Raras
Buscar doenças, sintomas, genes...
Coração univentricular
ORPHA:1464CID-10 · Q20.4CID-11 · LA89.0DOENÇA RARA

Coração Univentricular (CUV) é um defeito cardíaco congênito grave, ou seja, um problema sério no coração com o qual o bebê já nasce. Ele se caracteriza por ter os dois átrios (as câmaras superiores do coração) conectados total ou quase totalmente a uma única cavidade que funciona como ventrículo. Os sinais e sintomas podem incluir: insuficiência cardíaca (o coração não consegue bombear o sangue de forma eficaz), dificuldade para ganhar peso e crescer, lábios e pele azulados (cianose), baixo nível de oxigênio no sangue (hipoxemia) e dificuldades no desenvolvimento neurológico.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Coração Univentricular (CUV) é um defeito cardíaco congênito grave, ou seja, um problema sério no coração com o qual o bebê já nasce. Ele se caracteriza por ter os dois átrios (as câmaras superiores do coração) conectados total ou quase totalmente a uma única cavidade que funciona como ventrículo. Os sinais e sintomas podem incluir: insuficiência cardíaca (o coração não consegue bombear o sangue de forma eficaz), dificuldade para ganhar peso e crescer, lábios e pele azulados (cianose), baixo nível de oxigênio no sangue (hipoxemia) e dificuldades no desenvolvimento neurológico.

Pesquisas ativas
6 ensaios
130 total registrados no ClinicalTrials.gov
Publicações científicas
1.094 artigos
Último publicado: 2026 Apr 16

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Infancy
+ neonatal
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q20.4
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

❤️
Coração
5 sintomas
🫁
Pulmão
3 sintomas
🫃
Digestivo
2 sintomas
📏
Crescimento
1 sintomas

+ 3 sintomas em outras categorias

Características mais comuns

90%prev.
Hipertensão venosa pulmonar
Muito frequente (99-80%)
90%prev.
Estenose pulmonar valvar
Muito frequente (99-80%)
55%prev.
Déficit de crescimento
Frequente (79-30%)
55%prev.
Desconforto respiratório
Frequente (79-30%)
55%prev.
Taquipneia
Frequente (79-30%)
55%prev.
Coração esquerdo hipoplásico
Frequente (79-30%)
14sintomas
Muito frequente (2)
Frequente (6)
Ocasional (6)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 14 características clínicas mais associadas, ordenadas por frequência.

Hipertensão venosa pulmonarPulmonary venous hypertension
Muito frequente (99-80%)90%
Estenose pulmonar valvarValvular pulmonary stenosis
Muito frequente (99-80%)90%
Déficit de crescimentoFailure to thrive
Frequente (79-30%)55%
Desconforto respiratórioRespiratory distress
Frequente (79-30%)55%
TaquipneiaTachypnea
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico1.094PubMed
Últimos 10 anos200publicações
Pico202489 papers
Linha do tempo
2026Hoje · 2026🧪 1990Primeiro ensaio clínico📈 2024Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
1Fase 12
·Pré-clínico7
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 9 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Coração univentricular

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

5 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

130 ensaios clínicos encontrados, 6 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
530 papers (10 anos)
#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

Ver todas no PubMed

📚 EuropePMC327 artigos no totalmostrando 198

2026

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

Open heart
2026

Hematopoietic Stem Cell Transplantation in a Fontan Patient With Myelodysplastic Syndrome.

JACC. Case reports
2025

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

European heart journal. Imaging methods and practice
2026

Outcomes of interstage home monitoring and unanticipated readmissions in infants with a functional univentricular heart.

Cardiology in the young
2026

Right ventricular outflow tract stenting for obstructive lesions in adult congenital heart disease.

International journal of cardiology. Congenital heart disease
2026

Cardiac Autonomic Function and Atrial Arrhythmias in Adult Patients With a Univentricular Physiology After Fontan Palliation.

CJC pediatric and congenital heart disease
2026

Cardiac Resynchronization Therapy for Failing Single Ventricle Physiology.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2026

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

Critical care explorations
2026

Augmenting Venous Return in Fontan Circulation: The Role of the Skeletal Muscle Pump and Implications for Mechanical Compression Devices-A Review of the Literature.

Pediatric cardiology
2026

Progressive cardiac strangulation by an epicardial lead in a single-ventricle patient: a case report.

Cardiology in the young
2026

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

Journal of cardiopulmonary rehabilitation and prevention
2026

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

American journal of physiology. Heart and circulatory physiology
2026

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

Hepatology research : the official journal of the Japan Society of Hepatology
2026

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

Pediatric cardiology
2026

Impact of Inflow Ratio on a Double-Inflow Cavopulmonary Assist Device.

