Background: Elevated Fontan pressure (FP) alone cannot fully predict clinical outcomes. We hypothesized thathemodynamic profiling using a cardiac index (CI)-systemic vascular resistance (SVR) plot could characterize c...Background: Elevated Fontan pressure (FP) alone cannot fully predict clinical outcomes. We hypothesized thathemodynamic profiling using a cardiac index (CI)-systemic vascular resistance (SVR) plot could characterize clinicalfeatures and predict the prognosis of post-Fontan patients. Methods: We included post-Fontan patients whounderwent cardiac catheterization at age < 10 years. Patients were classified into four categories: A, CI ≥ 3, SVRindex (SVRI) ≥ 20;B, CI < 3, SVRI ≥ 20;C, CI ≥ 3, SVRI < 20;and D, CI < 3, SVRI < 20. The primary outcome wasfreedom from the combined endpoint: new onset of protein-losing enteropathy or plastic bronchitis, heart transplant,and death. Clinical and hemodynamic variables and freedom from the endpoint were compared betweenthe hemodynamic categories and outcome predictors were evaluated. Results: Eighty-three patients wereincluded. Median follow-up was 5.3 years. Category A/B/C/D consisted of 4/15/53/11 patients, respectively. Allthe patients in category A were New York Heart Association I/II and had a significantly lower pulmonary vascularresistance index (PVRI). Patients in category C had lower pulmonary/systemic blood flow. Patients in category Dhad a higher PVRI and had the poorest freedom from the endpoint (44% at 5 years). Elevated FP and category Dwere outcome predictors. Conclusions: CI-SVR plots was a novel adjunctive method for Fontan hemodynamicprofiling.展开更多
NOWADAYS,Fontan circulation always refers to the hemodynamic status after total cavo-pul-monary connection, which was first created in the 1990s. There are two modern surgeryforms, one creating a channel within the r...NOWADAYS,Fontan circulation always refers to the hemodynamic status after total cavo-pul-monary connection, which was first created in the 1990s. There are two modern surgeryforms, one creating a channel within the right atrium and another making an extra-cardiac channel to connect infe-rior venous cava directly to the right pulmonary artery. Parturients with Fontan circulation are at increased risk of cardiac morbidity and thrombotic complications. We pre-sented a parturient with reduced platelet status of un-known reason undergoing an emergent cesarean delivery.展开更多
<u>Background:</u><span style="font-family:Verdana;"> Extracorporeal</span><span style="font-family:;" "=""> </span><span style="font-fami...<u>Background:</u><span style="font-family:Verdana;"> Extracorporeal</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">membrane oxygenation is a rescue life support technique used in life threatening</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">conditions of refractory respiratory and/or cardiac distress. Indication for extracorporeal life support in children</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">depends on age and varies from pulmonary to cardiac pathologies. In some cases</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> it may be used as a bridge to a</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">therapeutic procedure.</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">We described here the management of respiratory failure due to hemoptysis in a child with a Fontan circulation</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">and veno-venous extracorporeal membrane oxygenation which served as a bridge to angio-embolization. Hemoptysis can be a life threatening condition which can lead to hypovolemic shock and impaired alveolar gas</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">exchange. The latter can result in respiratory failure and consequent asphyxia.</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">When hemoptysis occurs in a patient with a univentricular</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">heart and a Fontan circulation, management of this</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">clinical situation can be challenging due to the particular physiology of the latter. Total cavopulmonary connection is a palliative surgical repair which constitutes Fontan circulation as a definitive treatment in patients with a univentricular heart. </span><u><span style="font-family:Verdana;">Methods:</span></u><span style="font-family:Verdana;"> Case report description of a 16 year-old boy with a univentricular heart and a Fontan circulation</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">who presented hemoptysis managed with a veno-venous extracorporeal membrane oxygenation (ECMO) as a</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">bridge to angio-embolization. </span><u><span style="font-family:Verdana;">Results:</span></u><span style="font-family:Verdana;"> Hemoptysis due to diffuse intra-alveolar hemorrhage from collateral circulation was successfully treated</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">in this young patient with pulmonary vascular embolization. This allowed to wean the patient from</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">extra-corporeal membrane oxygenation. </span><u><span style="font-family:Verdana;">Conclusion:</span></u><span style="font-family:Verdana;"> Veno-venous ECMO can be life-saving as a bridge to angio-embolization for severe hemoptysis in</span></span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">patients with Fontan circulation. The reported case allows to underline that </span><span style="font-family:Verdana;">our multidisciplinary approach in</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">this complex pediatric patient surely</span><span style="font-family:Verdana;"> participated to improve outcome.</span>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
