A malformação de Ebstein é uma anomalia cardíaca congênita rara caracterizada pelo deslocamento rotacional dos folhetos septais e inferiores da válvula tricúspide, de modo que eles fiquem articulados dentro do ventrículo direito, e não como esperado na junção atrioventricular.
Introdução
O que você precisa saber de cara
A malformação de Ebstein é uma anomalia cardíaca congênita rara caracterizada pelo deslocamento rotacional dos folhetos septais e inferiores da válvula tricúspide, de modo que eles fiquem articulados dentro do ventrículo direito, e não como esperado na junção atrioventricular.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 13 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 30 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
1 gene identificado com associação a esta condição. Padrão de herança: Autosomal dominant, Not applicable.
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)
1,439 variantes patogênicas registradas no ClinVar.
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 — Anomalia de Ebstein da válvula tricúspide
Centros de Referência SUS
24 centros habilitados pelo SUS para Anomalia de Ebstein da válvula tricúspide
Centros para Anomalia de Ebstein da válvula 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.
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Publicações mais relevantes
Septal Leaflet Augmentation During the Da Silva Cone Repair: Valvar Function and Anatomical Features Compared to Non-Patch Repair.
To evaluate outcomes of Da Silva Cone repair (CR) using autologous pericardial patch for septal leaflet augmentation in Ebstein's anomaly (EA) and identify tricuspid valve (TV) morphology associated with patch use. A single-site retrospective study included patients undergoing CR for EA from Jan 2016-Nov 2024. Patients with prior Starnes procedure or TV surgery were excluded. Patients were categorized into patch and non-patch groups. The primary outcome was TV function (stenosis or regurgitation) at latest follow-up with a secondary composite outcome including re-operation or >moderate residual tricuspid regurgitation (TR). Adjusted regression analyses identified factors associated with outcomes and patch augmentation. Of 134 patients identified, 108 were included (50 patch, 58 non-patch). Patch patients were older (13.1 vs. 6.9 years, p=0.04). TR improved from ≥moderate in 78.7% pre-CR to ≤mild in 79.0% at follow-up. TV function and follow-up TV-related re-operation rates (6.0% vs. 1.7%) were comparable between groups (p>0.05) with a median follow-up of 1.1 years (IQR 0.6-3.2). Intraoperative factors associated with patch use were multiple TV orifices (OR=4.24, 95%CI: 1.59-11.27, p<0.01), absence of severe inferior leaflet displacement (OR=3.24, 95%CI: 1.11-9.47, p=0.03), and absence of extreme TV rotation (OR=3.19, 95%CI: 1.21-8.41, p=0.02). Autologous pericardial patch augmentation of the septal leaflet provides comparable early TV function and freedom from re-operation, making it an effective complement during CR.
Biventricular Repair in Symptomatic Neonates with Ebstein Anomaly: 25-30 years of follow-up.
The first report of successful biventricular repairs of Ebstein Anomaly in the severely symptomatic neonate was published in 2000 and 2002. We sought to ascertain the long-term follow-up of the survivors over the ensuing 25-31 years. Eight consecutive neonates (n=6) and young infants (n=2) underwent primary biventricular repair of Ebstein anomaly between April 1994 and March 2001 by one surgeon at the Children's Hospital of Oklahoma. Complete follow-up of all patients was achieved in August 2025 by personal contact with the patients, their parents and/or their cardiologists. All patients gave consent to their data to be included in this review. Three neonates had associated pulmonary atresia and two had functional pulmonary atresia, one with circular shunt and cardiogenic shock. Two had an associated ventricular septal defect. One 2.5-month-old ventilator-dependent neonate had undergone a Starnes' single ventricle palliation elsewhere. All patients had severe tricuspid regurgitation and Great Ormond Street Echocardiography (GOSE) scores grade III (n=2) or IV (N=6). Three neonates had symptomatic tachyarrhythmias prior to surgery. Simplified biventricular repair was done in all 8 patients and all associated cardiac defects were repaired. There was one hospital death (1/8, 12.5%) in a 2.1kg neonate with anatomical pulmonary atresia. All 7 hospital survivors are currently alive and well. The transplant-free survival at 25-31-year follow-up of the hospital survivors (n=7) was 100%. The reoperation-free survival was 75% at 15 years and 43% at 25 years follow-up. No patient developed tachyarrhythmias postoperatively; one patient developed atrial fibrillation at 22 years follow-up. Biventricular repair in symptomatic neonates with Ebstein anomaly is feasible and associated with excellent long-term outcomes. Re-repair or replacement of the tricuspid valve can be anticipated over a follow-up period of 15-30 years.
Risk factors for morbidity and mortality in Ebstein's anomaly: a registry-based study of 398 patients.
