Cardiopatia univentricular

Autologous Induced Pluripotent Stem Cells of Cardiac Lineage for Congenital Heart Disease

NCT05647213

Extracellular Vesicle Micro RNA Profiling in Congenital Heart Disease: Fetal-Maternal Regulation in Neonatal Thrombosis

NCT06434207

Adult Congenital Heart Disease International EValuation of the Effectiveness of SGLT2i Registry

NCT06932081

PK/PD of Digoxin in Infants With SVHD

Fase II

NCT06613477

Venous Compression in Fontan

NCT07220226

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NCT07220226 · Venous Compression in FontanEm breve

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#1

Oncogenic Signaling Activation and Potential Biomarkers in Congestive Hepatopathy Revealed by Proteomic Analysis.

Hepatology research : the official journal of the Japan Society of Hepatology2026 Jan 29

Fontan surgery improves outcomes in univentricular heart disease; however, some patients develop severe Fontan-associated liver disease (FALD). Even during adolescence, hepatocellular carcinoma occurs in some patients with FALD, although the mechanism remains unclear. In this study, we conducted proteomic analyses of liver tissues obtained using laser capture microdissection (LCM) and serum samples to investigate FALD-related peripheral liver oncogenic factors and explore potential biomarkers. Ten-week-old mice underwent sham surgery, and age-matched mice underwent partial ligation of the suprahepatic inferior vena cava as a congestive hepatopathy (CH) model. Control liver (CL) and serum were collected from the control models, whereas peripheral congestive liver (PCL) and serum were obtained from the CH models. LCM-isolated liver and serum samples were subjected to qualitative and quantitative proteomic analyses. Differentially expressed proteins (DEPs) were identified by mass spectrometry. We identified 3904 hepatic proteins and 2947 serum proteins. Cancer-related proteins were upregulated in PCL, whereas hepatoprotective proteins were reduced compared with those in CL. Enrichment analysis revealed the upregulation of oncogenic signaling pathways in PCL. Twenty DEPs (e.g., transforming growth factor-beta-induced protein ig-h3, complement C1q subcomponent subunit A, and Dickkopf-related protein 3) were concurrently increased in PCL and serum of the CH models. PCL upregulated oncogenic proteins and activated oncogenic signaling pathways. DEPs detectable in the liver and serum indicate potential FALD biomarkers. These findings offer insights into the pathophysiology of FALD and hepatocarcinogenesis and support the development of novel diagnostic and therapeutic strategies.

#2

Association of Pre-Fontan Hemodynamics With Long-Term Outcomes After Fontan Palliation: A Study From the Pediatric Cardiac Care Consortium.

Circulation2026 Feb 10

Long-term outcomes after Fontan vary widely. Although pre-Fontan hemodynamics predict early failure, their association with long-term outcomes remains unclear. We hypothesized that pre-Fontan hemodynamics predict long-term risk of death or transplantation. We analyzed data from the Pediatric Cardiac Care Consortium, a US-based multicenter registry including patients undergoing first-time Fontan with pre-Fontan catheterization and long-term follow-up. Patients undergoing a Fontan procedure before 18 years of age at any time between 1982 and 2011 were included in the study. Outcomes of interest were in-hospital Fontan failure (death or takedown) and postdischarge death or transplantation, identified through matching with the National Death Index and the Organ Procurement and Transplantation Network through 2022. Associations between pre-Fontan hemodynamics and long-term risk for death or transplantation were assessed with Kaplan-Meier survival curves and extended Cox regression. Among 1175 patients (736 [62.6%] male, 626 [53.3%] with systemic left ventricle), 1111 were discharged with Fontan physiology. Over a median postdischarge follow-up of 20.6 years (interquartile range, 18.2-24.4 years), 85 deaths and 49 transplantations occurred. Pre-Fontan mean pulmonary arterial pressure was the strongest hemodynamic predictor of postdischarge outcomes with a continuous association and no clear inflection point; 25-year transplantation-free survival declined from 83.7% (95% CI, 77.6-88.3) in the low mean pulmonary arterial pressure tertile to 73.7% (95% CI, 65.5-80.3) in the highest tertile (log-rank P=0.02). Each 1-SD increase in mean pulmonary arterial pressure was associated with 1.33-fold higher odds of in-hospital failure (adjusted odds ratio, 1.33 [95% CI, 1.00-1.77]; P=0.05) and a 2.2-fold higher hazard of death or transplantation (adjusted hazard ratio, 2.20 [95% CI, 1.62-3.00]; P<0.01) estimated at discharge. This hazard declined 3% per year after discharge (adjusted hazard ratio per year, 0.97 [95% CI, 0.95-0.99]; P<0.01) and resolved by 17 years in patients with systemic right ventricle and by 23 years in those with systemic left ventricle. Additional independent risk factors included systemic right ventricle versus systemic left ventricle (adjusted hazard ratio, 2.39 [95% CI, 1.65-3.46]; P<0.01) and delayed Fontan completion (>4 years of age versus 2 to 4 years of age; adjusted hazard ratio, 1.80 [95% CI, 1.25-2.60]; P=0.02). Elevated pre-Fontan mean pulmonary arterial pressure is a strong predictor of in-hospital and long-term post-Fontan risk of death or transplantation. Systemic right ventricle and delayed Fontan completion (>4 years of age) further increased risk. These findings support early Fontan consideration and ongoing hemodynamic surveillance to optimize long-term outcomes.