ASAIO journal (American Society for Artificial Internal Organs : 1992)
2026

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

Circulation
2026

Double-Drainage Levoatrial Cardinal Vein: Cardiac CT Evaluation of Compensatory Pulmonary Venous Pathway in Univentricular Heart.

JACC. Case reports
2026

Lung cancer in a young adult Fontan: what would you do?

Cardiology in the young
2025

Ultrasound Evaluation of Fontan-Associated Liver Disease: A State-of-the-Art Review.

Diagnostics (Basel, Switzerland)
2025

Unrepaired most severe form of hypoplastic left heart syndrome in adulthood: a case report of late diagnosis and long-term follow-up.

European heart journal. Case reports
2026

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

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography &amp; Interventions
2026

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

Archives of disease in childhood
2026

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

The Journal of pediatrics
2025

Pulmonary artery growth in Fontan: what is the most effective strategy?

Revista espanola de cardiologia (English ed.)
2025

Living with a univentricular heart: the journey of surviving on borrowed time.

BMC cardiovascular disorders
2025

Cardiac autonomic function in patients with single ventricle physiology after Fontan palliation: A literature review.

International journal of cardiology. Congenital heart disease
2025

Electroanatomical mapping-guided leadless pacemaker (MICRA-VR) implantation in the right atrium of a univentricular heart with Fontan circulation, an approach combining imaging and electrophysiology: insights from a case report.

European heart journal. Case reports
2025

Early Versus Delayed Bidirectional Superior Cavopulmonary Anastomosis (BSCA) in Infants: A Comparison of Outcomes.

Cureus
2025

Hepatic vein-derived factors may affect pulmonary arteriovenous malformations after single ventricle palliation by modulating vascular cell behavior.

Scientific reports
2025

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

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2025

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

BMC anesthesiology
2025

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

Archives of disease in childhood
2025

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
2025

Childhood Neurocognitive Outcomes in Patients With Fontan Circulation.

Journal of the American Heart Association
2025

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

Innovations (Philadelphia, Pa.)
2026

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

European heart journal
2025

Prenatal detection and outcome of major heart defects in a country with universal screening.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2026

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

The Journal of thoracic and cardiovascular surgery
2025

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.

Journal of the American Heart Association
2025

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

Cardiology in the young
2025

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.

Cardiology in the young
2025

Percutaneous Pulmonary Artery Banding in the Low-Weight Term Newborn with Univentricular Heart.

Indian journal of pediatrics
2026

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

The Annals of thoracic surgery
2025

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

Angiogenesis
2025

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

Cardiology in the young
2025

Mediastinitis in pediatric cardiac surgery: an unusual complication. A single-center experience.

Archivos peruanos de cardiologia y cirugia cardiovascular
2025

Contemporary outcomes of the Fontan operation: a single-institution cohort from China.

Journal of thoracic disease
2025

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

Prenatal diagnosis
2025

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.

Cardiovascular diagnosis and therapy
2025

Double-Outlet Right Ventricle in an Adult With a Univentricular Heart and Total Situs Inversus.

JACC. Case reports
2025

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

Cardiology in the young
2025

Fontan-associated liver disease: paediatric experience in a reference centre in Colombia.

Cardiology in the young
2025

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

Journal of the American Heart Association
2026

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

World journal for pediatric &amp; congenital heart surgery
2025

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

Journal of the American Heart Association
2026

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

Pediatric cardiology
2025

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

American heart journal
2025

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

The Journal of thoracic and cardiovascular surgery
2025

Respiratory Function of Fontan Pediatric Patients with Hypoplastic Left Heart Syndrome and Other Morphologies of Functionally Single Ventricle-A Pilot Study.

Children (Basel, Switzerland)
2025

The single ventricle presenting late: surgical options.

Indian journal of thoracic and cardiovascular surgery
2026

30-Year Survival After the Fontan Operation in Denmark.

Pediatric cardiology
2025

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

Journal of the American Heart Association
2025

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

European heart journal. Cardiovascular Imaging
2026

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

Pediatric cardiology
2025

Percutaneous Atrioventricular Valve Repair With MitraClip in Failed Adult Fontan Circulation for Tricuspid Atresia.

JACC. Case reports
2026

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

Pediatric cardiology
2025

Establishment and Hemodynamic Assessment of the Superior Cavopulmonary Anastomosis in a Reproducible Porcine Model.

