A atresia da válvula tricúspide é uma forma de cardiopatia congênita na qual existe, ou a total ausência da válvula tricúspide ou a fusão das comissuras dos três folhetos valvulares. É uma malformação rara, correspondendo a cerca de 3% de todas as cardiopatias congênitas.
Introdução
O que você precisa saber de cara
Malformação congênita da valva tricúspide, podendo apresentar defeitos septais, morfologia cardiovascular anormal e isquemia cerebral. Associada a fadiga, trombose arterial e risco de infarto/AVC.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 26 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 74 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
2 genes identificados com associação a esta condição.
Promotes orthogonal branching of actin filaments and links actin filaments to membrane glycoproteins. Anchors various transmembrane proteins to the actin cytoskeleton and serves as a scaffold for a wide range of cytoplasmic signaling proteins. Interaction with FLNB may allow neuroblast migration from the ventricular zone into the cortical plate. Tethers cell surface-localized furin, modulates its rate of internalization and directs its intracellular trafficking (By similarity). Involved in cilio
Cytoplasm, cell cortexCytoplasm, cytoskeletonPerikaryonCell projection, growth coneCell projection, podosome
Periventricular nodular heterotopia 1
A developmental disorder characterized by the presence of periventricular nodules of cerebral gray matter, resulting from a failure of neurons to migrate normally from the lateral ventricular proliferative zone, where they are formed, to the cerebral cortex. PVNH1 is an X-linked dominant form. Heterozygous females have normal intelligence but suffer from seizures and various manifestations outside the central nervous system, especially related to the vascular system. Hemizygous affected males die in the prenatal or perinatal period.
Myosins are actin-based motor molecules with ATPase activity essential for muscle contraction. Forms regular bipolar thick filaments that, together with actin thin filaments, constitute the fundamental contractile unit of skeletal and cardiac muscle
Cytoplasm, myofibrilCytoplasm, myofibril, sarcomere
Cardiomyopathy, familial hypertrophic, 1
A hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death.
Variantes genéticas (ClinVar)
2,613 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
5 vias biológicas associadas aos genes desta condição.
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 — Malformação tricúspide, congênita
Centros de Referência SUS
24 centros habilitados pelo SUS para Malformação tricúspide, congênita
Centros para Malformação tricúspide, congênita
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.
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Publicações mais relevantes
[Analysis of optimizing indications for fetal pulmonary valvuloplasty based on a multi-parameter scoring system].
Objective: To establish a multi-parametric scoring system for predicting postnatal circulatory outcomes in fetuses with critical pulmonary stenosis (CPS) and pulmonary atresia (PA) with intact ventricular septum (IVS), in order to optimize the indications for fetal pulmonary valvuloplasty (FPV). Methods: This was a retrospective cohort study. A total of 96 fetuses diagnosed with CPS-IVS or PA-IVS at Women and Children's Hospital, Qingdao University between July 2018 and June 2021 were included. Fetuses who did not undergo FPV constituted the modeling cohort, while those who underwent FPV constituted the validation cohort. Fetuses were classified into biventricular and non-biventricular circulation groups based on postnatal circulatory outcomes. Prenatal fetal echocardiographic parameters were compared between groups using independent-samples t test, Mann-Whitney U test, χ2 test or Fisher exact test. Based on inter-group differences, parameters associated with postnatal circulatory outcomes were screened in the modeling cohort. For continuous variables, optimal cut-off values were determined using receiver operating characteristic (ROC) curve and the Youden index and then dichotomized. Right ventricular endocardial fibroelastosis (EFE) was graded as 0, 1 or 