#3

Maternal-Fetal Environment and Neurodevelopment in Patients with Single Ventricle Heart Disease.

The Journal of pediatrics2026 Mar

To assess the role of the maternal-fetal environment (MFE) on early neurodevelopmental and clinical outcomes in children with single ventricle heart disease (SVHD). We performed a retrospective, single-center study of patients with a prenatal diagnosis of SVHD from 2011-2022. Impaired MFE was defined as exposure to diabetes, pre-eclampsia, tobacco, or chronic or gestational hypertension in utero. Neurodevelopmental outcomes were assessed using the Bayley Scales of Infant and Toddler Development-III and 4 between 12 and 36 months of age. Among 160 patients with SVHD (117 hypoplastic left heart and 43 hypoplastic right heart), 78 completed neurodevelopmental testing. Exposure to an impaired MFE was associated with lower cognitive scores (P = .030), although this association became nonsignificant after adjustment for clinical covariates. Impaired MFE was also associated with lower language and receptive communication scores after clinical adjustment (P = .023 and P = .040, respectively) but became nonsignificant after further adjustment for the Child Opportunity Index. No significant differences were observed in fine or gross motor scores, neonatal complications, hospital or intensive care unit stay, and 1-year mortality or transplantation between groups. Among patients with SVHD, exposure to an impaired MFE was associated with lower cognitive and language outcomes, although these associations were attenuated after accounting for clinical and socioeconomic factors. These findings highlight the importance of early identification and monitoring of children with adverse prenatal exposures.

#4

Early outcomes of children with univentricular circulation undergoing Fontan surgery: the EuroFontan registry.

European heart journal2026 Feb 18

The Fontan operation and its modifications have transformed the management of children with functional single ventricle physiology. While outcomes have improved, peri-operative complications remain common. This study aimed to assess early outcomes and complications following Fontan completion in a large international European cohort. The EuroFontan registry is a multicentre retrospective study involving 21 congenital heart disease and transplant centres across Europe. Data were collected on patients who underwent Fontan surgery from January 1990 to January 2023. Analyses focused on the most recent 15-year period. A composite early adverse outcome (death within 30 days or in-hospital, low cardiac output syndrome, stroke, or Fontan takedown) was used. Predictors were assessed using multivariable mixed-effects logistic regression, with centre included as a random effect. Missing data were handled using multiple imputation with Rubin's rules, and sensitivity analyses were performed to assess robustness. Of 3510 patients, this analysis focused on 2075 individuals from the past 15 years, with a median age of 4.2 [interquartile range 3.2-6.2] years at Fontan completion. Early mortality was 1.0% (95% confidence interval .66%-1.54%). The composite adverse outcome occurred in 5.1% (95% confidence interval 4.2%-6.1%). On multivariable analysis, morphology other than tricuspid atresia was the only significant predictor of the composite outcome (odds ratio 2.2, 95% confidence interval 1.21-3.99, P = .01). Fontan surgery has evolved and, in contemporary practice, has low peri-operative morbidity and mortality. Morphology other than tricuspid atresia was associated with increased risk of early adverse outcomes, highlighting the importance of pre-operative risk stratification.

#5

Effects of ACE inhibitor and afterload reduction in single ventricles following bidirectional Glenn.

Open heart2026 Mar 24

Patients with single ventricles after bidirectional Glenn (BDG) are placed on ACE inhibitors (ACEIs) to reduce afterload and to decrease systemic to pulmonary collateral flow (SPC). The objective is to determine if ACEI use in BDG patients improves SPC and ventricular function. Single-centre retrospective study of BDG patients who underwent cardiac magnetic resonance (CMR) from 2010 to 2024. Demographics, medication, ventricular function and flow data were collected. Patients included were >1 year old and on ACEI for >3 months prior to CMR. A total of 320 BDG patients were studied, 137 were treated with ACEI with no difference in age, sex, medications (except for ACEI) or body surface area. SPC as a percentage of aortic flow was not different in the ACEI group, whether right (RV) or left ventricle (LV) dominant. Ejection fraction (EF) was lower and end- diastolic volume (EDV) was higher in BDG on ACEI for the entire group and for RV dominant. Although LV EDV was higher with those on ACEI, there was no difference in EF. There was no difference in ventricular dilation or EF on echocardiogram prior to starting drug. SPC positively correlated with EDV. SVC flow was lower for those on ACEI. BDG patients treated with ACEI did not demonstrate decreased SPC. There was a suggestion of no improvement of ventricular performance. Cerebral blood flow is decreased in BDG on ACEI using SVC flow. Findings suggest that routine ACEI use in single ventricle patients post BDG should be considered with caution and age-appropriate and clear indications along with measurable therapeutic targets should be considered.

