the injury of pulmonary vascular endothelium. We measured plasma concentrations of vWF: Ag in 89 patients with congenital heart defects (left to right shunting) by quantitativ... vWF: Ag is an important factor reflecting the injury of pulmonary vascular endothelium. We measured plasma concentrations of vWF: Ag in 89 patients with congenital heart defects (left to right shunting) by quantitative immunoelectrophoresis and assessed endothelial vWF: Ag directly by an immunoperoxidase stain applied io lung biopsy tissue in 10 patients. The patients with pulmonary hypertension had significanthigher vWF: Ag levels, the elevation was associated with the elevation of PVR (P<0.01). The vWF: Ag levels varied directly with PVR and PAR (r=0.89, 0.82, P<0.05).The regression equations of vWF: Ag-PVR. vWF: Ag-PAR we made cam help us to estimate PVR noninvasiverly. Our study will provide us a theoretical basis to choose an appropriate occasion for corrective suryery. 展开更多
关键词 von Willebrand factor pulmonary hypertension congenital heart DEFECTS
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Clinical Effect of an Improved Post-Operative Feeding Protocol“in Transition”Infants of Congenital Heart Disease with Pulmonary Hypertension
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作者 Huaying He Zhiyong Lin +4 位作者 Yuelan Weng Jianjie Zhou Man Ye Xiaowei Luo Qifeng Zhao 《Congenital Heart Disease》 SCIE 2021年第6期655-673,共19页
Background:To achieve successful management of infants with congenital heart disease(CHD)together with pulmonary hypertension(PH),postoperative care,especially feeding care is vital in addition to surgery.Postoperativ... Background:To achieve successful management of infants with congenital heart disease(CHD)together with pulmonary hypertension(PH),postoperative care,especially feeding care is vital in addition to surgery.Postoperative feeding is comprised of three stages:feeding in the intensive care unit,feeding in the general ward and family feeding,in which the general ward is considered as the“transitional stage”.At present,there is little research on the optimal mode of feeding care for the transitional stage,and there is no universally recognized and accepted protocol.Methods:We retrospectively analyzed 114 CHD infants with PH who underwent family-centered(FC)feeding care from July 2017 to December 2018,and prospectively studied 122 CHD infants with the same baseline level who adopted the improved mode,nurse-parent-driven(NPD)feeding mode from January 2019 to June 2020.The feasibility and efficacy of NPD as a“transitional”feeding nursing mode in CHD infants with PH were compared with the FC cohort by observing and analyzing the stress of family caregivers,feedingrelated complications,the proportion of breastfeeding,improvement of nutritional status,acquisition of knowledge and skills of feeding care,inpatient’s satisfaction rating and prognosis.Results:When compared with the FC feeding care,the NPD mode significantly reduced the burden of family caregivers,improved the rate of feeding care knowledge and skills and inpatient’s satisfaction rating,reduced the incidence of improper feeding-related complications,and enhanced the proportion of breastfeeding and nutritional status of infants at the“transitional stage”(all P<0.05).The self-assessment score of care ability of family caregivers and weight gain of children in the NPD group were significantly higher than those in the FC group(all P<0.05)during the follow-up.Conclusions:As a transitional mode of feeding in CHD infants with PH,NPD feeding care is superior to the conventional FC mode,which therefore can be adopted as a standard protocol in clinical practice. 展开更多
关键词 congenital heart disease pulmonary hypertension postoperative feeding care INFANT
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Plasma HGF and OPN as Potential Biomarkers of Pulmonary Arterial Hypertension in Congenital Heart Disease
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作者 Dongdong Zheng Chi Shen +2 位作者 Wenshi Liu Wenjing Lv Xiaofei Li 《Congenital Heart Disease》 SCIE 2021年第4期373-381,共9页
Objectives:Pulmonary arterial hypertension in congenital heart disease(PAH-CHD)is the most common type of PAH and increases morbidity and mortality in patients with CHD.Right heart catheterization(RHC)is the standard ... Objectives:Pulmonary arterial hypertension in congenital heart disease(PAH-CHD)is the most common type of PAH and increases morbidity and mortality in patients with CHD.Right heart catheterization(RHC)is the standard method to diagnose PAH.However,RHC is an invasive and complicated method with relatively high cost.Noninvasive,feasible,and cost-efficient methods are urgently needed.The objective of this study was to evaluate three potential biomarkers of PAH-CHD:Hepatocyte growth factor(HGF),osteopontin(OPN),and suppression of tumorigenicity 2(ST2).Methods:Plasma samples were collected from patients with CHD(n=46)and healthy individuals(n=22)and divided into four groups according to the severity of PAH.The levels of HGF,OPN,and ST2 were then analyzed using an enzyme-linked immunosorbent assay.Correlations between HGF,OPN,ST2,and clinical parameters of PAH-CHD were analyzed.Results:The plasma HGF levels in the moderate to the severe group were significantly higher than those