To investigate the long-term outcomes and risk factors for morbidity and mortality in patients with Ebstein's anomaly, including the effects of type and timing of valve surgery. For this retrospective, record-based study, all patients with Ebstein's anomaly enrolled in the German National Register for Congenital Heart Defects up to June 2021 were eligible for inclusion. Non-surgical patients (n = 194/49% of 398 patients) had less tricuspid valve regurgitation (p < 0.001) and heart failure symptoms (p < 0.001) than surgical patients (n = 204/51%). Postoperative survival at 10, 20, and 30 years was 97%, 93%, and 80%. Eighty-one (40%) patients underwent multiple surgeries. Re-operation rates were lowest in patients with first valve surgery during adolescence (p = 0.0076). Postoperative NYHA class > I was more frequent with surgery delayed to older age (p < 0.001). Initial corrective surgery was complicated by complete atrioventricular block (CAVB) in 17 (9%) of patients. CAVB was more likely with older age (p = 0.001), and tricuspid valve replacement compared to reconstruction (p = 0.029). CAVB was associated with all-cause death (p < 0.001). Cone reconstruction reduced the risk of CAVB (p = 0.008) and tricuspid valve regurgitation (p < 0.001) compared to monocusp reconstruction. This registry-based study of Ebstein's anomaly corroborates good surgical long-term results, while re-operation rates remain high. Patients operated before adolescence were at the highest risk of re-operation, while older age at the time of the first surgery increased the risk of CAVB. The cone reconstruction was associated with improved tricuspid valve function and a lower risk of CAVB compared to monocusp reconstructions. Choosing an optimal time window for surgery and use of the cone reconstruction may therefore further improve outcomes.
Changing Course-Starnes Takedown in High-Risk Children and Review of the Current Literature.
Neonates with Ebstein anomaly or severe tricuspid valve dysplasia represent a high-risk patient cohort. The modified Starnes procedure was historically performed for cases incompatible with biventricular (BV) repair, leading them towards single-ventricle palliation. However, in recent years, a paradigm shift has emerged in which the Starnes procedure in the acute setting can provide a means for right ventricular (RV) rehabilitation, by providing relief of RV volume overload and time for recovery of RV function, thus serving as a bridge to subsequent one and a half ventricle or BV repair. Herein, we present two cases at our institution of successful transition to BV repair after the Starnes procedure, despite an initially failed index BV repair as a neonate. Both patients presented with severe tricuspid valve dysplasia. The first patient underwent Starnes procedure at 10 days of life and takedown at 4 years of age after bilateral cavopulmonary shunt (BCPS) failure from recurrent bilateral pulmonary artery stenoses. The 2nd patient received Starnes at 4 months of age and successfully underwent takedown at 18mo after being deemed an unsuitable candidate for BCPS. Improvement in RV function was noted throughout the Starnes course. By providing our center's experience in the context of other case reports and series of BV conversion post-Starnes procedure, summarizing clinical and surgical considerations advocated in recent guidelines, and highlighting the topical areas of management that remain to be elucidated, we hope this discussion can lead to appropriate adoption of this new treatment strategy.
Use of right atrial wall to repair severely dysplastic tricuspid valve in an infant with Ebstein's anomaly.
The management of infants with Ebstein's anomaly is challenging and requires complex involvement of the multidisciplinary team. Surgical repair technique is dependent on the degree of tricuspid leaflet dysfunction and the decision to undergo univentricular or biventricular repair. Patients with a severely dysplastic tricuspid valve are less suited to a cone repair alone and require leaflet reconstruction. Currently used patch materials include autologous untreated pericardium and treated pericardium, with limitations such as unpredictable shrinkage and no growth potential, respectively. To overcome these challenges, we used living autologous wall of the right atrium to reconstruct the tricuspid valve leaflets in a 1-year-old girl with Ebstein's anomaly and a severely dysplastic tricuspid valve.
Publicações recentes
Left ventricular outflow tract obstruction after Ebstein tricuspid valve surgery: a case report.
📖 RevisãoCircular Shunt: A Loop Not to Be Ignored.
🥉 Relato de casoCase report of Ebstein's anomaly in a young female.
Heterozygous nonsense variants in laminin subunit 3α resulting in Ebstein's anomaly.
Impact of Cone vs. Hetzer Repair on Postoperative Outcomes in Patients With Ebstein's Anomaly: 10-Year Experience From a Single Institution.
📚 EuropePMC3 artigos no totalmostrando 198
Left bundle branch area pacing from the functional right ventricle in Ebstein's anomaly with atrioventricular block.
HeartRhythm case reportsSeptal Leaflet Augmentation During the Da Silva Cone Repair: Valvar Function and Anatomical Features Compared to Non-Patch Repair.
The Journal of thoracic and cardiovascular surgeryBiventricular Repair in Symptomatic Neonates with Ebstein Anomaly: 25-30 years of follow-up.