Publicações recentes

Long-Term Outcomes of Antithrombotic Treatment in a Cohort of 864 Patients With Fontan Circulation.

J Am Heart Assoc2026 Apr 16

The Benefits of an Intracardiac Conduit in the Fontan Operation for Complex Systemic Venous Anomalies in Patients with Single Ventricle Physiology.

World J Pediatr Congenit Heart Surg2026 Apr 15

Biological tissue engineering for Fontan failure using decellularized extracellular matrix: a systematic review.

J Transl Med2026 Apr 13

Outcomes of atrioventricular valve interventions in patients with univentricular heart.

Arch Cardiol Mex2026 Apr 6

Univentricular heart with anomalous venous drainage and pulmonary sling.

Rev Esp Cardiol (Engl Ed)2026 Apr 3

Ver todas no PubMed

📚 EuropePMCmostrando 199

Effects of ACE inhibitor and afterload reduction in single ventricles following bidirectional Glenn.

Open heart

European heart journal. Imaging methods and practice

15O-water PET perfusion in complex congenital heart disease.

CJC pediatric and congenital heart disease

Outcomes of Cardiac Repair in Heterotaxy Syndrome Patients-Tertiary Center Experience.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery

Cardiac Resynchronization Therapy for Failing Single Ventricle Physiology.

The Role of Serial Fetal Echocardiography in Postnatal Surgical Decision-Making for Borderline Left Ventricle: A Case Report.

Pediatric reports

Critical care explorations

Prediction of Adverse Events in Single Ventricle Physiology Infants Using Artificial Intelligence Tools.

Journal of cardiopulmonary rehabilitation and prevention

Pulmonary Restriction in Patients With a Fontan Circulation: DETERMINANTS, DETERIORATION OVER TIME, AND CLINICAL IMPLICATIONS.

Multimedia manual of cardiothoracic surgery : MMCTS

Use of right atrial wall to repair severely dysplastic tricuspid valve in an infant with Ebstein's anomaly.

American journal of physiology. Heart and circulatory physiology

Completion of Fontan circulation does not affect the low longitudinal contribution to stroke volume in patients with single ventricles.

Hepatology research : the official journal of the Japan Society of Hepatology

Oncogenic Signaling Activation and Potential Biomarkers in Congestive Hepatopathy Revealed by Proteomic Analysis.

Genetic Burden in Congenital Anomalies of the Mitral and Tricuspid Valves: A Case-Control Study.

Association of Pre-Fontan Hemodynamics With Long-Term Outcomes After Fontan Palliation: A Study From the Pediatric Cardiac Care Consortium.

Circulation

Imperforate tricuspid valve with severe hypoplasia associated with an atypical vascular ring in the set of left isomerism. First case report.

International journal for numerical methods in biomedical engineering

A One-Dimensional (1D) Computational Fluid Dynamics Study of Fontan-Associated Liver Disease (FALD).

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions

Transparavertebral Occlusion of a Large Venovenous Collateral in a Fontan Patient.

Archives of disease in childhood

Postoperative complications after paediatric cardiac surgery: the role of ethnicity and deprivation - a national cohort study.

The Journal of pediatrics

Maternal-Fetal Environment and Neurodevelopment in Patients with Single Ventricle Heart Disease.

Growth and neurodevelopment at 18-22 months in term infants with CHD undergoing cardiopulmonary bypass surgery: a retrospective cohort study.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery

Contemporary Real-world Outcomes of Stage 1 Palliation for Single-ventricle Patients: A Kids' Inpatient Database Analysis.

Percutaneous Fontan by inferior vena cava banding and subsequent creation of an intraatrial stent-conduit: a clinical renaissance of a less invasive approach?

Anesthetic management of an adult female with uncorrected univentricular heart and comorbid ectopic pregnancy for unilateral salpingectomy: a case report.

BMC anesthesiology

Archives of disease in childhood

Cause of death in children with heart disease: a cohort study.

Double inlet left ventricle in a 12-year-old European bison: a case report with anatomical assessment and postmortem computed tomography evaluation.

BMC veterinary research

Childhood Neurocognitive Outcomes in Patients With Fontan Circulation.