2 according to severity. OR of each parameter were calculated, and a multiparametric scoring system was constructed. ROC curve was used to evaluate its predictive performance. Post-FPV outcome improvement was assessed in the validation cohort. Results: Among the 96 fetuses, 78 were assigned to the modeling cohort and 18 to the validation cohort. Postnatally, biventricular circulation was achieved in 56 cases (72%) in the modeling cohort and 13 cases in the validation cohort. In the modeling cohort, the right ventricular long-axis/left ventricular long-axis (RV/LV), tricuspid annulus diameter/mitral annulus diameter (TV/MV) and pulmonary valve annulus/aortic valve annulus (PV/AV) in the non-biventricular group were lower than that in the biventricular group (all P<0.05). The optimal cut-off values for predicting postnatal circulatory outcomes were 0.65, 0.82 and 0.78, respectively. There was statistically significant difference in the distribution of circulatory outcomes between fetuses with grade 2 EFE and those with no or grade 1 EFE (χ2=21.28, P<0.001). Therefore, right ventricular EFE was dichotomized in the model as "none or grade 1" versus "grade 2". Each parameter was assigned a corresponding weight based on its OR value, and an integer score was obtained to construct the scoring system: grade 2 right ventricular EFE was assigned 5 points, RV/LV≤0.65 was assigned 2 points, TV/MV≤0.82 was assigned 2 points, and PV/AV≤0.78 was assigned 1 point, with a maximum total score of 10 points. At cut-off scores of 3, 4, and 5 points, the areas under the curve were 0.88, 0.91, and 0.88, respectively, with the highest specificity (0.95, 95% CI 0.84-0.99) observed at a score threshold of 5 points. In the validation cohort, 15 fetuses had scores ≥5 points, among whom 10 fetuses achieved biventricular circulation after birth, whereas in the modeling cohort, only 1 of 18 fetuses achieved biventricular circulation after birth. Conclusions: A multiparametric scoring system based on prenatal fetal echocardiographic parameters can effectively predict non-biventricular postnatal outcomes in fetuses with CPS-IVS and PA-IVS. By identifying high-risk patients, the indications for FPV can be optimized. 目的: 建立预测胎儿危重型肺动脉狭窄(CPS)和肺动脉闭锁(PA)伴完整室间隔(IVS)出生后循环结局的多参数评分系统,以优化胎儿肺动脉球囊扩张术(FPV)手术指征。 方法: 回顾性队列研究。纳入2018年7月至2021年6月青岛大学附属妇女儿童医院诊断为CPS-IVS和PA-IVS的96例胎儿为研究对象。未接受FPV的胎儿作为建模组,接受FPV的胎儿为验证组。按生后是否实现双心室循环分为双心室组和非双心室组,比较出生前胎儿超声心动图指标,组间比较采用独立样本t检验、Mann-Whitney U检验、χ2检验或Fisher 确切概率法。依据组间差异筛选建模组中与生后循环结局相关的指标,其中计量资料通过受试者工作特征曲线及Youden指数确定最佳截断值并二分类化,右心室心内膜纤维弹力增生(EFE)按程度分为0、1、2级。计算各参数OR并构建多参数评分系统。应用受试者工作特征曲线评价其预测能力。观察验证组FPV后的结局改善情况。 结果: 96例胎儿中建模组78例、验证组18例,分别56例(72%)、13例生后实现双心室循环。建模组中非双心室患儿右心室长轴径/左心室长轴径(RV/LV)、三尖瓣环直径/二尖瓣环直径(TV/MV)、肺动脉瓣环直径/主动脉瓣环直径(PV/AV)均低于双心室患儿(均P<0.05),其预测出生后循环结局的最佳截断值分别为0.65、0.82、0.78。右心室2级EFE与无或1级EFE胎儿在循环结局的分布上差异有统计学意义(χ2=21.28,P<0.001),因此在模型中将右心室EFE二分类为“无或1级”与“2级”。各项参数按其OR值分配相应权重并取整得分后构建评分系统:右心室EFE为2级记5分,RV/LV≤0.65记2分,TV/MV≤0.82记2分,PV/AV≤0.78记1分,总分最高为10分。评分系统在3、4和5分的界值时,曲线下面积分别为0.88、0.91和0.88,评分界值为5分时特异度最高(0.95,95%CI 0.84~0.99)。验证组中,15例评分≥5分,其中10例生后实现双心室循环,而建模组18例中仅1例实现双心室循环。 结论: 基于胎儿期超声心动图多参数构建的评分系统可有效预测CPS-IVS和PA-IVS胎儿出生后的非双心室结局。通过识别高风险者,可优化FPV适应证。.
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.
Reconstruction of an unguarded tricuspid orifice using a simplified sliding plasty technique.
Unguarded tricuspid orifice is a rare anomaly of the tricuspid valve characterized by complete absence of tricuspid valve tissue and chordae on a proportion of the annulus. Management strategies vary widely. We report a case of successful repair of an unguarded tricuspid orifice with a simplified technique. A 35-year-old male presented with severe tricuspid regurgitation and right ventricular volume overload. Intraoperative inspection of the valve revealed an unguarded tricuspid orifice. For repair, sliding plasty of anterior and posterior leaflets were performed, followed by ring annuloplasty and commissuroplasty. Postoperative, echocardiogram showed minimal residual tricuspid regurgitation and significantly improved right ventricular dimensions. This case highlights the possibility of successful repair of an unguarded tricuspid orifice. If feasible, repair can be a good choice.