The Journal of thoracic and cardiovascular surgeryRisk factors for morbidity and mortality in Ebstein's anomaly: a registry-based study of 398 patients.
Clinical research in cardiology : official journal of the German Cardiac SocietyChanging Course-Starnes Takedown in High-Risk Children and Review of the Current Literature.
Pediatric cardiologyUse of right atrial wall to repair severely dysplastic tricuspid valve in an infant with Ebstein's anomaly.
Multimedia manual of cardiothoracic surgery : MMCTSClinical and Imaging Predictors of Poor Prognosis in Ebstein Anomaly.
CJC pediatric and congenital heart diseaseTimely surgical intervention and risk stratification in patients with Ebstein anomaly: a 20-year retrospective cohort study.
International journal of surgery (London, England)Systemic atrioventricular valve replacement due to a supravalvular stenosing ring and ebsteinoid tricuspid valve in a patient with congenitally corrected transposition of the great arteries: a case report.
Archivos peruanos de cardiologia y cirugia cardiovascularBiventricular remodeling and function after Cone repair of Ebstein Anomaly: A review.
International journal of cardiology. Congenital heart diseaseTranscatheter Tricuspid Valve-in-Valve Replacement in a Patient with Ebstein Anomaly.
Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidirCardiovascular Profile Score and Perinatal Survival Among Fetuses With Ebstein's Anomaly or Tricuspid Valve Dysplasia: A Multi-Center Retrospective Cohort Study.
Prenatal diagnosisAnatomical severity of Ebstein's anomaly: a quantitative analysis based on cardiovascular MRI.
Open heartFirst surgical epicardial implantation of an extravascular implantable cardioverter-defibrillator lead for Ebstein anomaly in the United States.
HeartRhythm case reportsPrimary Tricuspid Regurgitation: From Neglect to Clinical Relevance.
Journal of personalized medicineCatheter ablation of macro-reentrant atrial tachycardia in adults with Ebstein anomaly: A narrative review.
Heart rhythm O2Perioperative management of a woman with Ebstein's anomaly undergoing caesarean section.
BMJ case reportsVersatility of the right atrial wall for tricuspid valve repair in children with Ebstein anomaly.
The Journal of thoracic and cardiovascular surgeryCone repair and right ventricular resection in an adult patient with Ebstein's anomaly.
Multimedia manual of cardiothoracic surgery : MMCTSEbstein 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 SocietyArrhythmias in Congenital Heart Disease: Ebstein Anomaly.
Cardiac electrophysiology clinicsFirst Confirmed Case of Leadless Pacemaker Insertion via Bidirectional Glenn Shunt in Ebstein's Anomaly: A Novel Approach to Pacing Challenges.
Journal of cardiovascular electrophysiologyEchocardiographic characteristics of 14 cases with uncommon manifestation of Ebstein anomaly in the fetus: a case series report.
Archives of gynecology and obstetricsA multifaceted clinical portrait of Ebstein's anomaly: a case series.
European heart journal. Case reportsSurgically Treated Giant Right Atrium in Isolated Severe Tricuspid Regurgitation.
World journal for pediatric & congenital heart surgeryOutcomes of the Fontan operation in patients with Ebstein anomaly: An Australia and New Zealand Fontan registry study.
JTCVS openSurgical Management of Ebstein's Anomaly in Elderly Patients: A Report of Two Cases.
CureusEbstein Anomaly and Atrial Conduction: Big Does Not Mean Bad.
Circulation. Arrhythmia and electrophysiologyInterventricular septal hematoma following cone repair for Ebstein's anomaly.
Indian journal of thoracic and cardiovascular surgeryEbstein anomaly update: the evolution of care and the revolution of surgical strategy.
Expert review of cardiovascular therapyFetal Conduction Disease and Arrhythmia in Ebstein's Anomaly and Tricuspid Valve Dysplasia Assessed by Fetal Magnetocardiography.
Journal of the American Heart AssociationMid-Term Recovery of Right Ventricular Function and Improvement of Left Ventricular Function After Da Silva Cone Procedure for Ebstein Anomaly.
Journal of cardiovascular development and diseaseA Staged Biventricular Approach Combining the Starnes and Cone Procedures in Ebstein's Anomaly: A Case Report and Literature Review.
Children (Basel, Switzerland)CARTO UNIVU-Guided Micra Implantation in Ebstein Anomaly.
JACC. Case reportsBiventricular remodeling after cone repair in Ebstein anomaly: magnetic resonance imaging data analysis.
Journal of thoracic diseaseLithium-induced Ebstein's Anomaly.
Heart views : the official journal of the Gulf Heart AssociationSurgical trends and outcomes of neonatal Ebstein's anomaly: Multi-institutional study.