Biomedicines
2025

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

Cardiology in the young
2025

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

Seminars in thoracic and cardiovascular surgery
2025

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

Journal of the American Heart Association
2025

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

The Journal of pediatrics
2025

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.

Journal of the American Heart Association
2025

Vasopressin as a postoperative management strategy in Fontan procedure: a comparative study.

Indian journal of thoracic and cardiovascular surgery
2025

Lactescent urine following pediatric cardiac surgery due to catheter migration.

BMC pediatrics
2025

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

Cardiology in the young
2025

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

Interdisciplinary cardiovascular and thoracic surgery
2025

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

The journal of maternal-fetal &amp; 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
2025

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

The Journal of thoracic and cardiovascular surgery
2025

Rethinking the pathophysiology of Fontan circulation: insights from Kenji Sunagawa and Arthur Guyton.

Interdisciplinary cardiovascular and thoracic surgery
2025

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

Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia
2025

Heart-Liver Interplay in Patients with Fontan Circulation.

Journal of clinical medicine
2025

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

Journal of cardiology
2025

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

Open heart
2025

Influence of Simulated Altitude Exposure (2500 m) on Patients with Fontan Palliation Based on Circulating Hypoxia-Associated Factors.

International journal of molecular sciences
2025

Hypoplastic left heart syndrome (HLHS) becomes of age: Assessing the young adult with HLHS including the neoaorta/aortic arch.

International journal of cardiology. Congenital heart disease
2025

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

Echocardiography (Mount Kisco, N.Y.)
2025

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

Scientific reports
2025

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

Multimedia manual of cardiothoracic surgery : MMCTS
2025

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

Journal of clinical medicine
2025

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

Cardiology in the young
2025

Catheter ablation of atrial flutter in an adult with a univentricular heart, common atrium, and single atrioventricular valve: a case report-'complex things don't always require a complex solution'.

European heart journal. Case reports
2025

Transcatheter edge-to-edge repair of severe atrioventricular valve regurgitation in unrepaired functionally univentricular heart with dextroversion.

The Journal of invasive cardiology
2025

Biventricular Repair of Univentricular Heart Lowers Risk of Liver Disease Compared With the Fontan Operation.

JACC. Advances
2025

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

Resuscitation
2024

Long-term survival in patients with univentricular heart: A nationwide, register-based cohort study.

International journal of cardiology. Congenital heart disease
2024

Long-term outcomes of extracardiac Gore-Tex conduits in Fontan patients.

International journal of cardiology. Congenital heart disease
2024

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

JACC. Advances
2024

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

Journal of the American Heart Association
2024

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

Medicina (Kaunas, Lithuania)
2024

The impact of blood viscosity modeling on computational fluid dynamic simulations of pediatric patients with Fontan circulation.

Physics of fluids (Woodbury, N.Y. : 1994)
2024

Defining the causes for Fontan circulatory failure in total cavopulmonary connection patients.

Interdisciplinary cardiovascular and thoracic surgery
2025

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

World journal for pediatric &amp; congenital heart surgery
2024

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

Cardiology in the young
2025

Mountains and Waves: Fontan Circulation in Different Environmental Conditions.

Pediatric cardiology
2025

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

Clinical anatomy (New York, N.Y.)
2024

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

Journal of the American Heart Association
2024

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

Turk gogus kalp damar cerrahisi dergisi
2024

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

The international journal of cardiovascular imaging
2024

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

Journal of the American Heart Association
2024

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

Cardiology in the young
2024

The Effect of Cardiac Rehabilitation in Paediatric Fontan Circulation Patients: A Prospective Intervention Study.

Medicina (Kaunas, Lithuania)
2024

Outcomes of fenestration versus none in extracardiac total cavopulmonary connection.

Asian cardiovascular &amp; thoracic annals
2024

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

Cardiology in the young
2025

Poor correlation of venous lactate with systemic oxygen saturation in the paediatric cardiac ICU: a pilot study.

Cardiology in the young
2024

Dual-chamber leadless pacemaker in complex adult congenital heart disease: a case report.

European heart journal. Case reports
2024

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

BMC cardiovascular disorders
2024

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

Cardiology in the young
2025

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

The Journal of thoracic and cardiovascular surgery
2024

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

Cardiology in the young
2024

High-Performing Fontan Patients: A Fontan Outcome Registry by Cardiac Magnetic Resonance Imaging Study.

JACC. Advances
2025

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

Pediatric cardiology
2025

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

The Journal of thoracic and cardiovascular surgery
2024

Predictors of spontaneous pregnancy loss in single ventricle physiology.

