A atresia tricúspide (AT) é uma malformação cardíaca congênita rara. Ela se caracteriza pela ausência da válvula tricúspide desde o nascimento, o que causa um desenvolvimento muito pequeno do ventrículo direito, fazendo com que o coração funcione como se tivesse apenas um ventrículo. A AT pode vir acompanhada de outras condições, como: grandes vasos (a aorta e a artéria pulmonar) em posição normal ou invertidos (transpostos – TGV); uma conexão obrigatória entre os átrios (as câmaras superiores do coração) que é vital para a sobrevivência, como um forame oval patente (um pequeno orifício entre os átrios que não se fechou após o nascimento) ou um defeito do septo atrial do tipo ostium secundum (um “buraco” na parede que separa os átrios); um defeito no septo ventricular (um “buraco” na parede que separa os ventrículos, as câmaras inferiores do coração), presente em 90% dos casos; uma obstrução na saída do sangue para os pulmões, que pode ser uma atresia pulmonar (ausência de abertura da válvula pulmonar), estenose (estreitamento) ou hipoplasia (subdesenvolvimento da válvula ou artéria pulmonar) — essa obstrução geralmente acontece na AT com grandes vasos em posição normal, mas também pode ocorrer com vasos transpostos; e coarctação da aorta (um estreitamento da artéria aorta) e/ou interrupção do arco aórtico (quando a aorta é “partida” em algum ponto). Essas últimas condições geralmente ocorrem na AT com grandes vasos transpostos.
Introdução
O que você precisa saber de cara
A atresia tricúspide (AT) é uma malformação cardíaca congênita rara. Ela se caracteriza pela ausência da válvula tricúspide desde o nascimento, o que causa um desenvolvimento muito pequeno do ventrículo direito, fazendo com que o coração funcione como se tivesse apenas um ventrículo. A AT pode vir acompanhada de outras condições, como: grandes vasos (a aorta e a artéria pulmonar) em posição normal ou invertidos (transpostos – TGV); uma conexão obrigatória entre os átrios (as câmaras superiores do coração) que é vital para a sobrevivência, como um forame oval patente (um pequeno orifício entre os átrios que não se fechou após o nascimento) ou um defeito do septo atrial do tipo ostium secundum (um “buraco” na parede que separa os átrios); um defeito no septo ventricular (um “buraco” na parede que separa os ventrículos, as câmaras inferiores do coração), presente em 90% dos casos; uma obstrução na saída do sangue para os pulmões, que pode ser uma atresia pulmonar (ausência de abertura da válvula pulmonar), estenose (estreitamento) ou hipoplasia (subdesenvolvimento da válvula ou artéria pulmonar) — essa obstrução geralmente acontece na AT com grandes vasos em posição normal, mas também pode ocorrer com vasos transpostos; e coarctação da aorta (um estreitamento da artéria aorta) e/ou interrupção do arco aórtico (quando a aorta é “partida” em algum ponto). Essas últimas condições geralmente ocorrem na AT com grandes vasos transpostos.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
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
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 10 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Atresia tricúspide
Centros de Referência SUS
24 centros habilitados pelo SUS para Atresia tricúspide
Centros para Atresia tricúspide
Detalhes dos centros
Hospital Universitário Prof. Edgard Santos (HUPES)
R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808
Serviço de Referência
Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
Serviço de Referência
Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
Serviço de Referência
Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)
Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207
Serviço de Referência
Hospital das Clínicas da UFG
Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424
Serviço de Referência
Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
Atenção Especializada
Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
Serviço de Referência
Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
Atenção Especializada
Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
Serviço de Referência
Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
Atenção Especializada
Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
Serviço de Referência
Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
Serviço de Referência
Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
Atenção Especializada
Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
Serviço de Referência
Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
Serviço de Referência
Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)
Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988
Serviço de Referência
Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
Serviço de Referência
Hospital de Clínicas de Porto Alegre (HCPA)
Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601
Serviço de Referência
Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
Serviço de Referência
Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
Serviço de Referência
Hospital de Base de São José do Rio Preto
Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798
Atenção Especializada
Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
Serviço de Referência
Hospital de Clínicas de Ribeirão Preto (HCRP-USP)
R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187
Serviço de Referência
UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
Serviço de Referência
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
Ensaios em destaque
🟢 Recrutando agora
2 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
10 ensaios clínicos encontrados, 2 ativos.
Publicações mais relevantes
Generation of three induced pluripotent stem cell clones from a functional single ventricle patient carrying the BRAF c.1897 T > C variant.
Cardiofaciocutaneous syndrome is a genetic disorder characterized by congenital heart disease, developmental delays and ectodermal abnormalities. Cardiofaciocutaneous syndrome is caused by pathogenic variants in the genes of the RAS/MAPK pathway, particularly BRAF. However, the mechanism by which congenital heart defects arise in RASopathy patients is still poorly understood. Therefore, using non-integrating episomal vectors, we generated three hiPSC clones from peripheral blood mononuclear cells from a 33-year old male carrying a c.1897 T > C missense variant in the BRAF gene, who was born with pulmonary stenosis, tricuspid atresia and hypoplastic right ventricle, consistent with a functional single ventricle.