Vertical right axillary thoracotomy: strategic insights for reproducible outcomes.
Vertical right axillary thoracotomy (VRAT) has become a useful minimally invasive technique for treating certain congenital and acquired heart conditions. By making a vertical cut along the right axillary line, VRAT provides excellent access to the heart while reducing chest wall trauma and improving cosmetic results. It has been effectively used for repairing atrial septal defects, partial anomalous pulmonary venous return, tricuspid valve issues, and some aortic and left-sided lesions in suitable patients. Although these benefits are clear, broader adoption is constrained by concerns about limited visibility and unfamiliar surgical angles. Nevertheless, growing experience shows that, with careful patient selection, proper planning, and adherence to standardized procedures, VRAT can be performed safely and achieve outcomes comparable to those of traditional sternotomy. It also reduces post-operative pain, shortens recovery time, and improves patient satisfaction. As demand for less invasive cardiac surgeries increases, VRAT stands out as a valuable and adaptable surgical option in modern minimally invasive practice.
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Annals of biomedical engineeringPerioperative management of a woman with Ebstein's anomaly undergoing caesarean section.
BMJ case reportsDevelopment of a Polymeric TAVR Device Tailored to Bicuspid Aortic Valve Patients Using In Silico Design Optimization and Evaluation.
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Interdisciplinary cardiovascular and thoracic surgeryElectrocardiographic and imaging findings in adult patients with congenitally corrected transposition of the great arteries: Case series.
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Computers in biology and medicineAortic Valve Function and Aortic Dilatation Associated with Bicuspid Aortic Valve in Early Childhood.
Journal of the American Society of Echocardiography : official publication of the American Society of EchocardiographyAssociation of Maternal Cardiac Disease and Echocardiographic Parameters With Neonatal Outcomes.
The American journal of cardiologyParkes Weber syndrome, a rare case of pulmonary hypertension: a case report.
European heart journal. Case reportsRole of the Right Ventricle Overhaul Procedure in Comprehensive Management for Pulmonary Atresia With Intact Ventricular Septum - A Magnetic Resonance Imaging-Based Study.
Circulation journal : official journal of the Japanese Circulation SocietySurgical Repair Of Severe Mitral And Tricuspid Valve Regurgitation In Patient With Left Ventricle Noncompaction - A Case Report.
Portuguese journal of cardiac thoracic and vascular surgeryIncidental Discovery of a Right Atrial Diverticulum in an Adult Patient.
Diagnostics (Basel, Switzerland)Percutaneous interventions in tricuspid valve disease: a new era in cardiac treatment.
Annals of medicine and surgery (2012)Outcomes of Fontan patients undergoing combined heart-liver transplantation in pediatric hospitals across the United States.
The Journal of thoracic and cardiovascular surgeryCardiovascular images: parachute mitral valve in a cat.
Journal of veterinary cardiology : the official journal of the European Society of Veterinary CardiologyEbstein anomaly update: the evolution of care and the revolution of surgical strategy.
Expert review of cardiovascular therapyAnalysis of Echocardiography and Risk Factors Related to Prognosis in Adult Patients with Isolated Congenitally Corrected Transposition of the Great Arteries.
Journal of clinical medicineThe Role of Right Ventricular Embryology in Echocardiographic Assessment of RV Function.
Echocardiography (Mount Kisco, N.Y.)A case of fatal infective endocarditis in uncorrected tetralogy of Fallot with persistent left superior vena cava.
Journal of forensic sciencesThe Predictive Role of Prenatal Echocardiography in the Diagnosis of Postnatal Pulmonary Stenosis.
Pediatric cardiologySupravalvar Ring of the Tricuspid Valve in a Patient With Congenitally Corrected Transposition of the Great Arteries.
CASE (Philadelphia, Pa.)Early echocardiographic predictors of death or early severe morbidity in extremely low gestational age neonates: a prospective study.
European journal of pediatricsFetal Conduction Disease and Arrhythmia in Ebstein's Anomaly and Tricuspid Valve Dysplasia Assessed by Fetal Magnetocardiography.