Journal of electrocardiology

An uncommon ECG presentation of post-Fontan patient: ECG challenge.

Innovations (Philadelphia, Pa.)

Textbook Outcome for the Fontan Operation: A Holistic Quality Metric in Congenital Heart Surgery.

Expanding the use of 3D printing in congenital heart surgery.

Translational pediatrics

Early outcomes of children with univentricular circulation undergoing Fontan surgery: the EuroFontan registry.

European heart journal

Contemporary outcomes and health care costs associated with single-ventricle heart failure admissions in adults in the United States.

Risk Factors for Reintervention Following Treatment for Aortic Coarctation With or Without Aortic Arch Hypoplasia in Sweden: From Isolated Coarctation to Complex Bi- and Univentricular Congenital Heart Disease.

A case of coronary sinus ostium atresia with muscular obstruction in a complex univentricular heart managed in stages.

The international journal of cardiovascular imaging

Beyond volumes and ejection fraction: practical insights into cine-CMR interpretation and applications.

World journal for pediatric & congenital heart surgery

A Learning Curve is Associated With Combined Hybrid Procedure and Single Ventricle-Ventricular Assist Device Insertion in Neonates With Hypoplastic Left Heart Syndrome.

Echocardiography (Mount Kisco, N.Y.)

Association of Global Longitudinal Strain and Long-Term Transplant-Free Survival in Fontan Patients.

A randomised clinical trial of regional cerebral perfusion versus deep hypothermic circulatory arrest: five-year and ten-year follow-up for neurodevelopmental outcomes in children with functional single ventricle.

Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology

20 years of experience with the Fontan procedure: Risk factors for adverse outcomes.

Association Between Initial Pulmonary Artery Band Circumference and Early and Late Outcomes in Single-Ventricle Patients.

Pathophysiology of endothelial dysfunction in Fontan circulation: from bench to bedside and back again.

Angiogenesis

Pediatric transplantation

Comprehensive Analysis of the First 35 Years of Pediatric Heart Transplantation in Sweden.

100 Episodes of Support With Pulsatile Paracorporeal Ventricular Assist Device in 99 Neonates, Infants, and Children At a Single Institution.

Multimedia manual of cardiothoracic surgery : MMCTS

Isolated subpulmonary ventricular assist device support for failing Fontan circulation in hypoplastic left heart syndrome.

The importance of evaluating anatomy below the diaphragm in staged single-ventricle patients presenting with cyanosis.

The Ultrasound and Genetic Characteristics of Fetuses With Laterality Defects-A Prenatal Cohort in Asian Population.

Prenatal diagnosis

Cardiovascular diagnosis and therapy

Left ventricular rotational abnormalities in adult patients with congenital heart disease late after Fontan procedure: detailed analysis from the CSONGRAD Registry and MAGYAR-Path Study.

Cardiac Transplantation After HYBRID+VAD Support in Patients With HLHS: Technical Considerations and Outcomes.

Ductus arteriosus obstruction induced by vertical vein stenting in single-ventricle physiology with supracardiac total anomalous pulmonary venous connection.

Requirements for biventricular repair after bilateral pulmonary artery banding for patients with borderline left ventricle.

JTCVS open

Clinical implications of major haemolysis in children supported by a pulsatile ventricular assist device.

Surgical Ventricular Septal Defect Repair.

JACC. Case reports

European journal of pediatrics

Reference models for individualized assessment of cardiorespiratory fitness in children and adolescents with congenital heart disease: a retrospective multicentre study.

Thirty-Year Prevalence of Spontaneous Hemoptysis in Palliated Single Ventricle Circulation.

World journal for pediatric & congenital heart surgery

Analysis of 112 Patients With Congenital Heart Disease Undergoing Cardiac Transplantation: Impact of Pretransplant Ventricular Assist Device.

World journal for pediatric & congenital heart surgery

A Single-Center Experience With En-Bloc Combined Heart+Liver Transplantation: Analysis of Eight Cases, Including Two Heart+Liver+Kidney Transplants.

Surgical, Hybrid, or Delayed Surgical Stage 1: Single-Ventricle Palliation Pathway Utilization and Outcome Trends.

Interdisciplinary cardiovascular and thoracic surgery

Lasso technique for bilateral pulmonary arterial banding.

Impact of Systemic Ventricular Morphology on Age-Adjusted (zlog-)NT-proBNP in Children with Univentricular Hearts.

Systolic performance of the single ventricle, exercise capacity, and endothelial function in pediatric Fontan patients.

American heart journal

Shifting strategies to address systemic outflow tract obstruction in single ventricle anatomy with transposed great arteries.

World journal for pediatric & congenital heart surgery

Transposition of the Great Arteries With Intact Ventricular Septum and Left Ventricular Outflow Tract Obstruction: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD).

30-Year Survival After the Fontan Operation in Denmark.

