BACKGROUND Fontan-associated liver disease(FALD)often occurs in patients with single-ventricle physiology following Fontan surgery,and ranges from liver congestion to cirrhosis.The assessment of the severity of FALD u...BACKGROUND Fontan-associated liver disease(FALD)often occurs in patients with single-ventricle physiology following Fontan surgery,and ranges from liver congestion to cirrhosis.The assessment of the severity of FALD using noninvasive methods is challenging.However,transient elastography(TE)may be useful for the non-invasive evaluation of FALD and prediction of clinical outcomes.AIM To evaluate the role of TE in the diagnosis of FALD and its association with clinically relevant events.METHODS This retrospective single-center study(Hospital Universitario La Paz,Madrid),including 91 post-Fontan patients aged>18 years old.Laboratory and ultrasound findings,and liver stiffness measurements(LSM)by TE(FibroScan®)were assessed.FALD was defined using ultrasound criteria hepatomegaly,liver surface nodularity,parenchymal heterogeneity,hyperechoic lesions,spleno-RESULTS Patient characteristics were:60.4%male;Mean age,33.3±8.2 years;Mean elapsed time since surgery,24.3±7.7 years;89%with FALD;73%with advanced FALD.LSM by TE was associated with FALD[odds ratio(OR)=1.34;95%confidence interval(95%CI):1.10-1.64;P=0.003]and advanced FALD(OR=1.10;95%CI:1.01-1.19;P=0.023).Areas under the curve(AUC)were 0.905 and 0.764 for FALD and advanced FALD,respectively.FALD cut-off values comprised:Optimal,20 kPa(sensitivity:92.3%;specificity:80.0%);Rule-out,15 kPa(sensitivity:96.9%);Rule-in,25 kPa(specificity:100%).A FALD algorithm was proposed based on LSM by TE and elapsed time since surgery(AUC:0.877;sensitivity,95.4%;specificity,80.0%;positive predictive value,96.9%;negative predictive value,72.7%).LSM by TE was associated with clinically relevant events(OR=1.07;95%CI:1.01-1.13;P=0.021)and all-cause mortality(OR=1.23;95%CI:1.02-1.47;P=0.026).CONCLUSION In adult patients post-Fontan surgery,TE is a useful noninvasive method for FALD diagnosis.The association between LSM by TE and clinically relevant events suggests a role in prognosis.展开更多
Background:Patients with Fontan physiology are predisposed to congestive hepatopathy,progressive liver fib rosis,and end-stage liver disease.Ulrasound-based shear wave elastography(SWE)is a non-invasive tool to diagno...Background:Patients with Fontan physiology are predisposed to congestive hepatopathy,progressive liver fib rosis,and end-stage liver disease.Ulrasound-based shear wave elastography(SWE)is a non-invasive tool to diagnose and mnonitor liver fibrosis,We sought to deternine whether the degreeof hemodynanic derangement priorto and after the Fontan operation is associated with irncreased liverstiffness measured by SWE.Methods:A single-center retrospectivestudy of patients with Pontan circulationwho underwert ultrasound elastography between 2008 and 2024 was conducted,Liver stiffness wasmeasuued by SWE and teported as vlocity in n/s.Henodynanic assessmert with catrdiac cathetaizationand echocardiograms were collected before and after the Fontan operation,Data was analyzed as acontinuous(lineat regression model)and a dichotomous variable(t-test).Results:78 pcst-Fortan ultrasoundelactography studies pt fotmedin 56 patients wee analyzed.Pre-Fontan hemnodyrnarrics ircluded medianeffective pulmonary flow of 2.45 L/min/m^(2)[2.21,3.16](p=0.46)ventricular end-diastolic pressure of10 mmHg[8,10.5](p=0.77),and median Glenn ptessue of 12 mmHg[13,15](p=0.83).Post-Fontan mediansystemnic cardiac index was 2.80 L/min/m^(2)[2.4,3.34](p=0.93),median ventricular end-diastolic pressureof 12 mmHg[13.5,14](p=0.99),median systenic saturation of 93%[87,96](p=0.77),median indexedpulmonary vasculat resistance of 1.80 WU·m^(2)[1.49,2.37](p=0.93),and median Fontan ptessue of 18 mmHg[16,21](p=0.86).No corelation was found between SWE and hemodynamics.On echoc ardiography,no correlation was found between SWE and systenic vantricular systolic function(p=0.35)or degree ofsystenic atriovertricula valve regurgitation (p=0.35).Conclusions:The degree of liver stffness by SWEinthis cohort did not correlate withpre and post-Fontan hermodynarnics on cat diac catheterization,degr ee ofverntriculat dysfuntiony or severity of atrioventriculat valve regurgitation by echocardiography.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
