Background:An increasing number of patients with Fontan circulation are reaching adulthood;however,long-term outcomes remain limited by Fontan failure,which is characterized by elevated central venous pressure(CVP)and...Background:An increasing number of patients with Fontan circulation are reaching adulthood;however,long-term outcomes remain limited by Fontan failure,which is characterized by elevated central venous pressure(CVP)and reduced cardiac output.Red blood cell distribution width(RDW),a readily available hematological parameter,is a known prognostic marker of heart failure.However,its relationship with invasive hemodynamics in adolescent and adult Fontan patients has not been fully examined.Objectives:To clarify the association between RDW and invasive hemodynamic indices in adolescent and adult Fontan patients and assess the utility of RDW as a noninvasive circulatory marker.Methods:This single-center retrospective study included consecutive Fontan patients aged≥16 years who underwent routine cardiac catheterization≥5 years after surgery,between June 2014 and July 2025.Laboratory data and catheter-derived hemodynamics were also analyzed.The primary endpoint was the correlation between RDW and CVP,and the secondary endpoint was the correlation between RDW and central venous oxygen saturation(ScvO_(2)).Results:Forty patients(median age:22 years)were analyzed.The median RDW was 13.3%,and the median CVP was 11.0 mmHg.RDW correlated positively with CVP(ρ=0.57,p<0.001)and negatively with ScvO_(2)(ρ=-0.66,p<0.001)and the cardiac index(ρ=-0.34,p=0.03).Patients with elevated RDW(>14.5%)had higher CVP(14.5 vs.10.5 mmHg,p<0.001)and lower ScvO_(2)(63.8%vs.76.1%,p<0.001),compared with those with normal RDW.Multivariable analysis identified RDW as an independent predictor of ScvO_(2)(p<0.001).Conclusions:In adolescents and adults after the Fontan procedure,RDW was significantly associated with elevated CVP and reduced ScvO_(2)and independently predicted impaired oxygen delivery.RDW is inexpensive,widely accessible,and may serve as a practical noninvasive biomarker for the early detection of Fontan failure and the optimization of invasive testing and interventions during long-term follow-up.展开更多
Fontan surgery is a widely used palliative procedure that significantly improves the survival period of patients with complex congenital heart disease(CHD).However,it does not decrease postoperative complication rate....Fontan surgery is a widely used palliative procedure that significantly improves the survival period of patients with complex congenital heart disease(CHD).However,it does not decrease postoperative complication rate.Previous studies suggested that elevated mean pulmonary artery pressure(m PAP)and vascular resistance lead to decreased exercise tolerance and myocardial dysfunction.Therapy with endothelial receptor antagonists(Bosentan)has been demonstrated to improve the patients'prognosis.A double-blind,randomized controlled trial was performed to explore the efficacy of Bosentan in treating patients who underwent the Fontan procedure.Eligible participants were randomly divided into Bosentan group and control group.Liver function was tested at a local hospital and the results were reported to the phone inspector every month.If the results suggested abnormal liver function,treatment would be adjusted or terminated.All the participants finished the follow-up study,with no patients lost to follow-up.Unblinding after 2-year follow-up,no mortality was observed in either group.However,secondary end-points were found to be significantly different in the comparable groups.The cardiac function and 6-min walking distance in the Bosentan group were significantly superior to those in the control group(P=0.018 and P=0.027).Bosentan could improve New York Heart Association(NYHA)functional status and improve the results of the 6-min walking test(6MWT)in Fontan patients post-surgery,and no other benefits were observed.Furthermore,a primary meta-analysis study systematically reviewed all the similar clinical trails worldwide and concluded an overall NYHA class improvement in Fontan patients who received Bosentan treatments.展开更多