Open heart
2024

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

Current opinion in pediatrics
2024

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

World journal for pediatric &amp; congenital heart surgery
2024

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

Journal of the American Heart Association
2024

Deferred Norwood in the setting of airway compression in double-inlet left ventricle with dextro-transposition of the great arteries.

Annals of pediatric cardiology
2025

Fontan Completion in Adult Patients with Functionally Univentricular Hearts.

The Thoracic and cardiovascular surgeon
2024

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

World journal for pediatric &amp; congenital heart surgery
2024

Outcomes of Patients Undergoing the Kawashima Procedure at an Early Age: A Single Center Experience.

World journal for pediatric &amp; congenital heart surgery
2024

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
2025

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

Pediatric cardiology
2024

A complex case of univentricular heart with multiple congenital malformations diagnosed in a newborn: a case report and literature review.

Annals of medicine and surgery (2012)
2024

Transcatheter Fontan completion: Creation of an extracardiac Fontan.

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography &amp; Interventions
2024

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

Journal of the American Heart Association
2024

Cardiovascular Outcomes in Fontan Patients With Right vs Left Univentricular Morphology: A Multicenter Study.

JACC. Advances
2025

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

Pediatric cardiology
2024

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

International journal of cardiology
2024

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

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2024

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

Advances in experimental medicine and biology
2024

Human Genetics of Tricuspid Atresia and Univentricular Heart.

Advances in experimental medicine and biology
2024

Clinical Presentation and Therapy of Tricuspid Atresia and Univentricular Heart.

Advances in experimental medicine and biology
2024

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

Heart, lung &amp; circulation
2024

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

The Annals of thoracic surgery
2024

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

Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography &amp; Interventions
2023

Open technique of performing total cavopulmonary connection on cardiopulmonary bypass.

Annals of pediatric cardiology
2024

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

Cureus
2024

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

European heart journal. Quality of care &amp; clinical outcomes
2024

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
2024

Surgical Management of Norwood Procedure for Atypical Aortic Arch.

World journal for pediatric &amp; congenital heart surgery
2024

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

Cardiology in the young
2024

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

The Annals of thoracic surgery
2024

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

Heart and vessels
2024

Role and Applications of Experimental Animal Models of Fontan Circulation.

Journal of clinical medicine
2024

[Angioplasty with ductal stent: experience at Garrahan Hospital].

Archivos de cardiologia de Mexico
2024

Gabapentin as a novel adjunct for postoperative irritability after superior cavopulmonary connection operation in children.

Cardiology in the young
2025

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

Pediatric cardiology
2024

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

Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
2024

Borderline Ventricles: From Evaluation to Treatment.

Diagnostics (Basel, Switzerland)
2025

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

Pediatric cardiology
2024

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

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
2024

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

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2024

In a porcine model of implantable pacemakers for pediatric unilateral diaphragm paralysis, the phrenic nerve is the best target.

Journal of cardiothoracic surgery
2024

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

Cardiology in the young
2024

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

Pediatric research
2024

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

The international journal of cardiovascular imaging
2024

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

Journal of clinical medicine
2024

Personalized Approach in Transcatheter Palliation of Congenital Heart Disease with Duct-Dependent Pulmonary Circulation: Right Ventricular Outflow Tract Stenting vs. Arterial Duct Stenting.

Journal of personalized medicine
2024

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

Journal of the American Heart Association
2024

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

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual
2024

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

Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual
2024

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

Scientific reports
2024

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

International journal of cardiology
2024

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

Journal of the American Heart Association
2024

What Is the Hypoplastic Left Heart Syndrome?

World journal for pediatric &amp; congenital heart surgery
2024

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

Revista espanola de anestesiologia y reanimacion
2024

Acute kidney injury and renal recovery following Fontan surgery.

JTCVS open
2024

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

Cardiology in the young
2024

Superior cavopulmonary shunt via right vertical axillary mini-thoracotomy.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2024

Anomalous anatomy-univentricular heart with three atrial appendages.

European heart journal. Case reports
2023

Left pulmonary artery reconstruction using cryopreserved pulmonary homograft.

Annals of pediatric cardiology
2024

Bidirectional glenn surgery without palliative pulmonary artery banding in univentricular heart with unrestricted pulmonary flow. Retrospective multicenter experience.

Journal of cardiothoracic surgery
2024

Impact of Fontan Fenestration on Adverse Cardiovascular Outcomes: A Multicentre Study.

The Canadian journal of cardiology
2024

Stage 1 and 2 Palliation: Comparing Ductal Stenting and Aorto-Pulmonary Shunts in Single Ventricles with Duct-Dependent Pulmonary Blood Flow.

