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
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
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
Objective To research the expression of endothelin-1 (ET-1) in vascular of lung, endocardium and myocardial vascular of congenital heart disease (CHD) ventricular septal defect (VSD) with pulmonary hypertension (PH...Objective To research the expression of endothelin-1 (ET-1) in vascular of lung, endocardium and myocardial vascular of congenital heart disease (CHD) ventricular septal defect (VSD) with pulmonary hypertension (PH). Methods The Streptavidin-peroxidase (SP) immunoassay was used to measure the expression of ET-1 in pulmonary arteriola, pulmonary veinlet, endocardium and endangium of vasa coronary of 20 cases VSD of CHD with PH, and contrast the expression level of these 20 cases VSD of CHD with PH. Results The expression of ET-1 PH patients in pulmonary arteriola, endocardium, and endangium of vasa coronary was much higher than that of the control group (P<0.05), but there was no significance variance in expression level of pulmonary veinlet between two groups (P>0.05). Conclusion If VSD of CHD was accompany with PH, the degree of PH has a positive correlation with the amount of ET-1 in pulmonary arteriola, ET-1 may be the cause of dynamic PH, and also the acceleration factor of the PH. However, the amount of ET-1 in endocardium and endangium of vasa coronary, may have significant connection with the myocardium hypertrophy in dynamic PH.展开更多
Background Echocardiogram is a simple and useful tool in disease assessment.In ventricular septal defect(VSD)patients with severe pulmonary hypertension(PH),it is difficult to judge whether further intervention is nee...Background Echocardiogram is a simple and useful tool in disease assessment.In ventricular septal defect(VSD)patients with severe pulmonary hypertension(PH),it is difficult to judge whether further intervention is needed.Therefore,this study aimed to investigate the application of echocardiogram in predicting the severity of pulmonary hypertension and guide subsequent management.Methods This study included VSD patients who underwent right heart catheterization(RHC)examination in Guangdong Provincial People's Hospital from January2011 to December 2022.An estimated pulmonary artery systolic pressure(e PASP)higher than 60 mm Hg was defined as severe PH in this study.Logistic regression analysis and receiver operating characteristic curve(ROC)analysis were used.Results A total of 186 VSD patients with severe PH(e PASP more than 60 mm Hg)were included in this study,with 158 cases in the non-correctable group and 28 cases in the collectable group.In univariable logistic regression,left atrium dimension(LA),left ventricular end-diastolic dimension(LVDd),left ventricular end-systolic dimension(LVDs),peak velocity of the pulmonary valve(PV),peak velocities from the early phase of the mitral inflow(MVE),bidirectional shunting and pericardial effusion were associated with a correctable shunt.When adjusted in multivariable model,only PV and bidirectional shunting remained statistically significant.The ROC curve found that PV exhibits good discriminative ability for correctable shunt(AUC[area under the curve]:0.779,95%CI:0.676-0.871)with a cut-off value of 1.465 m/s.The predictive performance of bidirectional shunting was not satisfactory,with a low AUC of 0.669(95%CI:0.571-0.766).Conclusions PV and bidirectional shunting are simple and clinically available parameters from echocardiogram in predicting PH severity,which not only avoids the repeated unnecessary cardiac catheterization,but also provides a reference basis for follow-up evaluation.展开更多
目的探讨成人先天性心脏病合并肺动脉高压患者围手术期血浆白细胞介素-17(IL-17)水平变化的规律及意义。方法择期体外循环(CPB)下行先天性心脏病修补术患者40例,根据肺动脉收缩压(PASP)分为非肺动脉高压组(Ⅰ组,PASP<30 mm Hg)以及...目的探讨成人先天性心脏病合并肺动脉高压患者围手术期血浆白细胞介素-17(IL-17)水平变化的规律及意义。方法择期体外循环(CPB)下行先天性心脏病修补术患者40例,根据肺动脉收缩压(PASP)分为非肺动脉高压组(Ⅰ组,PASP<30 mm Hg)以及肺动脉高压组(Ⅱ组,PASP≥30 mm Hg)。于麻醉诱导前(T_1)、CPB即刻(T_2)、停CPB后30 min(T_3)、术后6 h(T_4)、术后24 h(T_5)以及术后7 d(T_6)采集动脉血标本,检测血浆IL-17的水平,同时测定前5个时点患者的动脉氧分压[p(O_2)]及二氧化碳分压[p(CO_2)],计算氧合指数(OI)及肺泡-动脉氧分压差(AaDO_2)。结果整个围手术期Ⅱ组血浆IL-17水平高于Ⅰ组(P<0.05),且2组IL-17水平均于T3期达到峰值,之后降低;此时2组OI降低,而AaDO_2却明显升高。术后24 h(T_5),与Ⅰ组相比,Ⅱ组OI下降,AaDO_2出现升高(P<0.05)。结论高水平的IL-17在先天性心脏病肺动脉高压的形成中起一定促进作用,可能参与了CPB后的肺损伤,可作为评估病情的指标。展开更多
文摘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.