Early outcomes of children with univentricular circulation undergoing Fontan surgery: the EuroFontan registry.
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.
Development of 3D-Printed Congenital Heart Disease Models Using Feasible Low-Cost Workflow - A Potential Tool to Improve Pediatric Cardiology Education.
Congenital heart diseases (CHD) require an in-depth anatomopathological understanding. 3D printing is a promising educational tool for creating physical prototypes from imaging data, but costs are a major issue. This study aimed to describe a feasible low-cost workflow for the development of 3D-printed (3DP) CHD models and create a shareable educational set. Data from cardiac tomography images were used, following the steps of image acquisition, segmentation, digital design, slicing, 3D printing, and post-printing. Valvar structures were created from echocardiographic data using the cartographic heightmap technique and inserted into prototypes. 3DP models were evaluated by an expert team, enhanced, and applied to medical residents during an educational session. Free software, a desktop 3D printer, and low-cost materials were used. Twelve 3DP models were developed, including ventricular septal defect with patent ductus arteriosus, atrial septal defect, Tetralogy of Fallot, transposition of the great arteries, atrioventricular septal defect, coarctation of the Aorta, hypoplastic left heart syndrome, tricuspid atresia, pulmonary atresia, total anomalous pulmonary venous connection, Truncus arteriosus, and interrupted aortic arch. All residents (100%) agreed that prototypes were "faithful to the anatomy", "visually appealing", "motivated the study", and "allowed better spatial conceptualization". They related 3DP CHD models "are an important pedagogical resource" and can potentially benefit the education of "undergraduate students" (100%), "interdisciplinary team" (100%), "cardiologists, surgeons and residents" (100%), and "families" (93%). Development of 3DP CHD models using a low-cost workflow is feasible. Models developed are freely available for download and printing, intending to promote education to all interested in CHD. As cardiopatias congênitas (CC) demandam profundo conhecimento anatomopatológico. A impressão 3D (Imp3D) é uma ferramenta de ensino promissora para a criação de protótipos físicos a partir de dados de imagem, mas os custos representam um grande obstáculo. Descrever um fluxo de trabalho factível e de baixo custo para o desenvolvimento de modelos de CC em Imp3D e criar um conjunto de modelos de ensino compartilháveis. Foram utilizados conjuntos de imagens de tomografia cardíaca, seguindo as etapas de aquisição, segmentação, design digital, fatiamento, Imp3D e pós-impressão. Estruturas valvares foram criadas a partir de dados ecocardiográficos utilizando a técnica cartographic heightmap e inseridas nos protótipos. Os modelos de CC em Imp3D foram avaliados por uma equipe de especialistas, aprimorados e aplicados a residentes de cardiologia e cirurgia cardíaca durante uma sessão de ensino. Foram utilizados softwares gratuitos, impressora 3D desktop e materiais de baixo custo. Doze modelos de CC em Imp3D foram desenvolvidos, incluindo Comunicação Interventricular, Persistência do Canal Arterial, Comunicação Interatrial, Tetralogia de Fallot, Transposição das Grandes Artérias, Defeito do Septo Atrioventricular, Coarctação de Aorta, Síndrome do Coração Esquerdo Hipoplásico, Atresia Tricúspide, Atresia Pulmonar, Conexão Anômala Total de Veias Pulmonares, Truncus Arteriosus e Interrupção de Arco Aórtico. Todos os residentes (100%) concordaram que os protótipos eram “fiéis à anatomia”, “visualmente atraentes”, “motivaram o estudo” e “permitiram uma melhor conceitualização espacial”. Eles relataram que os modelos em Imp3D “são um importante recurso pedagógico” e podem potencialmente beneficiar a educação de “estudantes de graduação” (100%), “equipe interdisciplinar” (100%), “cardiologistas, cirurgiões e residentes” (100%) e “famílias” (93%). O desenvolvimento de modelos de CC em Imp3D utilizando metodologia de baixo custo é factível. Os arquivos digitais dos modelos desenvolvidos estão disponíveis gratuitamente para download e impressão, com o objetivo de promover a educação de todos os interessados no estudo das CC.
Early Experience with the First Canadian Paediatric Fontan Long-Term Follow-up Clinic.