BACKGROUND Single-ventricle congenital heart disease often requires the Fontan procedure,which can lead to Fontan-associated liver disease(FALD)and multi-organ failure.Combined heart-liver transplantation(CHLT)is a po...BACKGROUND Single-ventricle congenital heart disease often requires the Fontan procedure,which can lead to Fontan-associated liver disease(FALD)and multi-organ failure.Combined heart-liver transplantation(CHLT)is a potential lifesaving option for these patients.AIMTo investigate the outcomes and complications of CHLT in patients with failing Fontan physiology.METHODSSeven retrospective studies of 121 patients undergoing CHLT were systematically reviewed. Quality was assessedwith the Newcastle-Ottawa Scale. A meta-analysis using random-effects models to calculate odds ratios (ORs) ormean differences (MDs) with 95% confidence intervals.RESULTSThe pooled 30-day, 1-year, 5-year, and 10-year survival rates after CHLT were 92.6%, 86.78%, 81.17%, and 77.8%,respectively. The mean intensive care unit and total hospital lengths of stay were 8.46 and 28.16 days. Meanischemic time was 267.29 minutes, while cardiopulmonary bypass time was 260.27 minutes. Infections (30%), renalreplacement therapy (36.84%), and graft rejection (12.34%) were notable complications. Compared to orthotopicheart transplantation (OHT), CHLT significantly reduced mortality (OR: 0.30, P = 0.009) and ischemic time (MD:–65.93 minutes), with no major differences in perioperative morbidity.CONCLUSIONCHLT offers a survival advantage over OHT for patients with FALD and failing Fontan physiology. Futureprospective studies are warranted to refine eligibility and improve long-term survival.展开更多
Congenital heart disease(CHD)stands as the most common cardiovascular disorder among children,exerting a profound impact on the growth,development,and quality of life of the affected pediatric population.The modified ...Congenital heart disease(CHD)stands as the most common cardiovascular disorder among children,exerting a profound impact on the growth,development,and quality of life of the affected pediatric population.The modified Fontan procedure,the total cavopulmonary connection(TCPC),has become a pivotal palliative or definitive surgical method for treating complex CHD cases,including single ventricle and tricuspid valve atresia.Through staged surgical processes,this technique directly diverts vena cava blood into the pulmonary artery,thus improving the patient’s oxygenation status.Despite the initial success of the Fontan circulation in providing a means for survival in patients with complex CHD,a significant proportion of patients will eventually experience Fontan failure.Fontan failure is a complex syndrome characterized by a constellation of symptoms and signs,including heart failure,arrhythmia,protein-losing enteropathy,and plastic bronchitis.Understanding the contemporary management of failing modified Fontan after TCPC is crucial for optimizing patient outcomes,as the number of adult patients with Fontan circulation continues to grow due to improved surgical techniques and postoperative care.展开更多
Background:Thromboembolic events are a cause of significant morbidity and mortality in the Fontan population.We previously reported on coagulation profile changes in a cohort of patients with hypoplastic left heart sy...Background:Thromboembolic events are a cause of significant morbidity and mortality in the Fontan population.We previously reported on coagulation profile changes in a cohort of patients with hypoplastic left heart syndrome(HLHS)from Stage I through Fontan completion.In this report,we examine their clinical status,anticoagulation and incidence of thromboembolic events up to 20 years post Fontan.Methods:A retrospective chart review was conducted for twenty(20)surviving patients,from 1998 through December 2020.Patients who underwent orthotopic heart transplantation(OTx)were followed until their transplant.Patients who were found in the original study to have a factor VIII activity level>160%,were examined separately.Results:Most patients had follow-up within the last two years(2018–2020).Two patients underwent OTx and two patients died.Anticoagulation strategy was variable.Most patients were on aspirin monotherapy.There was a total of twelve thrombotic events(63.2%).These included six cerebrovascular accidents(two of which were fatal).Three out of the seven patients with elevated factor VIII activity from the original study had thromboembolic events(42.9%).Fontan complications were variable.Some degree of Fontan Associated Liver Disease was universal.Conclusions:This retrospective review of a group of single-ventricle patients post Fontan,illustrates the variability in anticoagulation therapy that exists in this population.A large proportion of patients suffered a significant thromboembolic event,including the patients with elevated factor VIII.Further investigation into the patients with elevated factor VIII may help determine whether a different antithrombotic strategy post Fontan would be beneficial.展开更多