文摘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.
文摘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.
文摘Objective To research the expression of endothelin-1 (ET-1) in vascular of lung, endocardium and myocardial vascular of congenital heart disease (CHD) ventricular septal defect (VSD) with pulmonary hypertension (PH). Methods The Streptavidin-peroxidase (SP) immunoassay was used to measure the expression of ET-1 in pulmonary arteriola, pulmonary veinlet, endocardium and endangium of vasa coronary of 20 cases VSD of CHD with PH, and contrast the expression level of these 20 cases VSD of CHD with PH. Results The expression of ET-1 PH patients in pulmonary arteriola, endocardium, and endangium of vasa coronary was much higher than that of the control group (P<0.05), but there was no significance variance in expression level of pulmonary veinlet between two groups (P>0.05). Conclusion If VSD of CHD was accompany with PH, the degree of PH has a positive correlation with the amount of ET-1 in pulmonary arteriola, ET-1 may be the cause of dynamic PH, and also the acceleration factor of the PH. However, the amount of ET-1 in endocardium and endangium of vasa coronary, may have significant connection with the myocardium hypertrophy in dynamic PH.
基金supported by the Guangdong Peak Project(No.DFJH201802)the National Natural Science Foundation of China(No.82371963)+1 种基金Guangdong Basic and Applied Basic Research Foundation(No.2021A1515012232/No.2023A1515011366)the Natural Science Foundation lauching fund of the Guangdong Provincial People's Hospital(No.8197070830/No.8207070477/No.8227070211)。
文摘Background Echocardiogram is a simple and useful tool in disease assessment.In ventricular septal defect(VSD)patients with severe pulmonary hypertension(PH),it is difficult to judge whether further intervention is needed.Therefore,this study aimed to investigate the application of echocardiogram in predicting the severity of pulmonary hypertension and guide subsequent management.Methods This study included VSD patients who underwent right heart catheterization(RHC)examination in Guangdong Provincial People's Hospital from January2011 to December 2022.An estimated pulmonary artery systolic pressure(e PASP)higher than 60 mm Hg was defined as severe PH in this study.Logistic regression analysis and receiver operating characteristic curve(ROC)analysis were used.Results A total of 186 VSD patients with severe PH(e PASP more than 60 mm Hg)were included in this study,with 158 cases in the non-correctable group and 28 cases in the collectable group.In univariable logistic regression,left atrium dimension(LA),left ventricular end-diastolic dimension(LVDd),left ventricular end-systolic dimension(LVDs),peak velocity of the pulmonary valve(PV),peak velocities from the early phase of the mitral inflow(MVE),bidirectional shunting and pericardial effusion were associated with a correctable shunt.When adjusted in multivariable model,only PV and bidirectional shunting remained statistically significant.The ROC curve found that PV exhibits good discriminative ability for correctable shunt(AUC[area under the curve]:0.779,95%CI:0.676-0.871)with a cut-off value of 1.465 m/s.The predictive performance of bidirectional shunting was not satisfactory,with a low AUC of 0.669(95%CI:0.571-0.766).Conclusions PV and bidirectional shunting are simple and clinically available parameters from echocardiogram in predicting PH severity,which not only avoids the repeated unnecessary cardiac catheterization,but also provides a reference basis for follow-up evaluation.
文摘目的探讨成人先天性心脏病合并肺动脉高压患者围手术期血浆白细胞介素-17(IL-17)水平变化的规律及意义。方法择期体外循环(CPB)下行先天性心脏病修补术患者40例,根据肺动脉收缩压(PASP)分为非肺动脉高压组(Ⅰ组,PASP<30 mm Hg)以及肺动脉高压组(Ⅱ组,PASP≥30 mm Hg)。于麻醉诱导前(T_1)、CPB即刻(T_2)、停CPB后30 min(T_3)、术后6 h(T_4)、术后24 h(T_5)以及术后7 d(T_6)采集动脉血标本,检测血浆IL-17的水平,同时测定前5个时点患者的动脉氧分压[p(O_2)]及二氧化碳分压[p(CO_2)],计算氧合指数(OI)及肺泡-动脉氧分压差(AaDO_2)。结果整个围手术期Ⅱ组血浆IL-17水平高于Ⅰ组(P<0.05),且2组IL-17水平均于T3期达到峰值,之后降低;此时2组OI降低,而AaDO_2却明显升高。术后24 h(T_5),与Ⅰ组相比,Ⅱ组OI下降,AaDO_2出现升高(P<0.05)。结论高水平的IL-17在先天性心脏病肺动脉高压的形成中起一定促进作用,可能参与了CPB后的肺损伤,可作为评估病情的指标。