The Fontan procedure has significantly improved survival in patients with single-ventricle physiology; however, multisystem complications have become a major source of late morbidity and mortality. In 2020, the Hospital for Sick Children (SickKids) established a dedicated paediatric Fontan Long-Term Follow-up Clinic. This study evaluated the prevalence and spectrum of cardiac and extracardiac morbidities in the clinic cohort since inception. This single-centre retrospective study included all patients referred between January 2020 and December 2024. Standardized assessments comprised cardiac evaluations (echocardiography, Holter monitoring, exercise testing, cardiac MRI) and extracardiac investigations of hepatic, renal, hematologic, endocrine, growth, and neurodevelopmental status. Fontan-associated liver disease (FALD) was graded using MR elastography and imaging findings. A total of 161 patients (mean age 13.2 ± 3.7 years; 58.4% male) were assessed at a mean of 9.5 ± 4.3 years post-Fontan completion. Tricuspid atresia was the most common diagnosis (26.1%). Cardiac function was generally preserved, with mean predicted peak VO₂ of 63.6 ± 17.6%. Moderate or severe atrioventricular valve regurgitation occurred in 21.5%, and ventricular dysfunction in 5.6%. Moderate or greater FALD was observed in 8.0%, with mean magnetic resonance elastography 4.58 kPa (Interquartile range, 4.03-5.40). Lymphopenia (64.6%), thrombocytopenia (16.8%), vitamin D deficiency (41.9%), and reduced bone density (28.0%) were frequent. Neurodevelopmental challenges were common (39.8% learning difficulties). Despite preserved cardiac performance, Fontan patients exhibited a high burden of hepatic, hematologic, endocrine, skeletal, and neurocognitive morbidities. Multidisciplinary, longitudinal follow-up is essential to optimize outcomes and quality of life.
Unusual right-to-left shunt in a patient with tricuspid atresia and stenosis of Fontan conduit.
A patient post-Fontan palliation with a venous collateral unusually arising from the renal vein. Since renal vein oxygen saturations are relatively high, there was not systemic desaturation despite a right-to-left shunt.
Publicações recentes
Single Ventricle: Radiology Primer for Preprocedural Evaluation.
Development of 3D-Printed Congenital Heart Disease Models Using Feasible Low-Cost Workflow - A Potential Tool to Improve Pediatric Cardiology Education.
🥈 ObservacionalEarly Experience with the First Canadian Paediatric Fontan Long-Term Follow-up Clinic.
Delayed Diagnosis of Tricuspid Atresia: A Case Report.
Unusual right-to-left shunt in a patient with tricuspid atresia and stenosis of Fontan conduit.
📚 EuropePMC728 artigos no totalmostrando 199
Development of 3D-Printed Congenital Heart Disease Models Using Feasible Low-Cost Workflow - A Potential Tool to Improve Pediatric Cardiology Education.
Arquivos brasileiros de cardiologiaEarly Experience with the First Canadian Paediatric Fontan Long-Term Follow-up Clinic.
Pediatric cardiologyDelayed Diagnosis of Tricuspid Atresia: A Case Report.
JNMA; journal of the Nepal Medical AssociationUnusual right-to-left shunt in a patient with tricuspid atresia and stenosis of Fontan conduit.
Cardiology in the youngFirst Report on Atrial Leadless Pacing in a Lateral Tunnel Fontan Patient.
Journal of cardiovascular electrophysiologyLung cancer in a young adult Fontan: what would you do?
Cardiology in the youngEjection Fraction-Related Differences in Left Ventricular and Atrial Strain Indices Among Pediatric Fontan Circulation with Systemic Left Ventricle Morphology.
Diagnostics (Basel, Switzerland)Recurrent Diffuse Chorioamniotic Hemosiderosis of the Placenta in a Mother With Congenital Heart Disease.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyAnesthetic Management of a 32-Year-Old Woman Undergoing Laparoscopic Gynecological Surgery With Prior Fontan Repair for a Complex Congenital Heart Defect.
The American journal of case reportsChallenges in AVNRT ablation in a patient with previous atrio-pulmonary Fontan surgery: a case report.
European heart journal. Case reportsGeneration of three induced pluripotent stem cell clones from a functional single ventricle patient carrying the BRAF c.1897 T > C variant.
Stem cell researchElectroanatomical 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 reportsCardio-Obstetric Care in a Patient With Tricuspid Atresia and Fenestrated Fontan.
JACC. Case reportsHigh risk and low incidence diseases: Cyanotic critical congenital heart disease.
The American journal of emergency medicineFontan Circulation in Complex Congenital Heart Disease: Do Early Benefits Outweigh Later Problems?
Circulation. Heart failureEbstein Anomaly and the Range of Right-Sided Heart Defects.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyEarly outcomes of children with univentricular circulation undergoing Fontan surgery: the EuroFontan registry.
European heart journalContemporary outcomes and health care costs associated with single-ventricle heart failure admissions in adults in the United States.
The Journal of thoracic and cardiovascular surgeryPolycythemia and its determinants among children with unoperated cyanotic congenital heart disease at Tikur Anbessa specialized hospital, Ethiopia: observational cross-sectional study.
BMC pediatricsOutcomes of the Fontan operation in patients with Ebstein anomaly: An Australia and New Zealand Fontan registry study.
JTCVS openThe complex and hazardous course for heterotaxy-associated congenital heart disease.
JTCVS openOutcomes of Fontan patients undergoing combined heart-liver transplantation in pediatric hospitals across the United States.
The Journal of thoracic and cardiovascular surgeryHypoplastic right heart with heterotaxy has worse five-year transplant-free survival than the hypoplastic left heart syndrome: a thirteen-year single-centre experience.