Journal of the American Heart AssociationIsolated Mild Fetal Tricuspid Regurgitation in Low-Risk Pregnancies: An Incidental Doppler Finding or a Marker of Postnatal Cardiac Risk?
Children (Basel, Switzerland)A case of heart failure due to multiple late complications after repair of tetralogy of Fallot in adulthood.
Journal of cardiology casesCommon Echocardiographic Diagnoses in Patients at Tertiary Care Center: An Observational Study.
JNMA; journal of the Nepal Medical AssociationLeft 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 therapyHypoplastic left heart syndrome with mitral regurgitation: a new marker of poor fetal outcome.
Journal of ultrasonographyLong-Term Impact of Tricuspid Valve Intervention on Survival, Tricuspid Valve, and Ventricular Function in Hypoplastic Left Heart Syndrome.
The Annals of thoracic surgeryAnaesthesia 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 surgeryCARTO UNIVU-Guided Micra Implantation in Ebstein Anomaly.
JACC. Case reportsReversed septal motion in congenital corrected transposition of the great arteries: A potential mechanism for tricuspid regurgitation.
The Journal of thoracic and cardiovascular surgeryAssessment of tricuspid annulus geometry in children with congenital heart disease using three-dimensional echocardiography.
Revista espanola de cardiologia (English ed.)Shifting strategies to address systemic outflow tract obstruction in single ventricle anatomy with transposed great arteries.
The Journal of thoracic and cardiovascular surgeryUnderstanding the hemodynamic changes in fetuses with coarctation of the aorta using a lumped model of fetal circulation.
PLoS computational biologyEbstein Repair in a High-Altitude Setting ≥2,500 m: First Experience from Bolivia.
The Thoracic and cardiovascular surgeonCone reconstruction after carpentier repair in ebstein anomaly: yes we can!
Journal of cardiothoracic surgeryRight and left ventricular filling in adult patients with repaired tetralogy of fallot: a 4D flow MRI study.
The international journal of cardiovascular imagingPrenatal Ultrasound Diagnosis of Fetal Type IV Intrahepatic Portosystemic Shunt: A Rare Case Report.
Journal of clinical ultrasound : JCUExercise Performance and Right Ventricular Remodeling After the Cone Repair in Patients With Different Severity of Ebstein Anomaly.
The Annals of thoracic surgeryClarifying the cardiovascular morphology and associated abnormalities in patients with tricuspid Atresia using multidetector computed tomography angiography.
The international journal of cardiovascular imagingAge Differences in Aortic Stenosis.
Reviews in cardiovascular medicineBicuspid aortic valve disease: advancements and challenges of transcatheter aortic valve implantation.
European heart journalRisk factor analysis for cardiac abnormalities in patients with idiopathic scoliosis.
Scientific reportsAsymptomatic Isolated Congenitally Corrected Transposition of the Great Arteries in a 25-Year-Old Male: A Case Report.
Journal of investigative medicine high impact case reportsSurgical Aortic Valve Replacement For Bicuspid And Tricuspid Valve Disease: 7-Year Outcomes In >1100 Patients.
Portuguese journal of cardiac thoracic and vascular surgeryBicuspid Aortic Valve among Children Presenting in Two Health Institutions in Enugu, South-East Nigeria.
Nigerian journal of clinical practice2025 American Association for Thoracic Surgery Congenital Cardiac Surgery Working Group- Expert consensus document on the management of patients with pulmonary atresia with intact ventricular septum.
The Journal of thoracic and cardiovascular surgeryThe American Association for Thoracic Surgery (AATS) 2025 Expert Consensus Document: Management of Ebstein anomaly in children and adults.
The Journal of thoracic and cardiovascular surgerySex-Specific Outcomes in Patients Undergoing Transcatheter Closure of Atrial Septal Defects: Do They Benefit Equally?
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & InterventionsEfficacy and Safety of the LifeTech™ Multifunctional Occluder (Konar-MFO) in Transcatheter Closure of Ventricular Septal Defects: A Systematic Review and Meta-Analysis.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & InterventionsAcquired bilateral atrio-ventricular valvular stenosis in a complex single-ventricle physiology following superior cavo-pulmonary connection.
BMJ case reportsA case of complete atrioventricular septal defect in which extracorporeal membrane oxygenation could be removed after performing the bilateral Glenn procedure for severe cardiac dysfunction after tricuspid valve replacement: a case report.
BMC pediatricsNormal fetal echocardiography ratios - a multicenter cross-sectional retrospective study.