Placental weight in pregnancies complicated by fetal congenital heart defects.

Placenta

Systemic Changes in Adults With a Fontan Circulation: Insights From the Plasma Proteome.

European heart journal. Cardiovascular Imaging

Intraventricular pressure difference estimation based on blood speckle tracking-invasive validation and early clinical application.

100 Episodes of Support With Berlin Heart: Comparison of Patients Who Underwent Ventricular Assist Device Explantation After Cardiac Recovery With Those Without Cardiac Recovery.

The Use of Pulmonary Vasodilators in Pediatric Patients with Single-Ventricle Palliation: A Ten-Year Experience in a Tertiary Care Center.

European journal of radiology

Peripheral vs. central parametric mapping of the liver in single ventricle patients: Measurement location matters.

Paracorporeal continuous-flow ventricular assist devices (cfVADs) for children less than 20 kg: Challenging the paradigm in the smallest patients.

Medical engineering & physics

A multiparameter comparative approach to the three-dimensional numerical analysis of the hemodynamics of total cavopulmonary connection.

Trends in cardiovascular medicine

Cardiorespiratory fitness in children with surgically corrected congenital heart disease: A meta-analysis and meta-regression.

Contemporary Outcomes for Superior Cavopulmonary Connection in Single Ventricle Palliation: Is There Room for Improvement?

Transthoracic intracardiac line use and complications in the paediatric single ventricle population.

Seminars in thoracic and cardiovascular surgery

When to Opt for Univentricular versus Biventricular Repair in Complex Congenitally Corrected Transposition of the Great Arteries.

Are We Getting Better?: Ongoing Challenges of Atrioventricular Valve Repair in Children With Single Ventricle Physiology.

The Journal of pediatrics

Outcome Disparities Between Rural and Urban Infants with Single-Ventricle Heart Disease.

Discordances in Kinetic Energy Between the Superior Cavopulmonary Connection and Single Ventricle Are Associated With Suboptimal Fontan Outcomes: A Pre-Fontan 4-Dimensional Flow Study.

International heart journal

Venous Pressure Cost for Cardiac Index Increases During Exercise in Patients with Fontan Circulation Versus Those with Biventricular Circulation.

Lactescent urine following pediatric cardiac surgery due to catheter migration.

BMC pediatrics

Pathophysiology and management of adults with complex congenital heart disease after biventricular repair.

Journal of cardiology

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery

Mid-term outcomes of classical hypoplastic left heart syndrome after Fontan procedure.

Extracorporeal membrane oxygenation: a bridge to palliation in single ventricle physiology.

Interdisciplinary cardiovascular and thoracic surgery

Relationship of pulmonary artery size and venovenous collaterals during staged single ventricle reconstruction and their impact on outcomes after Fontan procedure.

The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians

Pregnancy in ACHD women: crucial role of multidisciplinary clinical roadmap.

A cohort evaluation of surgical pathways reported to a national audit of children undergoing treatment for congenital heart disease in England and Wales.

Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia

Impact of Pulsatile Bidirectional Cavopulmonary Shunt on Pre-Fontan Hemodynamics in Single Ventricle Physiology: A Meta-Analysis Reveals Favorable Outcomes.

Sodium-glucose cotransporter-2 inhibitor use in patients with a Fontan circulation.

Coronary anomalies in single ventricles: Insights from selective angiographic assessment.

Journal of cardiology

Peripandemic outcomes of infants treated for sentinel congenital heart diseases in England and Wales.

Open heart

Journal of veterinary cardiology : the official journal of the European Society of Veterinary Cardiology

Multimodal imaging of congenital double-inlet left ventricle with hypoplastic right ventricle and ventricular septal defect in a young cat.

Multimedia manual of cardiothoracic surgery : MMCTS

Staged biventricular repair in aortic hypoplasia: the value of intraoperative myocardial perfusion.

The Canadian journal of cardiology

Right Ventricular Electrophysiology and Arrhythmias in Adults With Congenital Heart Disease: Scientific Basis for Management.

Echocardiography (Mount Kisco, N.Y.)

Multimodality Imaging of Bilateral Ductus Arteriosus in a Patient With Complex Heterotaxy and a Univentricular Heart.

Neurodevelopmental Outcomes After Nitric Oxide During Cardiopulmonary Bypass for Open Heart Surgery: A Randomized Clinical Trial.

JAMA network open

MiR-25-3p regulates pulmonary arteriovenous malformation after Glenn procedure in patients with univentricular heart via the PHLPP2-HIF-1α axis.

Scientific reports

Incidence and Prognosis of Surgical Heart Block in Patients with L-transposition of the Great Arteries.

Multimedia manual of cardiothoracic surgery : MMCTS

Berlin Heart EXCOR sVAD implantation technique for neonates and infants with functionally univentricular ductal-dependent systemic circulation.