This study is performed to investigate the effect of Fontan operations on cardiac contractility. Investigation of articles about the contractility in Fontan published in PubMed up to now revealed that the measurement ...This study is performed to investigate the effect of Fontan operations on cardiac contractility. Investigation of articles about the contractility in Fontan published in PubMed up to now revealed that the measurement of contractility values did not include the rudimentary ventricle. Connections between right atrium and right ventricle as well as right atrium and pulmonary artery were studied in the same group as well. In our opinion, for the single ventricular physiology, the RA-RV and RA-PA connected patients’ preload and afterload values cannot be calculated in the same group since volume of rudimentary ventricles must be considered from this point of view as well.展开更多
文摘Background:An increasing number of patients with Fontan circulation are reaching adulthood;however,long-term outcomes remain limited by Fontan failure,which is characterized by elevated central venous pressure(CVP)and reduced cardiac output.Red blood cell distribution width(RDW),a readily available hematological parameter,is a known prognostic marker of heart failure.However,its relationship with invasive hemodynamics in adolescent and adult Fontan patients has not been fully examined.Objectives:To clarify the association between RDW and invasive hemodynamic indices in adolescent and adult Fontan patients and assess the utility of RDW as a noninvasive circulatory marker.Methods:This single-center retrospective study included consecutive Fontan patients aged≥16 years who underwent routine cardiac catheterization≥5 years after surgery,between June 2014 and July 2025.Laboratory data and catheter-derived hemodynamics were also analyzed.The primary endpoint was the correlation between RDW and CVP,and the secondary endpoint was the correlation between RDW and central venous oxygen saturation(ScvO_(2)).Results:Forty patients(median age:22 years)were analyzed.The median RDW was 13.3%,and the median CVP was 11.0 mmHg.RDW correlated positively with CVP(ρ=0.57,p<0.001)and negatively with ScvO_(2)(ρ=-0.66,p<0.001)and the cardiac index(ρ=-0.34,p=0.03).Patients with elevated RDW(>14.5%)had higher CVP(14.5 vs.10.5 mmHg,p<0.001)and lower ScvO_(2)(63.8%vs.76.1%,p<0.001),compared with those with normal RDW.Multivariable analysis identified RDW as an independent predictor of ScvO_(2)(p<0.001).Conclusions:In adolescents and adults after the Fontan procedure,RDW was significantly associated with elevated CVP and reduced ScvO_(2)and independently predicted impaired oxygen delivery.RDW is inexpensive,widely accessible,and may serve as a practical noninvasive biomarker for the early detection of Fontan failure and the optimization of invasive testing and interventions during long-term follow-up.
基金supported by the National Natural Science Foundation of China(No.31330029)
文摘Fontan surgery is a widely used palliative procedure that significantly improves the survival period of patients with complex congenital heart disease(CHD).However,it does not decrease postoperative complication rate.Previous studies suggested that elevated mean pulmonary artery pressure(m PAP)and vascular resistance lead to decreased exercise tolerance and myocardial dysfunction.Therapy with endothelial receptor antagonists(Bosentan)has been demonstrated to improve the patients'prognosis.A double-blind,randomized controlled trial was performed to explore the efficacy of Bosentan in treating patients who underwent the Fontan procedure.Eligible participants were randomly divided into Bosentan group and control group.Liver function was tested at a local hospital and the results were reported to the phone inspector every month.If the results suggested abnormal liver function,treatment would be adjusted or terminated.All the participants finished the follow-up study,with no patients lost to follow-up.Unblinding after 2-year follow-up,no mortality was observed in either group.However,secondary end-points were found to be significantly different in the comparable groups.The cardiac function and 6-min walking distance in the Bosentan group were significantly superior to those in the control group(P=0.018 and P=0.027).Bosentan could improve New York Heart Association(NYHA)functional status and improve the results of the 6-min walking test(6MWT)in Fontan patients post-surgery,and no other benefits were observed.Furthermore,a primary meta-analysis study systematically reviewed all the similar clinical trails worldwide and concluded an overall NYHA class improvement in Fontan patients who received Bosentan treatments.