Pediatric cardiology
2024

Textbook Outcome for Superior Cavopulmonary Connection: A Metric for Single Ventricle Heart Surgery.

World journal for pediatric &amp; congenital heart surgery
2024

Extremely Long-Term Follow-Up of Dr William Glenn's Original Patient Cohort With Superior Cavopulmonary Anastomosis (1958-1990).

World journal for pediatric &amp; congenital heart surgery
2024

Histopathological Spectrum and Molecular Characterization of Liver Tumors in the Setting of Fontan-Associated Liver Disease.

Cancers
2024

A rare case of severe Ebstein's anomaly and hepatoblastoma in a neonate.

Cardiology in the young
2024

Relationship Between Obesity and Global Longitudinal Strain in the Pediatric Single Ventricle Fontan Population Across Ventricular Morphologies.

Journal of the American Heart Association
2024

Pulmonary blood flow in children with univentricular heart and unilateral diaphragmatic paralysis.

Interdisciplinary cardiovascular and thoracic surgery
2024

Deep Sedation in Pediatric Patients With Single Ventricle Physiology Outside of the Operating Room.

World journal for pediatric &amp; congenital heart surgery
2024

Comparison of ductus stent versus surgical systemic-to-pulmonary shunt as initial palliation in patients with univentricular heart.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2024

Neurodevelopmental outcomes in congenital heart disease: Usefulness of biomarkers of brain injury.

Anales de pediatria
2024

Decreased erythrocyte aggregation in Glenn and Fontan: univentricular circulation as a rheologic disease model.

Pediatric research
2024

A Deep Learning Pipeline for Assessing Ventricular Volumes from a Cardiac MRI Registry of Patients with Single Ventricle Physiology.

Radiology. Artificial intelligence
2023

Transcatheter Palliation With Pulmonary Artery Flow Restrictors in Neonates With Congenital Heart Disease: Feasibility, Outcomes, and Comparison With a Historical Hybrid Stage 1 Cohort.

Circulation. Cardiovascular interventions
2024

Parental Impressions and Perspectives of Efficacy in Prenatal Counseling for Single Ventricle Congenital Heart Disease.

Pediatric cardiology
2023

Living a Full Life With Half of a Heart: A Congenital Heart Disease Patient's Perspective.

JACC. Case reports
Ver todos os 327 no EuropePMC

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Doenças relacionadas

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Ordenadas pelo número de sintomas em comum.

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Oncogenic Signaling Activation and Potential Biomarkers in Congestive Hepatopathy Revealed by Proteomic Analysis.
    Hepatology research : the official journal of the Japan Society of Hepatology· 2026· PMID 41609439mais citado
  2. Association of Pre-Fontan Hemodynamics With Long-Term Outcomes After Fontan Palliation: A Study From the Pediatric Cardiac Care Consortium.
    Circulation· 2026· PMID 41568447mais citado
  3. Maternal-Fetal Environment and Neurodevelopment in Patients with Single Ventricle Heart Disease.
    The Journal of pediatrics· 2026· PMID 41389933mais citado
  4. Early outcomes of children with univentricular circulation undergoing Fontan surgery: the EuroFontan registry.
    European heart journal· 2026· PMID 41127893mais citado
  5. Effects of ACE inhibitor and afterload reduction in single ventricles following bidirectional Glenn.
    Open heart· 2026· PMID 41876133mais citado
  6. Long-Term Outcomes of Antithrombotic Treatment in a Cohort of 864 Patients With Fontan Circulation.
    J Am Heart Assoc· 2026· PMID 41988962recente
  7. 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 Surg· 2026· PMID 41984069recente
  8. Biological tissue engineering for Fontan failure using decellularized extracellular matrix: a systematic review.
    J Transl Med· 2026· PMID 41975418recente
  9. Outcomes of atrioventricular valve interventions in patients with univentricular heart.
    Arch Cardiol Mex· 2026· PMID 41941859recente
  10. Univentricular heart with anomalous venous drainage and pulmonary sling.
    Rev Esp Cardiol (Engl Ed)· 2026· PMID 41936912recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:1464(Orphanet)
  2. MONDO:0015451(MONDO)
  3. GARD:18725(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q3010837(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Coração univentricular
Compêndio · Raras BR

Coração univentricular

ORPHA:1464 · MONDO:0015451
Prevalência
Unknown
Herança
Not applicable
CID-10
Q20.4 · Ventrículo com dupla via de entrada
CID-11
Ensaios
6 ativos
Início
Infancy, Neonatal
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0152424
EuropePMC
Wikidata
Papers 10a
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