Blood Biomarkers as a Non-Invasive Method for the Assessment of the State of the Fontan Circulation.

Torn spiral patch repair of ventricular septation for single ventricle associated with D-malposition of the great arteries after 40 years.

The Canadian journal of cardiology

Right Ventricular Remodeling in Complex Congenital Heart Disease.

Echocardiographic predictors of surgical outcomes in pulmonary atresia with intact ventricular septum and development of a discriminatory calculator: an Australian tertiary institution experience.

Extracorporeal cardiopulmonary resuscitation outcomes in pre-Glenn single ventricle infants: Analysis of a ten-year dataset.

Resuscitation

ASAIO journal (American Society for Artificial Internal Organs : 1992)

Anatomical Aspects and Long-Term Outcomes of Additional Surgical Repair During Heart Transplantation in Adult Congenital Heart Disease.

Journal of the American College of Cardiology

Outcomes for Children With Congenital Heart Disease Undergoing Ventricular Assist Device Implantation: An ACTION Registry Analysis.

Cardiovascular engineering and technology

Pediatric Cardiovascular Multiscale Modeling using a Functional Mock-up Interface.

Printed Models for Better Prediction of Surgery in Patients with Double Outlet Right Ventricle.

The Journal of physiology

Enhanced myocardial perfusion in late gestation fetal lambs with impaired left ventricular inflow.

Cardiovascular therapeutics

Transcatheter Embolization in Congenital Cardiovascular Malformations-Variable Use of Vascular Plugs.

Ages and Stages Questionnaires in the assessment of young children after cardiac surgery.

Echocardiography (Mount Kisco, N.Y.)

Navigating the Spectrum of Double-İnlet Left Ventricle Presentations: Contemporary Retrospective Cohort Study.

Albuminuria Prevalence in Fontan Patients: A Systematic Review and Meta-Analysis.

Deterioration in Renal Function in Patients With a Fontan Circulation and Association With Mortality.

JACC. Advances

Feasibility, classification and potential clinical impact of non-invasive delineation of abdominal lymphatic vessels in patients following TCPC with T2 weighted MRI.

Scientific reports

Cardiovascular Outcomes Associated With Hypoplastic Left Heart Syndrome Versus Other Types of Single Right Ventricle: A Multicenter Study.

Medicina (Kaunas, Lithuania)

Cardiac Rehabilitation for Fontan Circulation Patients: A Systematic Review, and Meta-Analysis.

Hearing impairment following surgically repaired congenital heart disease in children: a prospective study.

BMJ paediatrics open

World journal for pediatric & congenital heart surgery

Management of Tricuspid Atresia With Normally Related Great Arteries and Left-Sided Obstruction.

Navigating life with single-ventricle CHD: psychosocial needs across adulthood.

Mountains and Waves: Fontan Circulation in Different Environmental Conditions.

Clinical anatomy (New York, N.Y.)

Relating normal human cardiac development to the anatomical findings in the congenitally malformed heart.

Eight-Year Outcomes of Cardiosphere-Derived Cells in Single Ventricle Congenital Heart Disease.

Turk gogus kalp damar cerrahisi dergisi

Mid-term results and late events after the Fontan operation: A single-center experience.

Cardiovascular diagnosis and therapy

Pulmonary hypertension in adults with congenital heart defects (ACHDs) in light of the 2022 ESC PAH guidelines-part II: supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management (shunt lesion, left ventricular disease, univentricular hearts), interventions, intensive care, ACHD follow-up, future perspective.

Cardiovascular diagnosis and therapy

Pulmonary hypertension in adults with congenital heart defects (ACHDs)-in light of the 2022 ESC PAH guidelines-part I: definition, epidemiology, classification, diagnostics, genetics, risk stratification and follow-up, gender aspects.

The international journal of cardiovascular imaging

Principal component analysis identified neo-aortic diameter variations post Norwood surgery associated with the single ventricle performance and flow quality.

Evaluating Long-Term Outcomes of Children Undergoing Surgical Treatment for Congenital Heart Disease for National Audit in England and Wales.

Magnetic resonance lymphangiography abnormalities as extracardiac biomarkers of pulmonary hypertension and functional phenotype in Fontan-Kreutzer univentricular circulation.

Asian cardiovascular & thoracic annals

Outcomes of fenestration versus none in extracardiac total cavopulmonary connection.

The impact of viral respiratory infection on surgical outcome of cavopulmonary shunt.

Making it work for all: equity in single ventricle monitoring.

BMC cardiovascular disorders

Comparative outcomes of cardioplegic arrest versus beating heart in pediatric undergoing extracardiac total cavopulmonary connection.

Usefulness of percutaneous transluminal coronary balloon angioplasty for coronary artery stenosis after surgery for CHD.