The Journal of thoracic and cardiovascular surgeryEbstein 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 surgery[Echocardiographic characteristics and prognostic evaluation of Ebstein anomaly in fetuses].
Zhonghua er ke za zhi = Chinese journal of pediatricsStaged Treatment for Critically Ill Neonates With Ebstein Anomaly: Cone Repair After Starnes Procedure.
Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annualExercise Performance and Right Ventricular Remodeling After the Cone Repair in Patients With Different Severity of Ebstein Anomaly.
The Annals of thoracic 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 surgeryPractical approach to using cardiac magnetic resonance imaging for pre-surgical planning in complex paediatric congenital heart disease.
Pediatric radiologyA dual-chamber leadless pacemaker implantation across percutaneous tricuspid valve-in-valve prosthesis in a patient with Ebstein anomaly.
HeartRhythm case reportsSUCCESSFUL ABLATION OF CONCEALED ACCESSORY PATHWAY MEDIATED SUPRAVENTRICULAR TACHYCARDIA IN A 5-YEAR-OLD CHILD WITH EBSTEIN ANOMALY.
Acta clinica CroaticaLeft 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, IncPresentation of Asymptomatic Ebstein's Anomaly: A Case Report.
CureusEbstein's anomaly in children and young adults: clinical features, arrhythmia, surgical management, and factors affecting arrhythmia and mortality.
Cardiology in the youngIntraoperative Hemodynamic Instability in a Patient With Ebstein's Anomaly Complicated With Eisenmenger Syndrome.
Case reports in cardiologyCircular Shunt: A Loop Not to Be Ignored.
Journal of cardiothoracic and vascular anesthesiaComplete surgical correction of tetralogy of Fallot with Ebstein's anomaly.
Annals of pediatric cardiologyTricuspid valve septal displacement cutoff value for mortality risk following biventricular repair in Ebstein anomaly.
Annals of pediatric cardiologySurgical strategies in Ebstein anomaly: 28 years' experience in a pediatric hospital.
Archivos de cardiologia de MexicoElectrophysiologic Considerations in Adult Patients with Ebstein's Anomaly.
Journal of personalized medicineCase report of Ebstein's anomaly in a young female.
SAGE open medical case reportsPrimary cardiac myxofibrosarcoma in a patient with Ebstein's anomaly: First reported case.
International journal of surgery case reportsCombined Norwood and Modified Left-Sided Starnes Procedures for Congenitally Corrected Transposition of the Great Arteries.
World journal for pediatric & congenital heart surgeryEbstein's Anomaly in a Young Nigerian Lady.
West African journal of medicineSingle-chamber atrial pacing + Micra AV implantation: A case report.
MedicineDiagnosis of Congenital Heart Disease in Adulthood: How Often, How Relevant?
The American journal of cardiologyA case report of Ebstein's anomaly-Gerbode defect dyad: is there room for another anomaly in the same patient?
European heart journal. Case reportsEbstein Anomaly Successfully Treated With Levosimendan Postoperatively in a 60-Year-Old Female: A Case Report.
CureusPreliminary Study of a Degenerated Tricuspid Bioprosthetic Valve Implanted via Transcatheter Valve-in-Valve Implantation Guided by 3-Dimensional Printing.
CJC openFrom ECG to Imaging: Challenges in the Diagnosis of Adult Congenital Heart Diseases.
Journal of clinical medicineThe cone repair allows right ventricle rehabilitation with excellent tricuspid valve function following the Starnes procedure.
The Journal of thoracic and cardiovascular surgeryLarge Right Atrial Size on Cardiac MRI is Associated with Post-operative Right Ventricular Dysfunction After the Cone Operation for Ebstein Anomaly.
Pediatric cardiologyAssessing Left Ventricular Pathology in Patients with Ebstein Anomaly Using Cardiovascular Magnetic Resonance: Looking Past the Right Heart.
Pediatric cardiologyMolecular Pathways and Animal Models of Ebstein's Anomaly.
Advances in experimental medicine and biologyHuman Genetics of Ebstein Anomaly.
Advances in experimental medicine and biologyClinical Presentation and Therapy of Ebstein Anomaly.
Advances in experimental medicine and biologyBiventricular function after Ebstein anomaly repair from a single-center echocardiography study.
Hellenic journal of cardiology : HJC = Hellenike kardiologike epitheoreseUncommon manifestation of Ebstein anomaly: A case report of apical displacement involving all tricuspid valve leaflets.
Clinical case reportsManagement of Ebstein Anomaly in the Current Era: The Story of One Fetus and the Collaboration of Many-A Case Report.
Journal of cardiovascular development and diseaseThe Connection Between Anatomical Substrate and Clinical Severity in Fetal Ebstein Anomaly.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyHeart Transplantation for Ebstein Anomaly due to Late Surgical Presentation.