in the mild group,nonPAH group,and healthy control group(p<0.05).Derived from a receiver operating characteristic(ROC)curve analysis,a cut-off value of 356.75 ng/ml for the HGF concentration was able to predict PAH-CHD with 53%sensitivity and 89%specificity.The HGF level was positively related to pulmonary arterial systolic pressure(PASP)(r=0.36,p<0.05)and pulmonary vascular resistance(PVR)(r=0.36,p<0.05).Plasma OPN levels in the mild group were significantly higher than other groups and positively correlated with the pulmonary-systemic shunt ratio(Qp/Qs)(r=0.33,p<0.05).There was no statistically significant between-group difference in plasma soluble ST2(sST2)levels.Conclusion:The plasma HGF level was elevated in PAH-CHD patients and can be used as a potential biomarker.The plasma OPN level was positively correlated with the Qp/Qs. 展开更多
关键词 congenital heart disease pulmonary hypertension hepatocyte growth factor OSTEOPONTIN suppression of tumorigenicity 2 BIOMARKER
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Assessment of Reversibility in Pulmonary Hypertension Related to Congenital Heart Disease by Using Biomarkers and Clinical Features
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作者 Liping Wang Mingjie Zhang +3 位作者 Xi Chen Yachang Pang Jiaqi Liu Zhuoming Xu 《Congenital Heart Disease》 SCIE 2022年第1期87-97,共11页
Background:Reversibility of pulmonary hypertension(PH)is closely related to the treatment options for and prognosis of children with congenital heart disease.Objective:We combined patient-specific clinical features in... Background:Reversibility of pulmonary hypertension(PH)is closely related to the treatment options for and prognosis of children with congenital heart disease.Objective:We combined patient-specific clinical features including diagnosis,age and echocardiographic results,and biomarkers of pulmonary vascular dysfunction to explore the noninvasive methods that can be used to accurately evaluate the reversibility of pulmonary hypertension in congenital heart disease(PH-CHD).Methods:Based on the preoperative systolic pulmonary arterial pressure(sPAP),70 CHD patients were divided into normal,PH-CHD suspected,and confirmed groups.Additionally,biomarkers of circulating endothelial cells(CECs),endothelin-1(ET-1),and endothelial nitric oxide synthase(eNOS)were detected.Patients were categorized into reversible(RPH)and irreversible(IRPH)groups according to the sPAP 6 months after surgery.Risk stratification was performed according to the clinical features and biomarkers.Results:CECs and ET-1 levels in the confirmed group were significantly higher.eNOS was higher in the confirmed and suspected groups than that in the normal group.CECs in the IRPH group were significantly higher compared to the RPH group.No such intergroup differences were observed with respect to ET-1 and eNOS levels.The ROC curve showed that the risk stratification was of high diagnostic value to evaluate reversibility.Conclusion:The CECs,eNOS,and ET-1 were closely related with PH-CHD.CECs and risk stratification have high practical value in assessing the reversibility of PH-CHD. 展开更多
关键词 pulmonary hypertension in congenital heart disease risk stratification circulating endothelial cells ENDOTHELIN-1 endothelial nitric oxide synthase
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Combined effect of milrinone and NO to treat pulmonary hypertension after cardiopulmonary bypass in congenital heart disease patients
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作者 FANG Gai WEI Xin Pan Jian-hui WANG Rui-ting 《麻醉与监护论坛》 2009年第3期142-144,共3页
关键词 肺部高血压 氧化氮 先天性心脏缺陷 心肺旁路
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Treatment of patients with bosentan in postoperation of congenital heart disease with pulmonary arterial hypertension:a double-blind,randomized controlled trial
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作者 张雅娟 《外科研究与新技术》 2011年第3期187-188,共2页
Objective Endothelin is a key role in the pathogenic of pulmonary arterial hypertension. High concentrations of endothelin 1 have been recorded in plasma and lungs of patients with pulmonary artery hypertension associ... Objective Endothelin is a key role in the pathogenic of pulmonary arterial hypertension. High concentrations of endothelin 1 have been recorded in plasma and lungs of patients with pulmonary artery hypertension associated with congenital heart disease,and the concentrations of endothelin-1 was correlated with severity degree 展开更多
关键词 Treatment of patients with bosentan in postoperation of congenital heart disease with pulmonary arterial hypertension
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Development of pulmonary hypertension remains a major hurdle to corrective surgery in Down syndrome
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作者 Akash Batta Juniali Hatwal 《World Journal of Cardiology》 2024年第1期1-4,共4页