Journal of perinatal medicineSurgical Management of a Patient With Non-Fallot-Type Absent Pulmonary Valve Syndrome.
World journal for pediatric & congenital heart surgeryPractical approach to using cardiac magnetic resonance imaging for pre-surgical planning in complex paediatric congenital heart disease.
Pediatric radiologyPrenatal Genetic Diagnosis of Williams-Beuren Syndrome with Atypical and Complex Phenotypes.
Clinical laboratoryCardiac function in children with congenital adrenal hyperplasia.
Journal of pediatric endocrinology & metabolism : JPEMPresenting novel 2D echocardiographic views for pulmonary valve assessment in tetralogy of fallot patients.
BMC cardiovascular disordersRuptured aneurysm of the non-coronary sinus of Valsalva with detachment of the tricuspid valve annulus: an unusual complication of a rare cardiac anomaly.
The international journal of cardiovascular imagingDiagnostic Value of Echocardiographic Indices in Determining Right Atrial Pressure Compared to Catheterization in Pediatrics and Young Adults.
Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidirConcurrent Persistent Truncus Arteriosus and Left Atrial Diverticulum in a Domestic Short-Haired Cat.
Animals : an open access journal from MDPISecond trimester echocardiography.
Best practice & research. Clinical obstetrics & gynaecologyFate of Residual Patch Leak After Surgical Closure of Congenital Perimembranous Ventricular Septal Defects.
World journal for pediatric & congenital heart surgeryPathophysiology and management of adults with complex congenital heart disease after biventricular repair.
Journal of cardiologyRole of tricuspid valve repair in pulmonary atresia with intact ventricular septum: fresh autologous pericardium as a viable option.
Interdisciplinary cardiovascular and thoracic surgeryCorrelation between T-lymphocyte subsets and hemodynamics in patients with congenital heart disease-associated pulmonary arterial hypertension.
MedicineWhole-Exome Sequencing Identifies Novel GATA5/6 Variants in Right-Sided Congenital Heart Defects.
International journal of molecular sciencesEndocardial-to-mesenchymal transition underlies cardiac outflow tract septation and bicuspid aortic valve formation in the Syrian hamster model.
Scientific reportsGenotype-Phenotype Correlations, Treatment, and Prognosis of Children With Early-Onset (Neonatal) Marfan Syndrome.
Clinical geneticsPulmonary artery systolic pressure associated with inflammatory factors among pediatric congenital heart disease with pulmonary arterial hypertension after cardiopulmonary bypass.
Jornal de pediatriaEvaluation of first-trimester ultrasound screening strategy for fetal congenital heart disease.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyVentricular Assist Device Implantation in a Patient Congenitally Corrected Transposition of the Great Arteries With I, D, D.
ASAIO journal (American Society for Artificial Internal Organs : 1992)De Novo Deletion in the 12q24.23q24.31 Chromosomal Region Causing a Neurodevelopmental Syndrome in a Female Saudi Patient: A Case Report.
CureusClosure of perimembranous ventricular septal defects using the Amplatzer vascular plug II: experience of a Mexican centre.
Cardiology in the young[Analysis of 15 cases of ductus arteriosus stent placement without a guiding catheter through femoral artery approach].
Zhonghua er ke za zhi = Chinese journal of pediatricsNative Tricuspid Valve Endocarditis Delayed Glenn Procedure: A Case Report.
World journal for pediatric & congenital heart surgeryA Cardiocraniofacial Syndrome Associated With a Novel Missense Variant in GATA6: A Fetal Case Report.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyLeft Bundle Branch Pacing in a Pediatric Patient With Tricuspid Valve Replacement for a Complex Ebstein Anomaly.
Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, IncComplex congenital heart and lung defects as a cause of hydrops fetalis in French bulldogs -micro-CT with contrast study.
Scientific reportsCharacterization and Localization of Focal Atrial Tachycardia in Patients With Adult Congenital Heart Disease.
JACC. Clinical electrophysiologyPrevalence, predictors, and mortality of Super-Fontan patients in a single-centre nationwide cohort.
Cardiology in the youngIntrahepatic portosystemic shunt: salvage mechanism for oligohydramnios complicating fetal growth restriction.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyThe Role OF 3D echocardiography in prediction of pulmonary vascular resistance and its reversibility in simple congenital heart disease with secondary pulmonary hypertension.
Current problems in cardiologyHeart Transplantation Outcomes for Fontan Patients When Using Contemporary Strategies: Heart-Liver Transplantation and Ventricular Assist Device Therapy.