World journal for pediatric & congenital heart surgery[Comment on the firsts insights of the arterial hypertension study in México (RIHTA): are we doing things right?].
Archivos de cardiologia de MexicoEbstein's Anomaly of the Tricuspid Valve.
Rhode Island medical journal (2013)The American Association for Thoracic Surgery (AATS) 2024 expert consensus document: Management of neonates and infants with Ebstein anomaly.
The Journal of thoracic and cardiovascular surgeryPerinatal outcomes following early prenatal diagnosis: insights from a single-center experience with Ebstein anomaly and tricuspid valve dysplasia.
Archives of gynecology and obstetricsStudy on the correlation between retrograde ductus arteriosus flow and right ventricular function evaluated by Z-score of tricuspid annular plane systolic excursion in fetuses with Ebstein anomaly.
Journal of clinical ultrasound : JCUParadoxical septic embolism in an Ebstein's anomaly patient leading to brain abscess: A case report.
Journal of cardiology casesEbstein's Anomaly of the Tricuspid Valve, Mitral Valve Prolapse and Left Ventricular Non-Compaction: A Triple Trouble.
Heart, lung & circulationEbstein's anomaly: an electrophysiological perspective.
Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacingSurgical Management of Ebstein Anomaly: The Australia and New Zealand Experience.
World journal for pediatric & congenital heart surgeryDiscussion to: Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infants.
JTCVS openOutcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infants.
JTCVS openLong-Term Outcomes of Modified Cone Reconstruction for Ebstein's Anomaly in Pediatric Patients in a Single Center.
Korean circulation journalAtrial Fibrillation Ablation in Congenital Heart Disease: Therapeutic Challenges and Future Perspectives.
Journal of the American Heart AssociationPrenatal sonographic findings of prominent fetal tricuspid annulus: A case report.
Radiology case reportsDistal His bundle pacing in a patient with surgically corrected complex Ebstein anomaly and symptomatic second-degree atrioventricular block: a case report.
European heart journal. Case reportsUsing DaSilva Cone Operation to Establish 1.5 or 2 Ventricle Circulation After Initial Single Ventricle Palliation with Starnes Procedure.
Pediatric cardiologyStrategy and technique for surgical treatment of Ebstein's anomaly.
Chinese medical journalVentricular-Ventricular Interactions in Critically Ill Infants with Ebstein Anomaly or Dysplasia of the Tricuspid Valve: Time to Remember the Forgotten Left Ventricle.
Journal of the American Society of Echocardiography : official publication of the American Society of EchocardiographyAn incidental finding of a right atrial myxoma with undiagnosed Ebstein anomaly: a case report.
European heart journal. Case reportsCardiac Catheterization in Adults with Ebstein Anomaly: Pathophysiologic Insights Regarding Surgical Repair and Prognosis.
Pediatric cardiologyImpact of Neonatal Intervention on Left Ventricular Performance in Ebstein's Anomaly and Tricuspid Valve Dysplasia.
Journal of the American Society of Echocardiography : official publication of the American Society of EchocardiographyRare loss-of-function variants in matrisome genes are enriched in Ebstein's anomaly.
HGG advancesEbstein anomaly with severe tricuspid valve regurgitation: An unusual case of an 84-year natural course.
Kardiologia polskaLong-term Outcome of Simultaneous Transcatheter Pulmonary and Tricuspid Valve-in-Valve Implantation in Ebstein Malformation With Pulmonary Insufficiency.
CJC pediatric and congenital heart diseaseCommentary: Ebstein anomaly with circular shunt-round and round we go but not necessarily very fast.
The Journal of thoracic and cardiovascular surgeryIntraoperative Transesophageal Echocardiography Evaluation of Tricuspid Valve Re-repair for Ebstein's Anomaly.
CureusSignificantly elevated hepatic extracellular volume in adult patients with a Fontan circulation and its correlation with impaired functional capacity.
The international journal of cardiovascular imagingTransplacental non-steroidal anti-inflammatory drugs versus expectant management in fetal Ebstein anomaly with circular shunt: Systematic review and meta-analysis.
Prenatal diagnosisThe Academic Impact of Congenital Heart Surgeons' Society (CHSS) Studies.
World journal for pediatric & congenital heart surgeryTranscatheter thrombectomy of acute pulmonary embolism in an adult Fontan patient: a case report.
European heart journal. Case reportsHeterozygous nonsense variants in laminin subunit 3α resulting in Ebstein's anomaly.
HGG advancesThe concept of cone creation to treat isolated tricuspid valve dysplasia and the case of a double-orifice tricuspid valve.
JTCVS techniquesCone repair after tricuspid valve replacement in Ebstein anomaly.