Fontan operation is indicated in a single ventricle congenital heart disease which creates a shunt between superior vena cava and pulmonary arteries leading to increased pressure in central venous system and congestiv...Fontan operation is indicated in a single ventricle congenital heart disease which creates a shunt between superior vena cava and pulmonary arteries leading to increased pressure in central venous system and congestive hepatopathy,namely,Fontan-associated liver disease(FALD)[1].Recently,the long-term prognosis after Fontan operation has been improving and the number of patients diagnosed with hepatocellular carcinoma(HCC)arising from FALD is increasing[1].There are several publications on the safety of laparoscopic,conventional surgery and interventional radiological modalities in FALD-HCC patients[2-5].However,there are no reports regarding the robotic hepatectomy for the FALD-HCC patients.This was the first report showing the safety of robotic anatomical hepatectomy in FALD-HCC patients.展开更多
Background: The population of Fontan patients, patients born with a single functioningventricle, is growing. There is a growing need to develop algorithms for this population that can predicthealth outcomes. Artiffcia...Background: The population of Fontan patients, patients born with a single functioningventricle, is growing. There is a growing need to develop algorithms for this population that can predicthealth outcomes. Artiffcial intelligence models predicting short-term and long-term health outcomes forpatients with the Fontan circulation are needed. Generative adversarial networks (GANs) provide a solutionfor generating realistic and useful synthetic data that can be used to train such models. Methods: Despitetheir promise, GANs have not been widely adopted in the congenital heart disease research communitydue, in some part, to a lack of knowledge on how to employ them. In this research study, a GAN was usedto generate synthetic data from the Pediatric Heart Network Fontan I dataset. A subset of data consistingof the echocardiographic and BNP measures collected from Fontan patients was used to train the GAN.Two sets of synthetic data were created to understand the effect of data missingness on synthetic datageneration. Synthetic data was created from real data in which the missing values were imputed usingMultiple Imputation by Chained Equations (MICE) (referred to as synthetic from imputed real samples). Inaddition, synthetic data was created from real data in which the missing values were dropped (referred to assynthetic from dropped real samples). Both synthetic datasets were evaluated for ffdelity by using visualmethods which involved comparing histograms and principal component analysis (PCA) plots. Fidelitywas measured quantitatively by (1) comparing synthetic and real data using the Kolmogorov-Smirnovtest to evaluate the similarity between two distributions and (2) training a neural network to distinguishbetween real and synthetic samples. Both synthetic datasets were evaluated for utility by training aneural network with synthetic data and testing the neural network on its ability to classify patients thathave ventricular dysfunction using echocardiograph measures and serological measures. Results: Usinghistograms, associated probability density functions, and (PCA), both synthetic datasets showed visualresemblance in distribution and variance to real Fontan data. Quantitatively, synthetic data from droppedreal samples had higher similarity scores, as demonstrated by the Kolmogorov–Smirnov statistic, for all butone feature (age at Fontan) compared to synthetic data from imputed real samples, which demonstrateddissimilar scores for three features (Echo SV, Echo tda, and BNP). In addition, synthetic data from droppedreal samples resembled real data to a larger extent (49.3% classiffcation error) than synthetic data fromimputed real samples (65.28% classiffcation error). Classiffcation errors approximating 50% represent datasetsthat are indistinguishable. In terms of utility, synthetic data created from real data in which the missingvalues were imputed classiffed ventricular dysfunction in real data with a classiffcation error of 10.99%.Similarly, utility of the generated synthetic data by showing that a neural network trained on synthetic dataderived from real data in which the missing values were dropped could classify ventricular dysfunction inreal data with a classiffcation error of 9.44%. Conclusions: Although representing a limited subset of thevast data available on the Pediatric Heart Network, generative adversarial networks can create syntheticdata that mimics the probability distribution of real Fontan echocardiographic measures. Clinicians can usethese synthetic data to create models that predict health outcomes for Fontan patients.展开更多