Cardiology in the youngTricuspid Atresia and Transposition of the Great Arteries in a Neonate With Successful Surgical Correction: A Case Report.
Clinical case reportsLeft 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 therapyAnaesthesia strategies for caesarean section in superior cavopulmonary anastomosis.
BMJ case reportsSurgical Treatment of a Rare Case of Uhl Anomaly, Tricuspid Atresia, Absent Pulmonary Valve, Hypoplastic Right Ventricle, and Right Ventricular Coronary Artery Fistula.
World journal for pediatric & congenital heart surgeryA Retrospective Study on the Imaging Spectrum of Functional Single Ventricle and Its Associations.
The Indian journal of radiology & imagingTranscatheter Fenestration Closure after Fontan Surgery.
The journal of Tehran Heart CenterShifting strategies to address systemic outflow tract obstruction in single ventricle anatomy with transposed great arteries.
The Journal of thoracic and cardiovascular surgeryRestrictive Atrial Communication in Tricuspid Atresia With Minimal Duct-Dependent Pulmonary Circulation in a Newborn.
JACC. Case reportsLongitudinal Trends in Pediatric Survival by Congenital Heart Defect in Texas, 1999 to 2017.
JACC. AdvancesClarifying the cardiovascular morphology and associated abnormalities in patients with tricuspid Atresia using multidetector computed tomography angiography.
The international journal of cardiovascular imagingDiagnosis of Diastolic Dysfunction in Adults With Failing Fontan Circulation.
JACC. AdvancesPercutaneous Atrioventricular Valve Repair With MitraClip in Failed Adult Fontan Circulation for Tricuspid Atresia.
JACC. Case reportsTricuspid atresia with absent pulmonary valve and intact ventricular septum: Fontan operation after complete exclusion of the right ventricle.
Cardiology in the young[MEP-23] Managing Recurrent Chylothorax Post Pediatric Cardiac Surgery.
Turk gogus kalp damar cerrahisi dergisiDe Novo Deletion in the 12q24.23q24.31 Chromosomal Region Causing a Neurodevelopmental Syndrome in a Female Saudi Patient: A Case Report.
CureusPulmonary Embolism in a Palliative Aortopulmonary Shunt.
JACC. Case reportsImpact of cardiac position, morphology and operative technique on long-term Fontan outcomes in heterotaxy†.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryPrevalence, predictors, and mortality of Super-Fontan patients in a single-centre nationwide cohort.
Cardiology in the youngOff-pump Laks-type central shunt for tricuspid atresia with small branch pulmonary arteries.
Multimedia manual of cardiothoracic surgery : MMCTSQuantifying Additional Procedures in Functionally Single-Ventricle Disease: A National Cohort Study.
Annals of thoracic surgery short reportsPrenatal Predictors and Early Postnatal Outcomes in Fetuses Diagnosed with Tricuspid Atresia.
Diagnostics (Basel, Switzerland)A Case of Antenatal Diagnosis of Absent Pulmonary Valve Syndrome with Intact Ventricular Septum, Large Patent Ductus Arteriosus, and Ascending Aorta Dilatation.
The journal of Tehran Heart CenterThoracic Lymphatic Perfusion Patterns Assessed by Magnetic Resonance Imaging and Late Fontan Failure.
Diagnostics (Basel, Switzerland)Emergent surgical intervention for a neonate with premature constriction of the ductus arteriosus with ductal-dependent tricuspid atresia: a case report.
European heart journal. Case reportsManagement of Tricuspid Atresia With Normally Related Great Arteries and Left-Sided Obstruction.
World journal for pediatric & congenital heart surgeryMid-term results and late events after the Fontan operation: A single-center experience.
Turk gogus kalp damar cerrahisi dergisiComparison of pulmonary artery growth between ductus stent and systemic-to-pulmonary shunt as single-ventricle palliation.
The Journal of thoracic and cardiovascular surgeryA Comparison of Active and Passive Surveillance Strategies for Selected Birth Defects in New York.
Birth defects researchSuccessful Thoracic Duct Embolization Following Fontan-Related Chylothorax in a Six-Year-Old Girl: A Case Report.
CureusA 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)Molecular Pathways and Animal Models of Tricuspid Atresia and Univentricular Heart.
Advances in experimental medicine and biologyHuman Genetics of Tricuspid Atresia and Univentricular Heart.
Advances in experimental medicine and biologyClinical Presentation and Therapy of Tricuspid Atresia and Univentricular Heart.
Advances in experimental medicine and biologyPresentation of Complex Congenital Cardiac Anomalies in a Newborn Pediatric Patient: A Case Report.
CureusClinical 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 SocietiesBridge to transplant in single-ventricle anatomy: subpulmonary support with EXCOR ventricular assist device associated with pulmonary artery reconstruction.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryA Female Newborn With Occipital Encephalocele and a Hypoplastic Right Ventricle Secondary to Tricuspid and Pulmonary Atresia: A Case Report.