JTCVS techniquesPregnancy outcomes in women with Ebstein's anomaly: data from the Registry of Pregnancy And Cardiac disease (ROPAC).
Open heartEbstein Anomaly: More Than Just a Tricuspid Valve Issue.
Journal of the American College of CardiologyEchocardiographic Parameters for Risk Prediction in Borderline Right Ventricle: Review with Special Emphasis on Pulmonary Atresia with Intact Ventricular Septum and Critical Pulmonary Stenosis.
Journal of clinical medicineTricuspid cleft or tetracuspid valve? Usefulness of three-dimensional echocardiogram in the assessment of isolated tricuspid regurgitation in pediatrics.
Echocardiography (Mount Kisco, N.Y.)Comparative outcomes and risk analysis after cone repair or tricuspid valve replacement for Ebstein's anomaly.
JTCVS openOutcomes after implantation of right-sided mechanical valve prostheses in congenital heart disease.
Heart (British Cardiac Society)A multidisciplinary approach to re-intervention in an Ebstein patient: a case report.
European heart journal. Case reportsPermanent pacemaker implantation in post one-and-a-half ventricle repair with tricuspid valve replacement in Ebstein anomaly: An innovative technique.
Pacing and clinical electrophysiology : PACEPeripartum anesthetic management in patients with Ebstein anomaly: a case series.
Proceedings (Baylor University. Medical Center)Long-term clinical outcomes of tricuspid valve replacement using bovine versus porcine valves: a nationwide population-based study.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgerySevere form of Ebstein's anomaly associated with ventricular septal defect.
General thoracic and cardiovascular surgery casesPercutaneous Mechanical Circulatory Support as a Bridge to Transplantation in Ebstein Anomaly.
Mayo Clinic proceedingsSurgical experience in adults with Ebstein's anomaly: Long-term results.
Cardiovascular journal of AfricaNeurological, Extracardiac, and Cardiac Manifestations of Ebstein's Anomaly Along With its Genetics, Diagnostic Techniques, Treatment Updates, and the Future Ahead.
CureusEarly spontaneous closure of large arterial ducts in two term neonates with Ebstein anomaly after failed attempts of transcatheter closure.
Cardiology in the youngEarly and long-term outcomes of bioprosthetic versus mechanical tricuspid valve replacement: A nationwide population-based study.
The Journal of thoracic and cardiovascular surgeryTreating Supraventricular Tachycardia With Amiodarone in a Patient With Ebstein's Anomaly.
CureusContemporary Early Postoperative Cone Repair Outcomes for Patients With Ebstein Anomaly.
Mayo Clinic proceedingsPercutaneous Closure of Paravalvular Leak After Tricuspid Valve Replacement for Ebstein's Anomaly.
Texas Heart Institute journal[Atypical chest pain revealing an asymptomatic Ebstein´s anomaly: case report].
The Pan African medical journalEbstein's anomaly in a French bulldog.
Veterinary research forum : an international quarterly journalTailored strategy to match anatomy and physiology with intervention can improve outcomes of symptomatic neonates with Ebstein anomaly.
JTCVS openEbstein's anomaly with pericardial effusion in a 55-year-old lady: A case report.
Annals of medicine and surgery (2012)Does the right ventricle size influence the left ventricle size and function in children with Ebstein anomaly?
Echocardiography (Mount Kisco, N.Y.)Sequential percutaneous tricuspid and pulmonary valve implantation in a young child operated previously for Ebstein Anomaly.
Cardiology in the youngPrognostic implications of atrial fibrillation in adults with Ebstein anomaly.
Heart (British Cardiac Society)Cardiac remodeling after tricuspid valve repair in Ebstein's anomaly: a magnetic resonance study.
European radiologyLate-gestation prediction of outcome in tricuspid valve dysplasia and Ebstein's anomaly using fetal tricuspid regurgitation waveform analysis.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyEbstein's Anomaly: How to Identify Intracardiac Structures When Electrical Signals Do Not Match the Anatomy.
The Journal of innovations in cardiac rhythm managementMultimodality Imaging in Ebstein Anomaly.
Pediatric cardiologyThe Use of Feature Tracking Technique for the Quantification of Ventricular Strain Pattern in Patients with Ebstein's Anomaly: A Case-Control Study.
Iranian journal of medical sciencesDiagnosis and treatment of congenital tricuspid valve malformation in a case of monozygotic twins.
Journal of cardiothoracic surgery[Ebstein's anomaly with ventricular preexcitation in an adult patient].
Revista medica del Instituto Mexicano del Seguro SocialDetection of early signs of right ventricular systolic impairment in unoperated Ebstein's anomaly by cardiac magnetic resonance feature tracking.
Cardiovascular diagnosis and therapyCardiac Implantable Electronic Devices in Ebstein Anomaly: Management and Outcomes.