Objective:The objective of this study was to compare cardiac transplant operative and postoperative courses of patients with failed Fontan physiology who were initially palliated with a Norwood(FFN)to those without a ...Objective:The objective of this study was to compare cardiac transplant operative and postoperative courses of patients with failed Fontan physiology who were initially palliated with a Norwood(FFN)to those without a prior Norwood(FF).Methods:A single-institution retrospective review of all patients with Fontan failure who under-went cardiac transplantation from 2003–2021 was completed-22 underwent prior Norwood(FFN)and 11 did not(FF).Descriptive and inferential statistics were calculated for operative course and patient outcomes.Results:The operative course of the FFN cohort appeared to be more complex(not statistically significant,but clinically relevant)-this group exclusively experienced sternal re-entry events(3 of 22 patients)and concomitant neo-aor-tic reconstruction(6 patients),had a longer duration of surgery(median of 682 min vs.575.5 min),more time on circulatory arrest(median of 25.5 min vs.12.5 min),and more frequent use of open sternal management[50%of patients(11/22)vs.27.3%of patients(3/11)].Postoperatively,these patients underwent more mediastinal explora-tions[other than sternal closure;40.9%of patients(9/22)vs.18.2%of patients(2/11)],spent more time on mechanical ventilation(median of 5 days vs.2 days),had a longer length of stay(median of 30 days vs.19 days),and required more catheter-based re-interventions[22.7%of patients(5/22)vs.9.1%of patients(1/11)].Conclu-sion:Although underpowered,our results suggest that the operative course of FFN patients is more challenging,based mostly on neo-aortic arch issues.In turn,this likely leads to a more complex postoperative course.We are currently collaborating with other institutions to increase the cohort size and power of the study.展开更多
文摘BACKGROUND Fontan-associated liver disease(FALD)often occurs in patients with single-ventricle physiology following Fontan surgery,and ranges from liver congestion to cirrhosis.The assessment of the severity of FALD using noninvasive methods is challenging.However,transient elastography(TE)may be useful for the non-invasive evaluation of FALD and prediction of clinical outcomes.AIM To evaluate the role of TE in the diagnosis of FALD and its association with clinically relevant events.METHODS This retrospective single-center study(Hospital Universitario La Paz,Madrid),including 91 post-Fontan patients aged>18 years old.Laboratory and ultrasound findings,and liver stiffness measurements(LSM)by TE(FibroScan®)were assessed.FALD was defined using ultrasound criteria hepatomegaly,liver surface nodularity,parenchymal heterogeneity,hyperechoic lesions,spleno-RESULTS Patient characteristics were:60.4%male;Mean age,33.3±8.2 years;Mean elapsed time since surgery,24.3±7.7 years;89%with FALD;73%with advanced FALD.LSM by TE was associated with FALD[odds ratio(OR)=1.34;95%confidence interval(95%CI):1.10-1.64;P=0.003]and advanced FALD(OR=1.10;95%CI:1.01-1.19;P=0.023).Areas under the curve(AUC)were 0.905 and 0.764 for FALD and advanced FALD,respectively.FALD cut-off values comprised:Optimal,20 kPa(sensitivity:92.3%;specificity:80.0%);Rule-out,15 kPa(sensitivity:96.9%);Rule-in,25 kPa(specificity:100%).A FALD algorithm was proposed based on LSM by TE and elapsed time since surgery(AUC:0.877;sensitivity,95.4%;specificity,80.0%;positive predictive value,96.9%;negative predictive value,72.7%).LSM by TE was associated with clinically relevant events(OR=1.07;95%CI:1.01-1.13;P=0.021)and all-cause mortality(OR=1.23;95%CI:1.02-1.47;P=0.026).CONCLUSION In adult patients post-Fontan surgery,TE is a useful noninvasive method for FALD diagnosis.The association between LSM by TE and clinically relevant events suggests a role in prognosis.