Comparison of pulmonary artery growth between ductus stent and systemic-to-pulmonary shunt as single-ventricle palliation.

Psychosocial burden and quality of life of parents with children with univentricular hearts compared to ASD parents and parents of heart-healthy children.

Postoperative Outcomes of Fontan Operation in a Multicenter Italian Study. How Far Have We Gone? Early Outcomes After Fontan Operation.

Single ventricle palliation in congenitally corrected transposition of the great arteries: An international multicenter study.

Predictors of spontaneous pregnancy loss in single ventricle physiology.

Open heart

Non-invasive assessment of severe liver fibrosis in patients with Fontan-associated liver disease: The VALDIG-EASL FONLIVER cohort.

Journal of hepatology

Survival of Children With Critical Congenital Heart Defects in the National Birth Defects Prevention Study.

Birth defects research

Current opinion in pediatrics

Diastolic dysfunction: assessment and implications on the single ventricle circulation.

World journal for pediatric & congenital heart surgery

Impact of Atrioventricular Valve Intervention at Each Stage of Single Ventricle Palliation.

The Journal of innovations in cardiac rhythm management

Leadless Pacemaker Implantation in Fontan Patients with Multimodality Imaging: Tips and Tricks.

Low Ventricular Stiffness Is Associated With Suboptimal Outcomes in Patients With a Single Right Ventricle After the Fontan Operation: A Novel Phenotype.

Outcomes of Neonatal Cardiac Surgery: A European Congenital Heart Surgeons Association Study.

The Thoracic and cardiovascular surgeon

Fontan Completion in Adult Patients with Functionally Univentricular Hearts.

World journal for pediatric & congenital heart surgery

Breast Milk and Necrotizing Enterocolitis in Congenital Heart Disease: A Case-Control Study.

Influence of Sex, Race and Ethnicity, and Deprivation on Survival and Completion of the Fontan Pathway for Children With Functionally Single Ventricle Heart Disease.

Circulation

Fontan Circulation and Aortic Stiffness: Insights into Vascular Dynamics and Haemodynamic Interplay.

Bifocal coronary sinus pacing and transcatheter tricuspid valve-in-valve implantation: an innovative combined approach.

Future cardiology

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions

Transcatheter Fontan completion: Creation of an extracardiac Fontan.

Retrospective Cohort Study of Additional Procedures and Transplant-Free Survival for Patients With Functionally Single Ventricle Disease Undergoing Staged Palliation in England and Wales.

European journal of heart failure

Haemodynamic contributors to pulmonary hypertension across the spectrum of adult congenital heart disease.

Adult Congenital Heart Disease Transplantation: Does Univentricle Physiology Impact Early Mortality?

Risk of Cardiac Reoperation: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

Annals of the Academy of Medicine, Singapore

2023

Fetal congenital heart diseases: Diagnosis by anatomical scans, echocardiography and genetic tests.

European heart journal. Case reports

Successful thromboaspiration of bilateral pulmonary thromboembolism in a Fontan circulation patient: a case report.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery

Contemporary management of borderline left ventricle.

Structured Tube Weaning Using the Hunger Provocation Method in Infants with Single Ventricle Heart Defects: A Multicenter Study.

International journal of cardiology

The utility of novel STE parameters in echocardiographic assessment of single ventricle after Fontan palliation.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery

Extracorporeal membrane oxygenation becomes a conceptual part of postoperative care after Norwood surgery for single-ventricle patients.

Advances in experimental medicine and biology

Molecular Pathways and Animal Models of Tricuspid Atresia and Univentricular Heart.

Advances in experimental medicine and biology

Human Genetics of Tricuspid Atresia and Univentricular Heart.

Advances in experimental medicine and biology

Clinical Presentation and Therapy of Tricuspid Atresia and Univentricular Heart.

Heart, lung & circulation

Heart Transplantation after Univentricular Palliation: Improved Outcomes and Increased Complexity.

Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy: How Best to Approach.

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions

Atrial septal interventions during and after hybrid stage I palliation of hypoplastic left heart syndrome.

A Case of Double Inlet Left Ventricle in a 63-Year-Old Female Patient.

Cureus

Heart Transplantation Following Fontan Failure: Long-Term Survival Analysis.

Pneumologie (Stuttgart, Germany)

[Pulmonary hypertension in adults with congenital heart disease in light of the 2022-ESC-PAH guidelines - Part II: Supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management (shunt lesions, left ventricular disorders, univentricular hearts), interventions, intensive care, follow-up, future perspectives].

European heart journal. Quality of care & clinical outcomes

Serial cardiopulmonary exercise testing in young patients after one-and-half ventricle repair and Fontan procedure: a comparative study.