Circulation. Arrhythmia and electrophysiologyStarr-Edwards tricuspid valve in a patient with Ebstein anomaly: older than half of the century and still functioning well.
European heart journalPersistent left superior vena cava in a 29-year-old lady with Ebstein's anomaly and complete heart block. A case report and literature review.
Annals of medicine and surgery (2012)Treatment of Severe Fetal Ebstein's Anomaly with Prenatal Nonsteroidal Anti-Inflammatory Therapy.
Fetal diagnosis and therapyComprehensive Evaluation of Pediatric Patients with Ebstein Anomaly Requires Both Echocardiography and Cardiac Magnetic Resonance Imaging.
Pediatric cardiologyReport of persistent left superior vena cava associated with Ebstein's anomaly of tricuspid valve.
ARYA atherosclerosisTranscatheter Systemic AV Valve-in-Valve Implantation in a Patient With LTGA and Ebstein Anomaly.
JACC. Case reportsMoyamoya Disease Complicating Ebstein's Anomaly.
Internal medicine (Tokyo, Japan)Implantation of a leadless pacemaker in a patient with mechanical tricuspid valve.
HeartRhythm case reportsEbstein's Anomaly: From Fetus to Adult-Literature Review and Pathway for Patient Care.
Pediatric cardiologyA Systematic Review of Ebstein's Anomaly with Left Ventricular Noncompaction.
Journal of cardiovascular development and diseaseSuccessful Radiofrequency Ablation of the Right Lateral Accessory Pathway with Modified Carpentier Reconstruction Surgery in a Patient with Ebstein Anomaly Concomitant of Secundum Atrial Septal Defect, Atrial Fibrillation, and Wolff-Parkinson-White Syndrome.
Case reports in vascular medicineSurgical treatment of tricuspid valve dysplasia in children.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryNew trend: Surgical correction of Ebstein's anomaly using modified cone repair and a combination of different techniques.
Journal of cardiac surgeryDisplacement of all three leaflets of tricuspid valve: A rare variant of Ebstein anomaly.
Clinical case reports[Successful Surgical Repair for Adult Ebstein Disease Using Cone Reconstruction Combined with One and a Half Ventricle Repair].
Kyobu geka. The Japanese journal of thoracic surgeryThe Mid-Term Outcomes of Cone Repair or Replacement of Tricuspid Valve in Patients with Ebstein's Anomaly: Our Experience.
The heart surgery forumCardiac MRI predictors of right ventricular dysfunction after the Da Silva cone operation for Ebstein's anomaly.
International journal of cardiology. Congenital heart diseaseLeft Ventricular Dysfunction in Neonatal Ebstein's Anomaly and Tricuspid Valve Dysplasia.
Journal of the American Society of Echocardiography : official publication of the American Society of EchocardiographyNarrative review of Ebstein's anomaly beyond childhood: Imaging, surgery, and future perspectives.
Cardiovascular diagnosis and therapyDual atrial rhythms: a case report of an unusual cause of pacemaker syndrome.
European heart journal. Case reportsImpact of fetal haemodynamics on surgical and neurodevelopmental outcomes in patients with Ebstein anomaly and tricuspid valve dysplasia.
Cardiology in the youngAcute Right Ventricular Reshaping After TriClip in Ebstein's-Like Anomaly Assessed by Multimodality Imaging.
The Journal of invasive cardiologyA case of redo tricuspid valve repair after neonatal cone operation for Ebstein's anomaly.
General thoracic and cardiovascular surgeryEbstein Anomaly and Right Aortic Arch in Patient with Charge Syndrome.
Medicina (Kaunas, Lithuania)Transcatheter tricuspid valve-in-valve implantation for degenerative surgical bio-prosthesis using SAPIEN 3: A case series.
Clinical case reportsModified Starnes procedure as a bridge to 2-ventricle repair in neonatal Ebstein's anomaly: A paradigm shift.
Multimedia manual of cardiothoracic surgery : MMCTSSuccessful One-and-a-Half Ventricle Repair of Right Ventricle Dysfunction Due to Lymphoblastic Leukemia Treatment in a Patient with Restrictive Cardiomyopathy.
The American journal of case reportsEbstein's Anomaly, Left Ventricular Noncompaction and Gerbode-Like Defect Triad (Fetal Diagnosis and Neonatal Course).
Case reports in pediatricsImaging for Transcatheter Edge-to-Edge Repair for Tricuspid Regurgitation in Ebstein Anomaly.
Circulation. Cardiovascular imagingThe Association of Pulmonary Flow Characteristics With Cardiac Function in Tricuspid Valve Malformation Fetuses: Study With Two-Dimensional Speckle Tracking Echocardiography.
Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in MedicineImproved Tricuspid Valve Function, Preload Recruitment and Ventricular Efficiency During Submaximal Exercise in Patients with Unoperated Ebstein's Anomaly: An MRI Study.