基金Our study used RedCap(Grant UL1TR001427.Together:Transforming and TranslatingDiscovery to Improve Health.David Nelson).
文摘Background:Patients with Fontan physiology are predisposed to congestive hepatopathy,progressive liver fib rosis,and end-stage liver disease.Ulrasound-based shear wave elastography(SWE)is a non-invasive tool to diagnose and mnonitor liver fibrosis,We sought to deternine whether the degreeof hemodynanic derangement priorto and after the Fontan operation is associated with irncreased liverstiffness measured by SWE.Methods:A single-center retrospectivestudy of patients with Pontan circulationwho underwert ultrasound elastography between 2008 and 2024 was conducted,Liver stiffness wasmeasuued by SWE and teported as vlocity in n/s.Henodynanic assessmert with catrdiac cathetaizationand echocardiograms were collected before and after the Fontan operation,Data was analyzed as acontinuous(lineat regression model)and a dichotomous variable(t-test).Results:78 pcst-Fortan ultrasoundelactography studies pt fotmedin 56 patients wee analyzed.Pre-Fontan hemnodyrnarrics ircluded medianeffective pulmonary flow of 2.45 L/min/m^(2)[2.21,3.16](p=0.46)ventricular end-diastolic pressure of10 mmHg[8,10.5](p=0.77),and median Glenn ptessue of 12 mmHg[13,15](p=0.83).Post-Fontan mediansystemnic cardiac index was 2.80 L/min/m^(2)[2.4,3.34](p=0.93),median ventricular end-diastolic pressureof 12 mmHg[13.5,14](p=0.99),median systenic saturation of 93%[87,96](p=0.77),median indexedpulmonary vasculat resistance of 1.80 WU·m^(2)[1.49,2.37](p=0.93),and median Fontan ptessue of 18 mmHg[16,21](p=0.86).No corelation was found between SWE and hemodynamics.On echoc ardiography,no correlation was found between SWE and systenic vantricular systolic function(p=0.35)or degree ofsystenic atriovertricula valve regurgitation (p=0.35).Conclusions:The degree of liver stffness by SWEinthis cohort did not correlate withpre and post-Fontan hermodynarnics on cat diac catheterization,degr ee ofverntriculat dysfuntiony or severity of atrioventriculat valve regurgitation by echocardiography.
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Fontan operation is indicated in a single ventricle congenital heart disease which creates a shunt between superior vena cava and pulmonary arteries leading to increased pressure in central venous system and congestive hepatopathy,namely,Fontan-associated liver disease(FALD)[1].Recently,the long-term prognosis after Fontan operation has been improving and the number of patients diagnosed with hepatocellular carcinoma(HCC)arising from FALD is increasing[1].There are several publications on the safety of laparoscopic,conventional surgery and interventional radiological modalities in FALD-HCC patients[2-5].However,there are no reports regarding the robotic hepatectomy for the FALD-HCC patients.This was the first report showing the safety of robotic anatomical hepatectomy in FALD-HCC patients.