Down syndrome is the most common chromosomal abnormality encountered in clinical practice with 50%of them having associated congenital heart disease(CHD).Shunt lesions account for around 75%of all CHDs in Down syndrom... Down syndrome is the most common chromosomal abnormality encountered in clinical practice with 50%of them having associated congenital heart disease(CHD).Shunt lesions account for around 75%of all CHDs in Down syndrome.Down syndrome patients,especially with large shunts are particularly predisposed to early development of severe pulmonary hypertension(PH)compared with shunt lesions in general population.This necessitates timely surgical correction which remains the only viable option to prevent long term morbidity and mortality.However,despite clear recommendations,there is wide gap between actual practice and fear of underlying PH which often leads to surgical refusals in Down syndrome even when the shunt is reversible.Another peculiarity is that Down syndrome patients can develop PH even after successful correction of shunt.It is not uncommon to come across Down syndrome patients with uncorrected shunts in adulthood with irreversible PH at which stage intracardiac repair is contraindicated and the only option available is a combined heartlung transplant.However,despite the guidelines laid by authorities,the rates of cardiac transplant in adult Down syndrome remain dismal largely attributable to the high prevalence of intellectual disability in them.The index case presents a real-world scenario highlighting the impact of severe PH on treatment strategies and discrimination driven by the fear of worse outcomes in these patients. 展开更多
关键词 Down syndrome congenital heart disease pulmonary hypertension Cardiac transplantation pulmonary vascular resistance Surgical correction
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Congenital heart“Challenges”in Down syndrome
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作者 Maria Drakopoulou Panayotis K Vlachakis +1 位作者 Costas Tsioufis Dimitris Tousoulis 《World Journal of Cardiology》 2024年第5期217-220,共4页
In this editorial,we comment on the article by Kong et al published in the recent issue of the World Journal of Cardiology.In this interesting case,the authors present the challenges faced in managing a 13-year-old pa... In this editorial,we comment on the article by Kong et al published in the recent issue of the World Journal of Cardiology.In this interesting case,the authors present the challenges faced in managing a 13-year-old patient with Down syndrome(DS)and congenital heart disease(CHD)associated with pulmonary arterial hypertension.In this distinct population,the Authors underscore the need for early diagnosis and management as well as the need of a multidisciplinary approach for decision making.It seems that the occurrence of CHD in patients with DS adds layers of complexity to their clinical management.This editorial aims to provide a comprehensive overview of the intricate interplay between DS and congenital heart disorders,offering insights into the nuanced diagnostic and therapeutic considerations for physicians. 展开更多
关键词 Down syndrome congenital heart disease Atrioventricular septal defect pulmonary hypertension Right heart catheterization
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Twin pregnancy with sudden heart failure and pulmonary hypertension after atrial septal defect repair: A case report
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作者 Chun-Xiao Tong Tao Meng 《World Journal of Clinical Cases》 SCIE 2023年第35期8350-8356,共7页
BACKGROUND Pulmonary arterial hypertension(PAH)in pregnancy is one of the major obstetric complications and is considered a contraindication to pregnancy as it is classified as a class IV risk in the revised risk clas... BACKGROUND Pulmonary arterial hypertension(PAH)in pregnancy is one of the major obstetric complications and is considered a contraindication to pregnancy as it is classified as a class IV risk in the revised risk classification of pregnancy by the World Health Organisation.Pregnancy,with its adaptive and expectant mechanical and hormonal changes,negatively affects the cardiopulmonary circulation in pregnant women.Do patients with repaired simple congenital heart disease(CHD)develop other pulmonary and cardiac complications during pregnancy?Can pregnant women with sudden pulmonary hypertension be treated and managed in time?In this paper,we present a case of a 39-year-old woman who underwent cesarean section at 33 wk'gestation and developed PAH secondary to repaired simple CHD.Our research began by a PubMed search for"pulmonary hypertension"and"pregnancy"and"CHD"case reports.Three cases were selected to review PAH in pregnancy after correction of CHD defects.These studies were reviewed,coupled with our own clinical experience.CASE SUMMARY Herein,a case involving a woman who underwent atrial septal defect repair at the age of 34,became pregnant five years later,and had a sudden onset of PAH and right heart failure secondary to symptoms of acute peripheral edema in the third trimester of her pregnancy.As a result,the patient underwent a cesarean section and gave birth to healthy twins.Within three days after cesarean delivery,her cardiac function deteriorated as the pulmonary artery pressure increased.Effec-tive postpartum management,including diuresis,significant oxygen uptake,vasodilators,capacity and anticoagulants management,led to improvements in cardiac function and oxygenation.The patient was discharged from hospital with a stable recovery and transferred to local hospitals for further PAH treatment.CONCLUSION This case served as a reminder to obstetricians of the importance of pregnancy after repair of CHD.It is crucial for patients with CHD to receive early correction.It suggests doctors should not ignore edema of twin pregnancy.Also,it provides a reference for the further standardization of antenatal,in-trapartum and postpartum management for patients with CHD worldwide. 展开更多