Journal of magnetic resonance imaging : JMRICase Report of a 33-year-old with Ebstein malformation of tricuspid valve and hypertrophic cardiomyopathy.
Cardiology in the youngCone Reconstruction for Ebstein Anomaly: Ventricular Remodeling and Preliminary Impact of Stem Cell Therapy.
Mayo Clinic proceedingsEbstein's Anomaly: Review of Arrhythmia Types and Morphogenesis of the Anomaly.
JACC. Clinical electrophysiologyInadvertent irreversible closure of arterial duct following therapeutic use of transplacental indomethacin in a fetus with severe Ebstein's anomaly and circular shunt.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyImpact of Cone vs. Hetzer Repair on Postoperative Outcomes in Patients With Ebstein's Anomaly: 10-Year Experience From a Single Institution.
Frontiers in cardiovascular medicineClinical use of percutaneous mechanical circulatory assistance in a patient with end-stage right-sided heart failure and massive tricuspid insufficiency due to congenital heart disease: first-in-the-world case report.
European heart journal. Case reportsTwo decades of experience on ablation in children with Ebstein's anomaly.
Cardiology in the youngA simplified repair method for adult Ebstein's anomaly.
Journal of cardiac surgerySurgical treatment of Ebstein anomaly in pediatric patients: A 10-year single-center study.
Journal of cardiac surgeryAccessing the inaccessible: Stereotactic radioablation of premature ventricular complexes originating in the right ventricle in a patient with a mechanical tricuspid valve.
HeartRhythm case reportsThe Association of Age and Repair Modification with Outcome after Cone Repair for Ebstein's Malformation.
Seminars in thoracic and cardiovascular surgeryCOVID-19-Positive Patient and Hypoxemia: Are Lungs Always to Be Blamed?
JACC. Cardiovascular interventionsLeft Ventricular Pathology in Ebstein's Anomaly-Myocardium in Motion: CMR Insights Into Left Ventricular Fibrosis, Deformation, and Exercise Capacity.
Circulation. Cardiovascular imaging"Leafletization" of Atrialized Ventricular Septum Endocardium in Ebstein Anomaly.
The Annals of thoracic surgeryEbstein's anomaly in the neonate.
Indian journal of thoracic and cardiovascular surgeryHemodynamics and Clinical Implications of Occult Left Ventricular Dysfunction in Adults Undergoing Ebstein Anomaly Repair.
Circulation. Cardiovascular imagingFrom Safety to Benefit in Cell Delivery During Surgical Repair of Ebstein Anomaly: Initial Results.
The Annals of thoracic surgerySuccessful Radiofrequency Ablation of an Atrial Flutter in an Elderly Patient With Uncorrected Ebstein's Anomaly.
CureusCommentary: Ventricular function improvement after the cone for Ebstein anomaly: It is time to incorporate magnetic resonance studies into every long-term postoperative protocol.
The Journal of thoracic and cardiovascular surgeryCone repair for Ebstein's anomaly and atrial fibrillation ablation in an adult patient.
Multimedia manual of cardiothoracic surgery : MMCTSNeonatal Cone Valvuloplasty for Pulmonary Atresia/Intact Ventricular Septum and Ebsteinoid Valve.
The Annals of thoracic surgeryElectocardiographic features in Ebstein's disease.
La Tunisie medicalePatent foramen ovale and atrial septal defect.
Echocardiography (Mount Kisco, N.Y.)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.
- Septal Leaflet Augmentation During the Da Silva Cone Repair: Valvar Function and Anatomical Features Compared to Non-Patch Repair.
- Biventricular Repair in Symptomatic Neonates with Ebstein Anomaly: 25-30 years of follow-up.
- Risk factors for morbidity and mortality in Ebstein's anomaly: a registry-based study of 398 patients.Clinical research in cardiology : official journal of the German Cardiac Society· 2026· PMID 41779126mais citado
- Changing Course-Starnes Takedown in High-Risk Children and Review of the Current Literature.
- Use of right atrial wall to repair severely dysplastic tricuspid valve in an infant with Ebstein's anomaly.
- Left ventricular outflow tract obstruction after Ebstein tricuspid valve surgery: a case report.
- Circular Shunt: A Loop Not to Be Ignored.
- Case report of Ebstein's anomaly in a young female.
- Heterozygous nonsense variants in laminin subunit 3α resulting in Ebstein's anomaly.
- Impact of Cone vs. Hetzer Repair on Postoperative Outcomes in Patients With Ebstein's Anomaly: 10-Year Experience From a Single Institution.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:1880(Orphanet)
- OMIM OMIM:224700(OMIM)
- MONDO:0009144(MONDO)
- GARD:6313(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q277452(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