Several guidelines have indicated that liver stiffness(LS)assessed by means of shear wave elastography(SWE)can safely replace liver biopsy in several clinical scenarios,particularly in patients with chronic viral hepa...Several guidelines have indicated that liver stiffness(LS)assessed by means of shear wave elastography(SWE)can safely replace liver biopsy in several clinical scenarios,particularly in patients with chronic viral hepatitis.However,an increase of LS may be due to some other clinical conditions not related to fibrosis,such as liver inflammation,acute hepatitis,obstructive cholestasis,liver congestion,infiltrative liver diseases.This review analyzes the role that SWE can play in cases of liver congestion due to right-sided heart failure,congenital heart diseases or valvular diseases.In patients with heart failure LS seems directly influenced by central venous pressure and can be used as a prognostic marker to predict cardiac events.The potential role of LS in evaluating liver disease beyond the stage of liver fibrosis has been investigated also in the hepatic sinusoidal obstruction syndrome(SOS)and in the Budd-Chiari syndrome.In the hepatic SOS,an increase of LS is observed some days before the clinical manifestations;therefore,it could allow an early diagnosis to timely start an effective treatment.Moreover,it has been reported that patients that were successfully treated showed a LS decrease,that reached pre-transplantation value within two to four weeks.It has been reported that,in patients with Budd-Chiari syndrome,LS values can be used to monitor short and long-term outcome after angioplasty.展开更多
Introduction:While previous studies only focused on the arrhythmic risk associated with specific correction strategies,this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-...Introduction:While previous studies only focused on the arrhythmic risk associated with specific correction strategies,this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair.Methods:In this single centre observational cohort study,we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment,patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes.Surgical history and clinical outcomes were reviewed.Results:86 patients were included in the study(54 male[62.8%],mean age 16.4±6.1 years),with median follow-up from definitive repair of 12.8 years(6.4–18.9 years).They underwent three different final repairs:23 patients(26.7%)univentricular palliation,43(50%)biventricular correction,and 20(23.3%)one and a half ventricle correction.Thirteen patients(15%)developed arrhythmia:6 patients(all the subgroups)sinus node disfunction(SND);2(biventricular repair)premature ventricular complexes;2(one and a half ventricle repair)non-sustained ventricular tachycardia;1(biventricular repair)intra-atrial re-entrant tachycardia;1(one and a half ventricle repair)supraventricular tachyarrhythmia;1(biventricular repair)atrial fibrillation.Three patients with SND needed a pacemaker implantation.Only Fontan circulation showed an association with SND,while the other two groups heterogeneous types of arrhythmias.Conclusions:The low arrhythmic risk is related to surgical repair,it does not appear to be associated with native cardiomyopathy,and it appears to increase with length of follow up.Continuous follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.展开更多
文摘Background: Elevated Fontan pressure (FP) alone cannot fully predict clinical outcomes. We hypothesized thathemodynamic profiling using a cardiac index (CI)-systemic vascular resistance (SVR) plot could characterize clinicalfeatures and predict the prognosis of post-Fontan patients. Methods: We included post-Fontan patients whounderwent cardiac catheterization at age < 10 years. Patients were classified into four categories: A, CI ≥ 3, SVRindex (SVRI) ≥ 20;B, CI < 3, SVRI ≥ 20;C, CI ≥ 3, SVRI < 20;and D, CI < 3, SVRI < 20. The primary outcome wasfreedom from the combined endpoint: new onset of protein-losing enteropathy or plastic bronchitis, heart transplant,and death. Clinical and hemodynamic variables and freedom from the endpoint were compared betweenthe hemodynamic categories and outcome predictors were evaluated. Results: Eighty-three patients wereincluded. Median follow-up was 5.3 years. Category A/B/C/D consisted of 4/15/53/11 patients, respectively. Allthe patients in category A were New York Heart Association I/II and had a significantly lower pulmonary vascularresistance index (PVRI). Patients in category C had lower pulmonary/systemic blood flow. Patients in category Dhad a higher PVRI and had the poorest freedom from the endpoint (44% at 5 years). Elevated FP and category Dwere outcome predictors. Conclusions: CI-SVR plots was a novel adjunctive method for Fontan hemodynamicprofiling.
文摘NOWADAYS,Fontan circulation always refers to the hemodynamic status after total cavo-pul-monary connection, which was first created in the 1990s. There are two modern surgeryforms, one creating a channel within the right atrium and another making an extra-cardiac channel to connect infe-rior venous cava directly to the right pulmonary artery. Parturients with Fontan circulation are at increased risk of cardiac morbidity and thrombotic complications. We pre-sented a parturient with reduced platelet status of un-known reason undergoing an emergent cesarean delivery.