关键词 congenital heart defects pulmonary hypertension Right heart failure Twin pregnancy Perioperative management Case report
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Multi-Institutional US Experience of the Occlutech AFR Device in Congenital and Acquired Heart Disease 被引量:1
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作者 Barry O’Callaghan Jenny Zablah +7 位作者 Joseph Vettukattil Daniel Levi Morris Salem Allison Cabalka Jason Anderson Makram Ebeid Ryan Alexy Gareth J.Morgan 《Congenital Heart Disease》 SCIE 2022年第1期107-116,共10页
Objectives:To detail the US multi-institutional experience with the Occlutech©(Occlutech International AB,Helsingborg,Sweden)atrial flow regulator(AFR)in children and adults with acquired or congenital heart dise... Objectives:To detail the US multi-institutional experience with the Occlutech©(Occlutech International AB,Helsingborg,Sweden)atrial flow regulator(AFR)in children and adults with acquired or congenital heart disease.Background:The creation of a long-term atrial communication is desirable in several cardiovascular disease phenotypes,most notably pulmonary arterial hypertension,disorders of increased left ventricular filling and increased cavopulmonary pressures in patients with a Fontan type circulation.Methods:Patients were identified for inclusion from the AFR device manufacturer database.Data was collected using a RedCap database following IRB approval.8 weeks of follow up data was sought for each patient based on available data.Data was analyzed and summarized using SPSS.Results:We report the experience of 6 US centers in the implantation of AFR devices in 15 patients,across a wide age range,with different disease phenotypes and a variety of indications.Implantation was technically successful in all patients and improvement was noted in both clinical and hemodynamic parameters.There were no immediate or intermediate term complications reported.3 patients died remote from implantation.Their deaths were not felt to be related to the AFR device or related procedural complications.Conclusion:Compassionate use of the AFR device in children and adults with congenital&acquired heart disease is technically feasible and produces beneficial short term hemodynamic and symptomatic improvement.Widespread uptake of this technique and treatment at specialist centers has the potential to provide significant benefits to a variety of complex patients with currently limited treatment options and indeterminate prognosis. 展开更多
关键词 congenital heart disease pulmonary hypertension left atrial hypertension single ventricle palliation diastolic heart failure
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Prevalence of pulmonary hypertension among children with Down syndrome:A systematic review and meta-analysis 被引量:1
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作者 Amar Taksande Divya Pujari +2 位作者 Patel Zeeshan Jameel Bharati Taksande Revat Meshram 《World Journal of Clinical Pediatrics》 2021年第6期177-191,共15页
BACKGROUND Pulmonary hypertension(PH)has serious short-and long-term consequences.PH is gaining increasing importance in high risk groups such as Down syndrome(DS)as it influences their overall survival and prognosis.... BACKGROUND Pulmonary hypertension(PH)has serious short-and long-term consequences.PH is gaining increasing importance in high risk groups such as Down syndrome(DS)as it influences their overall survival and prognosis.Hence,there is a dire need to collate the prevalence rates of PH in order to undertake definitive measures for early diagnosis and management.AIM To determine the prevalence of PH in children with DS.METHODS The authors individually conducted a search of electronic databases manually(Cochrane library,PubMed,EMBASE,Scopus,Web of Science).Data extraction and quality control were independently performed by two reviewers and a third reviewer resolved any conflicts of opinion.The words used in the literature search were“pulmonary hypertension”and“pulmonary arterial hypertension”;“Down syndrome”and“trisomy 21”and“prevalence”.The data were analyzed by Comprehensive Meta-Analysis Software Version 2.Risk of bias assessment and STROBE checklist were used for quality assessment.RESULTS Of 1578 articles identified,17 were selected for final analysis.The pooled prevalence of PH in these studies was 25.5%.Subgroup analysis was carried out for age,gender,region,year of publication,risk of bias and etiology of PH.CONCLUSION This review highlights the increasing prevalence of PH in children with DS.It is crucial for pediatricians to be aware of this morbid disease and channel their efforts towards earlier diagnosis and successful management.Community-based studies with a larger sample size of children with DS should be carried out to better characterize the epidemiology and underlying etiology of PH in DS. 展开更多
关键词 Down syndrome pulmonary hypertension PREVALENCE Trisomy 21 Persistent pulmonary hypertension congenital heart disease
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