CureusCardiovascular physical examination as a screening tool for congenital heart disease in newborns at a teaching hospital in Ghana.
Ghana medical journalCorrigendum: Comparison of cardiac function between single left ventricle and tricuspid atresia: assessment using echocardiography combined with computational fluid dynamics.
Frontiers in pediatricsMorbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life.
European journal of preventive cardiologyPostmortem magnetic resonance imaging findings of tricuspid atresia with ventricular and atrial septal defects and subvalvular pulmonic stenosis in a Japanese native Noma horse.
Journal of equine scienceComparison 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 SurgeryImpact of pulsatile pulmonary blood flow on cardiopulmonary exercise performance after the Fontan procedure.
JTCVS openAnatomical and physiological diagnostic discrepancies in fetuses with single-ventricle congenital heart disease in a contemporary cohort.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyA Rare Case of Tricuspid Atresia Absent Pulmonary Valve Diagnosed on Fetal Echocardiography.
CASE (Philadelphia, Pa.)Tricuspid atresia with atrial and ventricular septal defects in a kitten.
Journal of veterinary cardiology : the official journal of the European Society of Veterinary CardiologyPrenatal sonographic findings of prominent fetal tricuspid annulus: A case report.
Radiology case reports[Percutaneous management of severe hemoptysis in a patient after fontan surgery].
Archivos peruanos de cardiologia y cirugia cardiovascularLong-Term Survival Is Superior in Patients With Pulsatile Pulmonary Flow After the Björk Procedure.
World journal for pediatric & congenital heart surgeryInvited Commentary: The Rudimentary Right Ventricle and Achieving Pulsatile Pulmonary Blood Flow After the Björk Procedure for Tricuspid Atresia: How Little Is Too Little?
World journal for pediatric & congenital heart surgeryDichorionic Diamniotic Twin Pairs with Complex Congenital Heart Disease.
Pediatric cardiologyTricuspid Atresia with Absent Pulmonary Valve with Nearly Discontinuous Branch Pulmonary Arteries.
Pediatric cardiologyComparing palliation strategies for single-ventricle anatomy with transposed great arteries and systemic outflow obstruction.
JTCVS techniquesLiving to Tell the Tale: A First-Person Chronicle of the Development of the Fontan-Kreutzer Procedure.
World journal for pediatric & congenital heart surgeryTricuspid atresia 1c accompanying neonatal encephalopathy treated with pulmonary trunk banding and therapeutic hypothermia.
Annals of pediatric cardiologyEndocardial pacing in a single-ventricle patient with tricuspid atresia-a case report.
Clinical case reportsLate Diagnosis of Uncorrected Tricuspid Atresia with Transposition of the Great Arteries in an Adult Patient.
Radiology. Cardiothoracic imagingCombined tricuspid atresia- AV septal defect-a rare congenital cardiac abnormality.
Echocardiography (Mount Kisco, N.Y.)Long-Term outcomes in adult patients with congenital heart disease considered for transplantation: A single center study.
Clinical transplantationChallenges in diagnosing pulmonary embolism in an adult with Fontan physiology: a case report.
European heart journal. Case reportsDamus-Kaye-Stansel surgery in a patient with tricuspid atresia, transposition of the great arteries, and type A aortic arch interruption.
Archivos de cardiologia de MexicoInter-arterial course of the left coronary artery with a commissural origin in a 40-year-old patient with tricuspid atresia post Fontan shunt.
Indian journal of thoracic and cardiovascular surgeryInnominate vein reconstruction in patients with bilateral superior vena cava undergoing bilateral bidirectional superior cavopulmonary anastomosis.
Annals of pediatric cardiologyAtrial fibrillation in adult congenital heart disease and the general population.
Heart rhythmAn Unusual Mass in the Right Atrium After a Staged Extracardiac Total Cavopulmonary Connection in a Case of Tricuspid Atresia.
Journal of cardiothoracic and vascular anesthesiaHemostasis assessment in Fontan patients using the new thrombodynamics test.
The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of CardiologyComparison of cardiac function between single left ventricle and tricuspid atresia: assessment using echocardiography combined with computational fluid dynamics.
Frontiers in pediatricsGenetic variant in the BRAF gene compatible with Noonan spectrum disorders in an adult Fontan patient with refractory protein losing enteropathy: a follow-up report.
European heart journal. Case reportsA case report of infective endocarditis in a 52-year-old female with unrepaired tricuspid atresia and d-transposition of the great arteries.
European heart journal. Case reportsLoss of myocardial Hey2/Hrt2 function disrupts rightward shift of atrioventricular cushion tissue and causes tricuspid atresia.
Developmental dynamics : an official publication of the American Association of AnatomistsCongenital heart disease - A complex cardiac anomaly: A case report.
Journal of family medicine and primary careArterial switch for double-outlet left ventricle - Diagnostic and surgical considerations.
Annals of pediatric cardiologyPredictors of prolonged pleural effusion after Fontan operation.