World journal for pediatric & congenital heart surgeryEbstein's anomaly in children and young adults: clinical features, arrhythmia, surgical management, and factors affecting arrhythmia and mortality.
Cardiology in the youngEchocardiographic predictors of surgical outcomes in pulmonary atresia with intact ventricular septum and development of a discriminatory calculator: an Australian tertiary institution experience.
Cardiology in the youngUsefulness of the "En face view" method for diagnosing perimembranous ventricular septal defects.
Journal of echocardiographyA Rare Surgical Case of Isolated Double-Chambered Right Ventricle Without Cardiac Malformations in an Older Female Patient.
CureusA 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 CenterComputer-generated Clinical Decision-making in the Treatment of Pulmonary Atresia with Intact Ventricular Septum.
Cardiovascular engineering and technologyCircular Shunt: A Loop Not to Be Ignored.
Journal of cardiothoracic and vascular anesthesiaThoracic Lymphatic Perfusion Patterns Assessed by Magnetic Resonance Imaging and Late Fontan Failure.
Diagnostics (Basel, Switzerland)Ventricular septal defect characteristics influence presence of septal anatomical isthmuses in patients with repaired tetralogy of Fallot.
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 CardiologyEchocardiographic Changes in Infants with Severe Congenital Diaphragmatic Hernia After Fetoscopic Endoluminal Tracheal Occlusion (FETO).
Pediatric cardiologyEvaluation of Postnatal Echocardiographic Screening for Congenital Heart Defects - a Prospective Cohort Study at a German University Hospital.
Klinische Padiatrie[Patch Closure of the Ventricular Septal Defect].
Kyobu geka. The Japanese journal of thoracic surgery[Techniques of Atrial, Ventricular Incision and Closure].
Kyobu geka. The Japanese journal of thoracic surgeryCardiovascular profile score (CVPS) and selected cardiac parameters in fetuses with Vein of Galen malformation.
Ginekologia polskaSurgical strategies in Ebstein anomaly: 28 years' experience in a pediatric hospital.
Archivos de cardiologia de MexicoCase report of Ebstein's anomaly in a young female.
SAGE open medical case reportsEarly dehiscence of a tricuspid valve annuloplasty ring in an adolescent with hypoplastic left heart syndrome presenting with unconjugated hyperbilirubinemia.
Cardiology in the youngCross-Sectional Study of the Association Between Plasma Brain Natriuretic Peptide Levels and Left Ventricular Shortening Fraction in Fukuyama Congenital Muscular Dystrophy.
The Tohoku journal of experimental medicineManagement of Tricuspid Atresia With Normally Related Great Arteries and Left-Sided Obstruction.
World journal for pediatric & congenital heart surgeryA prediction model of echocardiographic variables to screen for potentially correctable shunts in adult atrial septal defect-pulmonary hypertension patients.
Journal of the Chinese Medical Association : JCMAPrimary cardiac myxofibrosarcoma in a patient with Ebstein's anomaly: First reported case.
International journal of surgery case reportsA Noninvasive Prediction Model With Simple Echocardiographic Variables for Shunts Closure Possibility in Patients With Posttricuspid Valve Shunt Defect.
Echocardiography (Mount Kisco, N.Y.)Combined Norwood and Modified Left-Sided Starnes Procedures for Congenitally Corrected Transposition of the Great Arteries.
World journal for pediatric & congenital heart surgeryEchocardiographic Screening Model for Improved Assessment of Atrial Septal Defect Closure: A Multicenter Retrospective Study.
Echocardiography (Mount Kisco, N.Y.)Ventricular Dysfunction in Patients With Congenital Diaphragmatic Hernia Who Die After Repair.
Journal of pediatric surgeryEffect of aortic valve phenotype and sex on aorta dilation in patients with aortic stenosis.
Open heartOutcomes in Adult Congenital Heart Disease Patients With Down Syndrome Undergoing a Cardiac Surgical Procedure.
The Annals of thoracic surgeryLongitudinal changes in systemic right ventricular remodeling in adult patients with transposition of the great vessels as assessed by cardiovascular magnetic resonance imaging.
Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic ResonanceComparison of pulmonary artery growth between ductus stent and systemic-to-pulmonary shunt as single-ventricle palliation.
The Journal of thoracic and cardiovascular surgeryPulmonary valve replacement-A 10-year single-center surgical experience in ACHD patients.