文摘BACKGROUND Single-ventricle congenital heart disease often requires the Fontan procedure,which can lead to Fontan-associated liver disease(FALD)and multi-organ failure.Combined heart-liver transplantation(CHLT)is a potential lifesaving option for these patients.AIMTo investigate the outcomes and complications of CHLT in patients with failing Fontan physiology.METHODSSeven retrospective studies of 121 patients undergoing CHLT were systematically reviewed. Quality was assessedwith the Newcastle-Ottawa Scale. A meta-analysis using random-effects models to calculate odds ratios (ORs) ormean differences (MDs) with 95% confidence intervals.RESULTSThe pooled 30-day, 1-year, 5-year, and 10-year survival rates after CHLT were 92.6%, 86.78%, 81.17%, and 77.8%,respectively. The mean intensive care unit and total hospital lengths of stay were 8.46 and 28.16 days. Meanischemic time was 267.29 minutes, while cardiopulmonary bypass time was 260.27 minutes. Infections (30%), renalreplacement therapy (36.84%), and graft rejection (12.34%) were notable complications. Compared to orthotopicheart transplantation (OHT), CHLT significantly reduced mortality (OR: 0.30, P = 0.009) and ischemic time (MD:–65.93 minutes), with no major differences in perioperative morbidity.CONCLUSIONCHLT offers a survival advantage over OHT for patients with FALD and failing Fontan physiology. Futureprospective studies are warranted to refine eligibility and improve long-term survival.
文摘Congenital heart disease(CHD)stands as the most common cardiovascular disorder among children,exerting a profound impact on the growth,development,and quality of life of the affected pediatric population.The modified Fontan procedure,the total cavopulmonary connection(TCPC),has become a pivotal palliative or definitive surgical method for treating complex CHD cases,including single ventricle and tricuspid valve atresia.Through staged surgical processes,this technique directly diverts vena cava blood into the pulmonary artery,thus improving the patient’s oxygenation status.Despite the initial success of the Fontan circulation in providing a means for survival in patients with complex CHD,a significant proportion of patients will eventually experience Fontan failure.Fontan failure is a complex syndrome characterized by a constellation of symptoms and signs,including heart failure,arrhythmia,protein-losing enteropathy,and plastic bronchitis.Understanding the contemporary management of failing modified Fontan after TCPC is crucial for optimizing patient outcomes,as the number of adult patients with Fontan circulation continues to grow due to improved surgical techniques and postoperative care.
文摘Background:Thromboembolic events are a cause of significant morbidity and mortality in the Fontan population.We previously reported on coagulation profile changes in a cohort of patients with hypoplastic left heart syndrome(HLHS)from Stage I through Fontan completion.In this report,we examine their clinical status,anticoagulation and incidence of thromboembolic events up to 20 years post Fontan.Methods:A retrospective chart review was conducted for twenty(20)surviving patients,from 1998 through December 2020.Patients who underwent orthotopic heart transplantation(OTx)were followed until their transplant.Patients who were found in the original study to have a factor VIII activity level>160%,were examined separately.Results:Most patients had follow-up within the last two years(2018–2020).Two patients underwent OTx and two patients died.Anticoagulation strategy was variable.Most patients were on aspirin monotherapy.There was a total of twelve thrombotic events(63.2%).These included six cerebrovascular accidents(two of which were fatal).Three out of the seven patients with elevated factor VIII activity from the original study had thromboembolic events(42.9%).Fontan complications were variable.Some degree of Fontan Associated Liver Disease was universal.Conclusions:This retrospective review of a group of single-ventricle patients post Fontan,illustrates the variability in anticoagulation therapy that exists in this population.A large proportion of patients suffered a significant thromboembolic event,including the patients with elevated factor VIII.Further investigation into the patients with elevated factor VIII may help determine whether a different antithrombotic strategy post Fontan would be beneficial.
文摘This study is performed to investigate the effect of Fontan operations on cardiac contractility. Investigation of articles about the contractility in Fontan published in PubMed up to now revealed that the measurement of contractility values did not include the rudimentary ventricle. Connections between right atrium and right ventricle as well as right atrium and pulmonary artery were studied in the same group as well. In our opinion, for the single ventricular physiology, the RA-RV and RA-PA connected patients’ preload and afterload values cannot be calculated in the same group since volume of rudimentary ventricles must be considered from this point of view as well.