Cardiology in the youngLongitudinal analysis of systemic ventricular function and atrioventricular valve function after the Fontan procedure.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryOutcome after stage 1 palliation in non-hypoplastic left heart syndrome patients as a univentricular palliation.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgerySingle cell evaluation of endocardial Hand2 gene regulatory networks reveals HAND2-dependent pathways that impact cardiac morphogenesis.
Development (Cambridge, England)Long-term survival and center volume for functionally single-ventricle congenital heart disease in England and Wales.
The Journal of thoracic and cardiovascular surgeryTricuspid atresia and common arterial trunk: a rare form of CHD.
Cardiology in the youngAssociation of Bulboventricular Foramen Size and Need for Early Intervention in Infants with Tricuspid Atresia or Double-Inlet Left Ventricle with Normally Related Great Arteries.
Journal of the American Society of Echocardiography : official publication of the American Society of EchocardiographyPrepregnancy exposure to dietary arsenic and congenital heart defects.
Birth defects researchPrenatal Diagnosis of a Rare Case of Double-Outlet Right Ventricle with Tricuspid Atresia.
The journal of Tehran Heart CenterTranscatheter Mitral Valve Repair in a Tricuspid Atresia Patient With Potts and Glenn Shunts.
JACC. Case reportsThe prognostic role of liver volumetry in Fontan patients.
Cardiology in the young20 years of experience with the Fontan procedure: characteristics and clinical outcomes of children in a tertiary referral hospital.
Cardiology in the youngSuccessful coil embolization for pediatric internal mammary artery aneurysm after the Fontan procedure: A case report and literature review.
Journal of cardiology casesDiagnosis of fetal congenitally unguarded tricuspid valve orifice by echocardiography.
Echocardiography (Mount Kisco, N.Y.)Rare association of absent pulmonary valve syndrome, complete atrioventricular canal defect, double outlet right ventricle, right aortic arch, and aberrant right subclavian artery in a fetal case.
Echocardiography (Mount Kisco, N.Y.)Francis Fontan (1929-2018): Pioneer pediatric cardiac surgeon.
Journal of medical biographyHypoplastic Left Heart Syndrome: Definition, Morphology, and Classification.
World journal for pediatric & congenital heart surgeryCommentary: Conduction system in tricuspid atresia: Anatomic insights continue to refine our approach.
JTCVS openIdentification of the atrioventricular conduction axis and its positional relationship with anatomical landmarks of a heart with tricuspid atresia.
JTCVS openOutcomes of bidirectional Glenn surgery done without prior cardiac catheterization.
The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of CardiologyAcute Liver Failure due to Altered Fontan Circulation in a Patient With Tricuspid Atresia: A Case Report.
Journal of investigative medicine high impact case reportsCommon arterial trunk in functionally univentricular hearts: a case series.
Cardiology in the youngReoperation after modified Björk procedure for tricuspid atresia.
Annals of pediatric cardiologyConsiderations in Critical-Care and Anesthetic Management of Adult Patients Living With Fontan Circulation.
The Canadian journal of cardiologyTricuspid atresia with absent pulmonary valve: A rare form of single ventricle.
Journal of cardiac surgeryTracheobronchial Release for Left Bronchus Compression After Aortic Arch Repair.
World journal for pediatric & congenital heart surgeryPostmortem 9.4-T MRI for Fetuses With Congenital Heart Defects Diagnosed in the First Trimester.
Frontiers in cardiovascular medicineJuxtaposition of atrial appendages: Evaluation of cardiovascular morphology and associated anomalies on multidetector computed tomography angiography.
Journal of cardiac surgeryCardiac Resynchronization Therapy Using Single Site Left Ventricular Pacing in a Tricuspid Atresia Patient With Left Bundle Branch Block.
CJC pediatric and congenital heart diseaseJuxtaposition of the Atrial Appendages: A Large Echocardiographic Series.
Pediatric cardiologyTricuspid atresia in adulthood.
European heart journal. Case reportsExercise Intolerance, Benefits, and Prescription for People Living With a Fontan Circulation: The Fontan Fitness Intervention Trial (F-FIT)-Rationale and Design.
Frontiers in pediatricsFetal Tricuspid Valve Agenesis/Atresia: Testing Predictions of the Embryonic Etiology.
Pediatric cardiologyCommon Arterial Trunk Associated with Functionally Univentricular Heart: Anatomical Study and Review of the Literature.
Journal of cardiovascular development and diseaseMechanical thrombectomy of COVID-19 DVT with congenital heart disease leading to phlegmasia cerulea dolens: a case report.
BMC cardiovascular disordersValve-Sparing Reimplantation for Right Coronary Artery Compression After Fontan Procedure.
The Annals of thoracic surgeryAnesthetic Management of Resection of Metastatic Occipital Malignancy in a Patient With Fontan Physiology.
CureusMultiple Organ Failure Associated with SARS-CoV-2 Infection in a Child with Down Syndrome: Is Trisomy 21 Associated with an Unfavourable Clinical Course?