PloS oneEbstein's Anomaly in a Young Nigerian Lady.
West African journal of medicinePathogenesis and Surgical Treatment of Congenitally Corrected Transposition of the Great Arteries (ccTGA): Part III.
Journal of clinical medicineDouble Outlet Left Ventricle with Intact Ventricular Septum: A Rare Prenatally Diagnosed Case Report.
Fetal diagnosis and therapyMultimodality imaging and functional assessment in patients with systemic right ventricle and biventricular physiology: a retrospective single-center study.
Monaldi archives for chest disease = Archivio Monaldi per le malattie del toraceSingle ventricle palliation in congenitally corrected transposition of the great arteries: An international multicenter study.
The Journal of thoracic and cardiovascular surgeryDouble-outlet Right Atrium with Double-orifice Tricuspid Valve: Report of a Rare Case and Review of the Literature.
Pediatric cardiologyDiagnosis of Congenital Heart Disease in Adulthood: How Often, How Relevant?
The American journal of cardiologyA Comparison of Active and Passive Surveillance Strategies for Selected Birth Defects in New York.
Birth defects researchImproved Risk Prediction Using a Refined European Guidelines Instrument in Pulmonary Arterial Hypertension Related to Congenital Heart Disease.
The American journal of cardiologyPathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II.
Journal of clinical medicineMidterm follow-up results of implantation of a fully biodegradable ventricular septal defect occluder.
Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciencesComparison Between Histidine-Tryptophan-Ketoglutarate Cardioplegia and Cold Blood Cardioplegia for Myocardial Protection in Tetralogy of Fallot Patients Undergoing Surgical Repair: A Randomized Clinical Trial.
Journal of cardiothoracic and vascular anesthesiaA surviving neonate with congenitally unguarded tricuspid valve orifice.
Journal of clinical ultrasound : JCURight ventricular function in Marfan patients remains stable despite multiple cardiac interventions.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryA predictable electrocardiogram.
Journal of electrocardiologyCalcific Aortic Stenosis - Inflammatory Disease.
Ceskoslovenska patologieEstablishment of a new human iPSC cell line (UOMi012-A) from a patient with congenital heart defect who has undergone Fontan procedure.
Stem cell researchLarge Right Atrial Size on Cardiac MRI is Associated with Post-operative Right Ventricular Dysfunction After the Cone Operation for Ebstein Anomaly.
Pediatric cardiologyMagnitude of Pulse Pressure Variation is Associated with Qp:Qs Imbalance during Pediatric Cardiac Surgery: A Two-Center Retrospective Study.
Reviews in cardiovascular medicinePremature Closure of the Ductus Arteriosus and Veno-Arterial Extracorporeal Membrane Oxygenation in Critically Ill Neonates: A 10-Year Single-Center Retrospective Study.
Pediatric cardiologyOutcomes following multivalve reoperation in adults with congenital heart disease: A 30-year, single-center study.
The Journal of thoracic and cardiovascular surgeryA true bicuspid tricuspid valve: 3D imaging to better understanding the forgotten valve.
The international journal of cardiovascular imagingParachute tricuspid valve unique case and the first case in Somalia.
Radiology case reportsIncreased Diastolic Energy Loss Associated with Cardiac Events in Adults with Pulmonary Atresia Suffering from Intact Ventricular Septum.
Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in MedicineA 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)Associações
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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.
- [Analysis of optimizing indications for fetal pulmonary valvuloplasty based on a multi-parameter scoring system].
- 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.
- Reconstruction of an unguarded tricuspid orifice using a simplified sliding plasty technique.
- Vertical right axillary thoracotomy: strategic insights for reproducible outcomes.
- Congenital Tricuspid Valve Cleft Diagnosis Several Years after Pulmonary Valve Intervention.
- Repair of Severe Tricuspid Regurgitation in a Neonate With Intrauterine Tricuspid Valve Chordae Tendinae Rupture.
- Arrhythmias in Congenital Heart Disease: Ebstein Anomaly.
- Tricuspid Valve Repair in Pediatric Patients Using Artificial Neochordae: Systematic Review of Current Evidence.
- Congenital Tricuspid Valve Stenosis with Small Ostium Secundum Atrial Septal Defect and Ventricular Septal Defect Eisenmenger Complex.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:98721(Orphanet)
- MONDO:0020289(MONDO)
- GARD:19555(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q55789283(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