文摘Background: The population of Fontan patients, patients born with a single functioningventricle, is growing. There is a growing need to develop algorithms for this population that can predicthealth outcomes. Artiffcial intelligence models predicting short-term and long-term health outcomes forpatients with the Fontan circulation are needed. Generative adversarial networks (GANs) provide a solutionfor generating realistic and useful synthetic data that can be used to train such models. Methods: Despitetheir promise, GANs have not been widely adopted in the congenital heart disease research communitydue, in some part, to a lack of knowledge on how to employ them. In this research study, a GAN was usedto generate synthetic data from the Pediatric Heart Network Fontan I dataset. A subset of data consistingof the echocardiographic and BNP measures collected from Fontan patients was used to train the GAN.Two sets of synthetic data were created to understand the effect of data missingness on synthetic datageneration. Synthetic data was created from real data in which the missing values were imputed usingMultiple Imputation by Chained Equations (MICE) (referred to as synthetic from imputed real samples). Inaddition, synthetic data was created from real data in which the missing values were dropped (referred to assynthetic from dropped real samples). Both synthetic datasets were evaluated for ffdelity by using visualmethods which involved comparing histograms and principal component analysis (PCA) plots. Fidelitywas measured quantitatively by (1) comparing synthetic and real data using the Kolmogorov-Smirnovtest to evaluate the similarity between two distributions and (2) training a neural network to distinguishbetween real and synthetic samples. Both synthetic datasets were evaluated for utility by training aneural network with synthetic data and testing the neural network on its ability to classify patients thathave ventricular dysfunction using echocardiograph measures and serological measures. Results: Usinghistograms, associated probability density functions, and (PCA), both synthetic datasets showed visualresemblance in distribution and variance to real Fontan data. Quantitatively, synthetic data from droppedreal samples had higher similarity scores, as demonstrated by the Kolmogorov–Smirnov statistic, for all butone feature (age at Fontan) compared to synthetic data from imputed real samples, which demonstrateddissimilar scores for three features (Echo SV, Echo tda, and BNP). In addition, synthetic data from droppedreal samples resembled real data to a larger extent (49.3% classiffcation error) than synthetic data fromimputed real samples (65.28% classiffcation error). Classiffcation errors approximating 50% represent datasetsthat are indistinguishable. In terms of utility, synthetic data created from real data in which the missingvalues were imputed classiffed ventricular dysfunction in real data with a classiffcation error of 10.99%.Similarly, utility of the generated synthetic data by showing that a neural network trained on synthetic dataderived from real data in which the missing values were dropped could classify ventricular dysfunction inreal data with a classiffcation error of 9.44%. Conclusions: Although representing a limited subset of thevast data available on the Pediatric Heart Network, generative adversarial networks can create syntheticdata that mimics the probability distribution of real Fontan echocardiographic measures. Clinicians can usethese synthetic data to create models that predict health outcomes for Fontan patients.
文摘Objective:The objective of this study was to compare cardiac transplant operative and postoperative courses of patients with failed Fontan physiology who were initially palliated with a Norwood(FFN)to those without a prior Norwood(FF).Methods:A single-institution retrospective review of all patients with Fontan failure who under-went cardiac transplantation from 2003–2021 was completed-22 underwent prior Norwood(FFN)and 11 did not(FF).Descriptive and inferential statistics were calculated for operative course and patient outcomes.Results:The operative course of the FFN cohort appeared to be more complex(not statistically significant,but clinically relevant)-this group exclusively experienced sternal re-entry events(3 of 22 patients)and concomitant neo-aor-tic reconstruction(6 patients),had a longer duration of surgery(median of 682 min vs.575.5 min),more time on circulatory arrest(median of 25.5 min vs.12.5 min),and more frequent use of open sternal management[50%of patients(11/22)vs.27.3%of patients(3/11)].Postoperatively,these patients underwent more mediastinal explora-tions[other than sternal closure;40.9%of patients(9/22)vs.18.2%of patients(2/11)],spent more time on mechanical ventilation(median of 5 days vs.2 days),had a longer length of stay(median of 30 days vs.19 days),and required more catheter-based re-interventions[22.7%of patients(5/22)vs.9.1%of patients(1/11)].Conclu-sion:Although underpowered,our results suggest that the operative course of FFN patients is more challenging,based mostly on neo-aortic arch issues.In turn,this likely leads to a more complex postoperative course.We are currently collaborating with other institutions to increase the cohort size and power of the study.