Case reports in pediatricsUmbilical artery to common femoral artery (CFA) transposition, a novel technique for limb salvage in a newborn: a case report.
The Turkish journal of pediatricsCohort study of intervened functionally univentricular heart in England and Wales (2000-2018).
Heart (British Cardiac Society)Emergency Department Presentation and Outcome of Children With Cyanotic Congenital Heart Diseases.
CureusAnalysis of outcomes in patients with abnormal laterality undergoing congenital heart surgery.
Cardiology in the youngPrevalent pharmacotherapy of US Fontan survivors: A study utilizing data from the MarketScan Commercial and Medicaid claims databases.
American heart journalIsoproterenol Challenge Unmasking Dynamic Bulbo-Ventricular Foramen Restriction.
Pediatric cardiologyA case of tricuspid atresia with pulmonary valve absence coexisting coronary-right ventricular fistula.
General thoracic and cardiovascular surgeryMaternal Smoking and Congenital Heart Defects, National Birth Defects Prevention Study, 1997-2011.
The Journal of pediatricsFontan operation for tricuspid atresia with absent pulmonary valve: a case series.
Cardiology in the youngPrenatal detection of congenital heart disease - results of a Swedish screening program 2013-2017.
BMC pregnancy and childbirthTemporal progression of P wave abnormality in a patient with classical or atriopulmonary Fontan.
Journal of electrocardiologyThe pulmonary vascular bed in patients with functionally univentricular physiology and a Fontan circulation.
Cardiology in the youngGenetic and functional analyses detect one pathological NFATC1 mutation in a Chinese tricuspid atresia family.
Molecular genetics & genomic medicineAn echocardiographic finding mimicking tricuspid atresia in a neonate with dilated cardiomyopathy.
Cardiology in the youngComplex Catheter-Based Structural Heart Reconstruction in a Patient With Tricuspid Atresia and Björk Palliative Conduit.
JACC. Case reportsTransition to Ductal Stenting for Single Ventricle Patients Led to Improved Survival: An Institutional Case Series.
World journal for pediatric & congenital heart surgeryCardiovascular Development and Congenital Heart Disease Modeling in the Pig.
Journal of the American Heart AssociationThe prenatal diagnosis of a rare circular shunt with absent pulmonary valve syndrome.
Journal of clinical ultrasound : JCUMRI characterization of hemodynamic patterns of human fetuses with cyanotic congenital heart disease.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyAbsent pulmonary valve with tricuspid atresia/stenosis: literature review with new three long-term cases.
Heart and vesselsSingle Ventricle-A Comprehensive Review.
Children (Basel, Switzerland)Double trouble: A hitherto undescribed association of tricuspid atresia and common arterial trunk!
Journal of cardiac surgeryFactors associated with mortality or transplantation versus Fontan completion after cavopulmonary shunt for patients with tricuspid atresia.
The Journal of thoracic and cardiovascular surgeryA unique arrangement of "kissing" atrial appendages.
Journal of cardiac surgeryEffects of universal critical CHD screening of neonates at a mid-sized California congenital cardiac surgery centre.
Cardiology in the youngTricuspid Atresia with Persistent Left Superior Vena Cava and Pulmonary Arterial Hypertension. Case Report.
Current problems in cardiologyTricuspid Atresia Type 1B With Persistent Left Superior Vena Cava in a Four-Month-Old Child: An Unusual Combination.
CureusThe Glenn procedure: Clinical outcomes in patients with congenital heart disease in pakistan.
Annals of cardiac anaesthesiaMyocardial deformation in patients with a single left ventricle using 2D cardiovascular magnetic resonance feature tracking: a case-control study.
The international journal of cardiovascular imagingA 40-Year-Old Man With Tricuspid Atresia, Status Post-Fontan, With Severe COVID-19 Pneumonia and Pneumothorax.
JACC. Case reportsGlenn surgery: a safe procedure in the path of univentricular correction.
Boletin medico del Hospital Infantil de MexicoComplex congenital heart diseases among children presenting for cardiac surgery in a tertiary health facility in Enugu; South-East Nigeria. A rising trend.
Nigerian journal of clinical practiceHigh Birth Prevalence of Congenital Heart Diseases in Conjoined Twins and Higher Order Multiple Births.
The American journal of cardiologyT-stenting and small protrusion technique for left main coronary injury post Bentall procedure.
Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidirH-type tracheo-oesophageal fistula in an infant.
BMJ case reportsTransvenous shock-only implantable cardioverter defibrillator after an atrio-pulmonary Fontan surgery.
Pacing and clinical electrophysiology : PACEOrthotopic Heart Transplantation in a Child with Single Ventricle after Pneumonectomy.
ASAIO journal (American Society for Artificial Internal Organs : 1992)Outcomes after the Fontan operation in the Middle East: A large Saudi Arabian single centre experience.
International journal of cardiologyBest management in isolated right ventricular hypoplasia with septal defects in adults.
Journal of cardiovascular and thoracic researchImaging characteristics and associations in twisted atrioventricular connections on multidetector computed tomography angiography.