文摘<u>Background:</u><span style="font-family:Verdana;"> Extracorporeal</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">membrane oxygenation is a rescue life support technique used in life threatening</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">conditions of refractory respiratory and/or cardiac distress. Indication for extracorporeal life support in children</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">depends on age and varies from pulmonary to cardiac pathologies. In some cases</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> it may be used as a bridge to a</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">therapeutic procedure.</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">We described here the management of respiratory failure due to hemoptysis in a child with a Fontan circulation</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">and veno-venous extracorporeal membrane oxygenation which served as a bridge to angio-embolization. Hemoptysis can be a life threatening condition which can lead to hypovolemic shock and impaired alveolar gas</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">exchange. The latter can result in respiratory failure and consequent asphyxia.</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">When hemoptysis occurs in a patient with a univentricular</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">heart and a Fontan circulation, management of this</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">clinical situation can be challenging due to the particular physiology of the latter. Total cavopulmonary connection is a palliative surgical repair which constitutes Fontan circulation as a definitive treatment in patients with a univentricular heart. </span><u><span style="font-family:Verdana;">Methods:</span></u><span style="font-family:Verdana;"> Case report description of a 16 year-old boy with a univentricular heart and a Fontan circulation</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">who presented hemoptysis managed with a veno-venous extracorporeal membrane oxygenation (ECMO) as a</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">bridge to angio-embolization. </span><u><span style="font-family:Verdana;">Results:</span></u><span style="font-family:Verdana;"> Hemoptysis due to diffuse intra-alveolar hemorrhage from collateral circulation was successfully treated</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">in this young patient with pulmonary vascular embolization. This allowed to wean the patient from</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">extra-corporeal membrane oxygenation. </span><u><span style="font-family:Verdana;">Conclusion:</span></u><span style="font-family:Verdana;"> Veno-venous ECMO can be life-saving as a bridge to angio-embolization for severe hemoptysis in</span></span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">patients with Fontan circulation. The reported case allows to underline that </span><span style="font-family:Verdana;">our multidisciplinary approach in</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">this complex pediatric patient surely</span><span style="font-family:Verdana;"> participated to improve outcome.</span>
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Several guidelines have indicated that liver stiffness(LS)assessed by means of shear wave elastography(SWE)can safely replace liver biopsy in several clinical scenarios,particularly in patients with chronic viral hepatitis.However,an increase of LS may be due to some other clinical conditions not related to fibrosis,such as liver inflammation,acute hepatitis,obstructive cholestasis,liver congestion,infiltrative liver diseases.This review analyzes the role that SWE can play in cases of liver congestion due to right-sided heart failure,congenital heart diseases or valvular diseases.In patients with heart failure LS seems directly influenced by central venous pressure and can be used as a prognostic marker to predict cardiac events.The potential role of LS in evaluating liver disease beyond the stage of liver fibrosis has been investigated also in the hepatic sinusoidal obstruction syndrome(SOS)and in the Budd-Chiari syndrome.In the hepatic SOS,an increase of LS is observed some days before the clinical manifestations;therefore,it could allow an early diagnosis to timely start an effective treatment.Moreover,it has been reported that patients that were successfully treated showed a LS decrease,that reached pre-transplantation value within two to four weeks.It has been reported that,in patients with Budd-Chiari syndrome,LS values can be used to monitor short and long-term outcome after angioplasty.
文摘Introduction:While previous studies only focused on the arrhythmic risk associated with specific correction strategies,this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair.Methods:In this single centre observational cohort study,we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment,patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes.Surgical history and clinical outcomes were reviewed.Results:86 patients were included in the study(54 male[62.8%],mean age 16.4±6.1 years),with median follow-up from definitive repair of 12.8 years(6.4–18.9 years).They underwent three different final repairs:23 patients(26.7%)univentricular palliation,43(50%)biventricular correction,and 20(23.3%)one and a half ventricle correction.Thirteen patients(15%)developed arrhythmia:6 patients(all the subgroups)sinus node disfunction(SND);2(biventricular repair)premature ventricular complexes;2(one and a half ventricle repair)non-sustained ventricular tachycardia;1(biventricular repair)intra-atrial re-entrant tachycardia;1(one and a half ventricle repair)supraventricular tachyarrhythmia;1(biventricular repair)atrial fibrillation.Three patients with SND needed a pacemaker implantation.Only Fontan circulation showed an association with SND,while the other two groups heterogeneous types of arrhythmias.Conclusions:The low arrhythmic risk is related to surgical repair,it does not appear to be associated with native cardiomyopathy,and it appears to increase with length of follow up.Continuous follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.