Maternal-fetal comorbidities and obstetrical outcomes of fetal single ventricle cardiac defects: 10 years' experience with a multidisciplinary management protocol at a single center.

Prenatal diagnosis

World journal for pediatric & congenital heart surgery

Surgical Management of Norwood Procedure for Atypical Aortic Arch.

Native aortic root thrombus leading to myocardial infarction in a single ventricle patient.

Managing Bilateral Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy.

Bilateral pulmonary artery banding facilitates the systemic ventricular outflow tract growth for biventricular and univentricular repair candidates of complex arch anomaly.

Heart and vessels

Role and Applications of Experimental Animal Models of Fontan Circulation.

Pediatrics and neonatology

Clinical implications of dextrocardia based on four visceroatrial situs studies.

Relationship of Aortopulmonary Collaterals and Pulmonary Artery Development During Staged Single Ventricle Reconstruction.

Biomechanics and modeling in mechanobiology

Myocardial biomechanical effects of fetal aortic valvuloplasty.

Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies

Clinical Outcomes After Tracheostomy in Children With Single Ventricle Physiology: Collaborative Research From the Pediatric Cardiac Intensive Care Society Multicenter Cohort, 2010-2021.

Diagnostics (Basel, Switzerland)

Borderline Ventricles: From Evaluation to Treatment.

The Incidence and Relevance of Hematochezia in the Interstage Congenital Heart Population.

Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology

Long-term incidence of arrhythmias in extracardiac conduit Fontan and comparison between systemic left and right ventricle.

Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology

Postnatal outcome following fetal aortic valvuloplasty for critical aortic stenosis.

Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology

Safety and feasibility pilot study of continuous low-dose maternal supplemental oxygen in fetal single ventricle heart disease.

Atrioventricular valve surgery in patients with univentricular heart and two separate atrioventricular valves.

Arginine-NO metabolites are associated with morbidity in single ventricle infants undergoing stage 2 palliation.

Pediatric research

ASAIO journal (American Society for Artificial Internal Organs : 1992)

Short-Term Outcomes, Functional Status, and Risk Factors for Requiring Extracorporeal Life Support After Norwood Operation: A Single-Center Retrospective Study.

The international journal of cardiovascular imaging

Cardiac magnetic resonance ventricular parameters correlate with cardiopulmonary fitness in patients with functional single ventricle.

Predicting Long-Term Childhood Survival of Newborns with Congenital Heart Defects: A Population-Based, Prospective Cohort Study (EPICARD).

Impact of an Impaired Maternal-Fetal Environment on Death in Children With Congenital Heart Defects Undergoing Surgery in Denmark From 1994 to 2018.

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual

Staged Ventricular Septation of the Double-Inlet Ventricle: How-I-Do-It.

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual

From Surgical to Total Transcatheter Stage I Palliation: Exploring Evidence and Perspectives.

In vitro hemodynamic performance of a blood pump for self-powered venous assist in univentricular hearts.

Scientific reports

International journal of cardiology

Quantification of ventricular stress in univentricular hearts during early childhood using age-independent zlog-NT-proBNP.

Long-Term Survival After Single-Ventricle Palliation: A Swedish Nationwide Cohort Study.

Alimentary pharmacology & therapeutics

Meta-analysis: Incidence of cirrhosis and hepatocellular carcinoma in patients with Fontan palliation.

World journal for pediatric & congenital heart surgery

What Is the Hypoplastic Left Heart Syndrome?

Revista espanola de anestesiologia y reanimacion

Anesthetic management of pulmonary artery banding in adult patient with single ventricle and uncorrected transposition of the great arteries.

Days alive and out of hospital for children born with single-ventricle heart disease.

Frontiers in cardiovascular medicine

Systematic approach to obtain axillary arterial access for pediatric heart catheterizations.

Próxima:Associações

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Cardiopatia univentricular.

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Próxima:Comunidades

Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Cardiopatia univentricular

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Próxima:Doenças relacionadas

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ordenadas pelo número de sintomas em comum.

Síndrome do coração direito hipoplásico

25 em comum

ORPHA:98723

Comunicação interatrial

22 em comum

ORPHA:1478

Anomalia dos grandes vasos (aorta, arco aórtico, artérias pulmonares), congênita

21 em comum

ORPHA:98724

Hipoplasia do ventrículo direito isolada

20 em comum

ORPHA:439

Malformação tricúspide, congênita

20 em comum

ORPHA:98721

Hipertensão arterial pulmonar

19 em comum

ORPHA:182090

Hipertensão arterial pulmonar idiopática/hereditária

19 em comum

ORPHA:422

Heterotaxia

Transposição das grandes artérias

ORPHA:450

Anomalia do retorno venoso

ORPHA:216675

ORPHA:98729

Situs ambiguus

Defeito do canal auriculoventricular

ORPHA:157769