Journal of cardiac surgeryDamus-Kay-Stansel procedure with ventricular septal defect enlargement.
Asian cardiovascular & thoracic annalsThe intra-extracardiac Fontan: preliminary results.
Indian journal of thoracic and cardiovascular surgeryA Rare Case Report of Guillain-Barré Syndrome Presenting as Unilateral Facial Palsy with Isolated acute Bulbar Palsy.
Journal of pediatric neurosciencesManagement of cesarean section in a patient with Fontan circulation: a case report of dramatic reduction of maternal oxygen consumption after delivery.
JA clinical reportsMisdiagnosis of Pulmonary embolism in a Fontan's patient: When standard protocol CT Pulmonary angiogram is inadequate.
Radiology case reportsHigher Incidence of Protein-Losing Enteropathy in Patients with Single Systemic Right Ventricle.
Pediatric cardiologyPrevalence rates study of selected isolated non-Mendelian congenital anomalies in the Hutterite population of Alberta, 1980-2016.
American journal of medical genetics. Part ABentall procedure for the repair of a neoaortic aneurysm after the Norwood procedure in a patient with tricuspid atresia and a discordant ventriculo-arterial connection.
Interactive cardiovascular and thoracic surgeryMonochorionic Twin Discordance for Horseshoe Lung and Tricuspid Atresia.
Fetal and pediatric pathologyPrenatal diagnosis of double-outlet right ventricle with tricuspid valve atresia, anomalous pulmonary vein connection, persistent left superior vena cava, and right atrial isomerism.
Journal of clinical ultrasound : JCULearning from a case of right ventricular outflow tract stenting in tricuspid atresia with critical pulmonary stenosis.
Cardiology in the youngCOVID-19 in an Adult With Tricuspid Atresia S/P Fontan Palliation.
JACC. Case reportsA case of tricuspid atresia with Prader-Willi syndrome.
Pediatrics international : official journal of the Japan Pediatric SocietyLarge Coronary Artery Fistula in Tricuspid Atresia - Pulmonary Atresia With Intact Ventricular Septum - A Unique Association Revealed on Dual-Source CT.
Heart, lung & circulationComparison of pleural effusion between fenestrated and nonfenestrated extracardiac Fontan: A prospective randomized study.
Journal of cardiac surgeryBronchial varices in a child with tricuspid atresia six years post Fontan correction.
Respirology case reportsThe Adult Patient with a Fontan.
Cardiology clinicsContributions of residual hypoxemia to exercise hyperventilation in Fontan patients.
Nagoya journal of medical scienceModification of Glenn anastomosis for total cavopulmonary connection conversion after atriopulmonary Fontan.
General thoracic and cardiovascular surgeryHypnosis and superficial cervical anesthesia for total thyroidectomy in a high-risk patient - A case report.
International journal of surgery case reportsA NONSENSE GATA6 MUTATION EXPLAINS HISTORY OF CONGENITAL HEART DEFECTS AND 10 YEARS OF POORLY-CONTROLLED DIABETES LACKING DKA IN A NON-OBESE 30 YEAR-OLD INCIDENTALLY FOUND TO HAVE PANCREATIC HYPOPLASIA.
AACE clinical case reportsTricuspid atresia: Where are we now?
Journal of cardiac surgerySystemic ventricular implantation of a leadless pacemaker in a patient with a univentricular heart and atrioventricular node calcification.
HeartRhythm case reportsMajor aortopulmonary collateral arteries in a case of unrepaired tricuspid and pulmonary atresia with single ventricle physiology.
Journal of cardiovascular computed tomographySingle-Stage Fontan Operation in a Patient With Coronary Sinus Ostium Atresia.
World journal for pediatric & congenital heart surgeryIdentification and analysis of KLF13 variants in patients with congenital heart disease.
BMC medical geneticsAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Atresia tricúspide.
É de uma associação que acompanha esta doença? Fale com a gente →
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Atresia tricúspide
Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.
Tire suas dúvidas
Perguntas, dicas e experiências compartilhadas aqui na página
Participe da discussão
Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.
Fazer loginDoenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
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.
- Generation of three induced pluripotent stem cell clones from a functional single ventricle patient carrying the BRAF c.1897 T > C variant.
- Early outcomes of children with univentricular circulation undergoing Fontan surgery: the EuroFontan registry.
- Development of 3D-Printed Congenital Heart Disease Models Using Feasible Low-Cost Workflow - A Potential Tool to Improve Pediatric Cardiology Education.
- Early Experience with the First Canadian Paediatric Fontan Long-Term Follow-up Clinic.
- Unusual right-to-left shunt in a patient with tricuspid atresia and stenosis of Fontan conduit.
- Single Ventricle: Radiology Primer for Preprocedural Evaluation.
- Delayed Diagnosis of Tricuspid Atresia: A Case Report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:1209(Orphanet)
- OMIM OMIM:605067(OMIM)
- MONDO:0011514(MONDO)
- GARD:5274(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q1283303(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
