Overview
Ebstein's anomaly is a congenital heart defect characterized by an abnormally positioned and malformed tricuspid valve, often leading to right ventricular dysfunction and varying degrees of cyanosis and heart failure 124.Diagnosis
Management
Special Populations
Key Recommendations
References
Showing 100 most recent of 176 indexed papers.
1 Pasqualin G, Sturla F, Cavazza B, Tondi L, Abbate M, Bevilacqua F et al.. Anatomical severity of Ebstein's anomaly: a quantitative analysis based on cardiovascular MRI. Open heart 2026. link 2 Bravo-Valenzuela NJ, Rizzo G, Mappa I, Respondek-Liberska M, Słodki M, Amorim LAR et al.. Prenatal Cardiac Ultrasound Markers of Outcomes in Ebstein's Anomaly: An International Multicenter Case Series. Pediatric cardiology 2025. link 3 Teramachi Y, Hornberger LK, Howley L, van der Velde ME, Al-Aklabi M, Eckersley LG. Impact 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 Echocardiography 2024. link 4 Yu S, Yang K, Chen X, Lu M, Zhao K, Yang S et al.. Cardiac remodeling after tricuspid valve repair in Ebstein's anomaly: a magnetic resonance study. European radiology 2023. link 5 Suriya JY, Raj A, Pillai AA, Satheesh S, Plakkal N, Kundra P et al.. Ebstein's anomaly during pregnancy: experience from a tertiary care centre - a case series and review of literature. Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology 2022. link 6 Schulz A, Marathe SP, Chávez M, Sleeper LA, Emani SM, Marx GR et al.. The Association of Age and Repair Modification with Outcome after Cone Repair for Ebstein's Malformation. Seminars in thoracic and cardiovascular surgery 2022. link 7 Rydzewska K, Sylwestrzak O, Krekora M, Słodki M, Respondek-Liberska M. Ebstein's anomaly: epidemiological analysis and presentation of different prenatal management. The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians 2022. link 8 Nabeshima J, Yamagishi M, Maeda Y, Hongu H, Nakatsuji H, Nagase T et al.. A case of redo tricuspid valve repair after neonatal cone operation for Ebstein's anomaly. General thoracic and cardiovascular surgery 2022. link 9 Ferrari I, Shehu N, Nagdyman N, Martinoff S, Ewert P, Stern H et al.. Improved 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 : JMRI 2022. link 10 Karagöz T, Ertuğrul İ, Aypar E, Adıgüzel A, Aykan HH, Şahin M et al.. Two decades of experience on ablation in children with Ebstein's anomaly. Cardiology in the young 2022. link 11 Prota C, Di Salvo G, Sabatino J, Josen M, Paredes J, Sirico D et al.. Prognostic value of echocardiographic parameters in pediatric patients with Ebstein's anomaly. International journal of cardiology 2019. link 12 Hasegawa M, Iwai S, Yamauchi S, Kugo Y, Kayatani F, Takahashi K et al.. Bilateral Pulmonary Artery Banding in Ebstein's Anomaly With Circular Shunting. The Annals of thoracic surgery 2019. link 13 Mrad Agua K, Burri M, Cleuziou J, Beran E, Meierhofer C, Nagdyman N et al.. Preoperative predictability of right ventricular failure following surgery for Ebstein's anomaly. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2019. link 14 Geerdink LM, Delhaas T, Helbing WA, du Marchie Sarvaas GJ, Heide HT, Rozendaal L et al.. Paediatric Ebstein's anomaly: how clinical presentation predicts mortality. Archives of disease in childhood 2018. link 15 Steinmetz M, Broder M, Hösch O, Lamata P, Kutty S, Kowallick JT et al.. Atrio-ventricular deformation and heart failure in Ebstein's Anomaly - A cardiovascular magnetic resonance study. International journal of cardiology 2018. link 16 Ciepłucha A, Trojnarska O, Kociemba A, Łanocha M, Barczynski M, Rozmiarek S et al.. Clinical aspects of myocardial fibrosis in adults with Ebstein's anomaly. Heart and vessels 2018. link 17 Boyle B, Garne E, Loane M, Addor MC, Arriola L, Cavero-Carbonell C et al.. The changing epidemiology of Ebstein's anomaly and its relationship with maternal mental health conditions: a European registry-based study. Cardiology in the young 2017. link 18 Steinmetz M, Usenbenz S, Kowallick JT, Hösch O, Staab W, Lange T et al.. Left ventricular synchrony, torsion, and recoil mechanics in Ebstein's anomaly: insights from cardiovascular magnetic resonance. Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance 2017. link 19 Kühn A, Meierhofer C, Rutz T, Rondak IC, Röhlig C, Schreiber C et al.. Non-volumetric echocardiographic indices and qualitative assessment of right ventricular systolic function in Ebstein's anomaly: comparison with CMR-derived ejection fraction in 49 patients. European heart journal. Cardiovascular Imaging 2016. link 20 Gangurde PB, Tidake A, Shah H, Mahajan A, Lokhandwala Y, Nathani P. Absent right bundle branch block: Is it a clue of pre-excitation in Ebstein's anomaly?. Indian heart journal 2016. link 21 Houser L, Zaragoza-Macias E, Jones TK, Aboulhosn J. Transcatheter closure of atrial septal communication during pregnancy in women with Ebstein's anomaly of the tricuspid valve and cyanosis. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions 2015. link 22 Knott-Craig CJ, Kumar TK, Arevalo AR, Joshi VM. Surgical management of symptomatic neonates with Ebstein's anomaly: choice of operation. Cardiology in the young 2015. link 23 Lange R, Burri M, Eschenbach LK, Badiu CC, da Silva JP, Nagdyman N et al.. Da Silva's cone repair for Ebstein's anomaly: effect on right ventricular size and function. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2015. link 24 Geerdink LM, Kapusta L. Dealing with Ebstein's anomaly. Cardiology in the young 2014. link 25 Goleski PJ, Sheehan FH, Chen SS, Kilner PJ, Gatzoulis MA. The shape and function of the left ventricle in Ebstein's anomaly. International journal of cardiology 2014. link 26 Sano S, Fujii Y, Kasahara S, Kuroko Y, Tateishi A, Yoshizumi K et al.. Repair of Ebstein's anomaly in neonates and small infants: impact of right ventricular exclusion and its indications. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2014. link 27 Morikawa M, Endo D, Yamada T, Cho K, Yamada T, Minakami H. Electronic fetal heart rate monitoring in five fetuses with Ebstein's anomaly. The journal of obstetrics and gynaecology research 2014. link 28 Jiang GP, Gao LC, Gong FQ, He J, Ye JJ. A new method for evaluating tricuspid valve displacement in children with Ebstein's anomaly: using the annulus and coronary sinus as a reference point. Pediatric cardiology 2014. link 29 Fratz S, Janello C, Müller D, Seligmann M, Meierhofer C, Schuster T et al.. The functional right ventricle and tricuspid regurgitation in Ebstein's anomaly. International journal of cardiology 2013. link 30 Oxenius A, Attenhofer Jost CH, Prêtre R, Dave H, Bauersfeld U, Kretschmar O et al.. Management and outcome of Ebstein's anomaly in children. Cardiology in the young 2013. link 31 Kühn A, De Pasquale Meyer G, Müller J, Petzuch K, Fratz S, Röhlig C et al.. Tricuspid valve surgery improves cardiac output and exercise performance in patients with Ebstein's anomaly. International journal of cardiology 2013. link 32 Sasikumar N, Krishna Manohar SR, Philip S, Cherian KM, Suresh Kumar R. Right ventricular exclusion and univentricular palliation for failed one and a half ventricle repair for Ebstein's anomaly. Heart, lung & circulation 2013. link 33 Dearani JA, Said SM, O'Leary PW, Burkhart HM, Barnes RD, Cetta F. Anatomic repair of Ebstein's malformation: lessons learned with cone reconstruction. The Annals of thoracic surgery 2013. link 34 Lee CM, Sheehan FH, Bouzas B, Chen SS, Gatzoulis MA, Kilner PJ. The shape and function of the right ventricle in Ebstein's anomaly. International journal of cardiology 2013. link 35 Robicsek F. Wilhelm Ebstein and the history of surgery for Ebstein's Disease. The Thoracic and cardiovascular surgeon 2013. link 36 Radojevic J, Inuzuka R, Alonso-Gonzalez R, Borgia F, Giannakoulas G, Prapa M et al.. Peak oxygen uptake correlates with disease severity and predicts outcome in adult patients with Ebstein's anomaly of the tricuspid valve. International journal of cardiology 2013. link 37 Vogel M, Marx GR, Tworetzky W, Cecchin F, Graham D, Mayer JE et al.. Ebstein's malformation of the tricuspid valve: short-term outcomes of the "cone procedure" versus conventional surgery. Congenital heart disease 2012. link 38 Attenhofer Jost CH, Edmister WD, Julsrud PR, Dearani JA, Savas Tepe M, Warnes CA et al.. Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein's anomaly. The international journal of cardiovascular imaging 2012. link 39 D'Andrea A, Scognamiglio G, Giordano F, Cuomo S, Russo MG, Rinaldi G et al.. An atypical assessment of Ebstein's anomaly in an 86-year-old man. Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace 2011. link 40 Badiu CC, Schreiber C, Hörer J, Ruzicka DJ, Wottke M, Cleuziou J et al.. Early timing of surgical intervention in patients with Ebstein's anomaly predicts superior long-term outcome. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2010. link 41 Song ZZ. A combination of right ventricular hypertrabeculation/noncompaction and Ebstein's anomaly. International journal of cardiology 2010. link 42 Williams AN, Wilson N, Sunderland R. Philosopher, pediatrician, pathologist? John Locke's thoughts on Rhicketts and a missed case of Ebstein's anomaly. Pediatric cardiology 2009. link 43 Malhotra SP, Petrossian E, Reddy VM, Qiu M, Maeda K, Suleman S et al.. Selective right ventricular unloading and novel technical concepts in Ebstein's anomaly. The Annals of thoracic surgery 2009. link 44 Chen Y, Lv G, Li B, Wang Z. Cerebral vascular resistance and left ventricular myocardial performance in fetuses with Ebstein's anomaly. American journal of perinatology 2009. link 45 Santoro G, Palladino MT, Russo MG, Calabrò R. Neonatal patent ductus arteriosus recanalization and stenting in critical Ebstein's anomaly. Pediatric cardiology 2008. link 46 Aggarwal S, Chintala K, Humes RA. Sildenafil use in a symptomatic neonate with severe Ebstein's anomaly of the tricuspid valve. American journal of perinatology 2008. link 47 Barbara DW, Edwards WD, Connolly HM, Dearani JA. Surgical pathology of 104 tricuspid valves (2000-2005) with classic right-sided Ebstein's malformation. Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology 2008. link 48 Dencker M, Stagmo M, Wollmer P. Severe dyspnoea on light exercise at high altitude: unusual presentation of Ebstein's anomaly. International journal of cardiology 2008. link 49 Joffe D, Gurvitz M, Stout K. Considerations of a one-and-one-half ventricle repair in a 47-year-old patient. Congenital heart disease 2008. link 50 Vettukattil JJ, Bharucha T, Anderson RH. Defining Ebstein's malformation using three-dimensional echocardiography. Interactive cardiovascular and thoracic surgery 2007. link 51 Muñoz-Castellanos L, Espinola-Zavaleta N, Kuri-Nivón M, Keirns C. Ebstein's Anomaly: anatomo-echocardiographic correlation. Cardiovascular ultrasound 2007. link 52 Arslan S, Gurlertop HY, Gundogdu F, Senocak H. Left ventricular noncompaction and mid-caviter narrowing associated with Ebstein's anomaly: three-dimensional transthoracic echocardiographic image. International journal of cardiology 2007. link 53 da Silva JP, Baumgratz JF, da Fonseca L, Franchi SM, Lopes LM, Tavares GM et al.. The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results. The Journal of thoracic and cardiovascular surgery 2007. link 54 Trojnarska O, Szyszka A, Gwizdała A, Siniawski A, Oko-Sarnowska Z, Chmara E et al.. Adults with Ebstein's anomaly--Cardiopulmonary exercise testing and BNP levels exercise capacity and BNP in adults with Ebstein's anomaly. International journal of cardiology 2006. link 55 Iturralde P, Nava S, Sálica G, Medeiros A, Márquez MF, Colin L et al.. Electrocardiographic characteristics of patients with Ebstein's anomaly before and after ablation of an accessory atrioventricular pathway. Journal of cardiovascular electrophysiology 2006. link 56 Guerra VC, Coles J, Smallhorn JF. Aneurysm of right atrium diagnosed by 3-dimensional real-time echocardiogram. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography 2005. link 57 Malec E, Dangel J, Mroczek T, Procelewska M, Januszewska K, Ko Cz J. Successful surgical treatment of a neonate with prenatal diagnosis of severe Ebstein's anomaly. Pediatric cardiology 2005. link 58 Bilge AK, Adalet K, Ozyiğit T, Ozben B, Yilmaz E. Tricuspid endocarditis in an adult patient with Ebstein's anomaly who has a residual pacemaker lead. The international journal of cardiovascular imaging 2005. link 59 Patel V, Nanda NC, Rajdev S, Mehmood F, Velayudhan D, Vengala S et al.. Live/real time three-dimensional transthoracic echocardiographic assessment of Ebstein's anomaly. Echocardiography (Mount Kisco, N.Y.) 2005. link 60 Akaishi J, Yamauchi H, Ochi M, Ogawa S, Asou T, Tanaka S. One and a half ventricle repair for Ebstein's anomaly. The Japanese journal of thoracic and cardiovascular surgery : official publication of the Japanese Association for Thoracic Surgery = Nihon Kyobu Geka Gakkai zasshi 2003. link 61 Pavoni D, Ragazzo S, Driussi M, Antonini-Canterin F, Pavan D, Zardo F et al.. Transient platypnea-orthodeoxia-like syndrome induced by propafenone overdose in a young woman with Ebstein's anomaly. Italian heart journal : official journal of the Italian Federation of Cardiology 2003. link 62 Watanabe M, Harada Y, Takeuchi T, Satomi G, Yasukouchi S. Modified Starnes operation for neonatal Ebstein's anomaly. The Annals of thoracic surgery 2002. link03800-6) 63 Lazorishinets VV, Glagola MD, Stychinsky AS, Rudenko MN, Knyshov GV. Surgical treatment of Wolf-Parkinson-White syndrome during plastic operations in patients with Ebstein's anomaly. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2000. link00466-8) 64 Huang CJ, Chiu IS, Lin FY, Chen WJ, Lin JL, Lo HM et al.. Role of electrophysiological studies and arrhythmia intervention in repairing Ebstein's anomaly. The Thoracic and cardiovascular surgeon 2000. link 65 Bockeria LA, Kagramanov II, Kokshenev IV, Chiaureli MR, Gadzhiev AA, Mitina IN et al.. Immediate results of the 'Bioglis' valve use in the surgical treatment of Ebstein's anomaly. The Journal of heart valve disease 2000. link 66 Hebe J. Ebstein's anomaly in adults. Arrhythmias: diagnosis and therapeutic approach. The Thoracic and cardiovascular surgeon 2000. link 67 Frescura C, Angelini A, Daliento L, Thiene G. Morphological aspects of Ebstein's anomaly in adults. The Thoracic and cardiovascular surgeon 2000. link 68 Chauvaud S. Ebstein's malformation. surgical treatment and results. The Thoracic and cardiovascular surgeon 2000. link 69 John S, Rao C. Prosthetic valve replacement in Ebstein's malformation: 30-year follow-up. The Annals of thoracic surgery 1999. link00500-7) 70 Kupilik N, Simon P, Moidl R, Wollenek G, Marx M, Wolner E et al.. Valve-preserving treatment of Ebstein's anomaly: perioperative and follow-up results. The Thoracic and cardiovascular surgeon 1999. link 71 Ai T, Horie M, Washizuka T, Albrecht C, Nishimoto T, Obayashi K et al.. Successful radiofrequency current catheter ablation of accessory atrioventricular pathway after tricuspid replacement in Ebstein's anomaly. Japanese circulation journal 1998. link 72 Jayaprakash S, Mond HG, Sparks PB, Grigg LE. Transvenous ventricular pacing options in Ebstein's anomaly. Indian heart journal 1998. link 73 Kiziltan HT, Theodoro DA, Warnes CA, O'Leary PW, Anderson BJ, Danielson GK. Late results of bioprosthetic tricuspid valve replacement in Ebstein's anomaly. The Annals of thoracic surgery 1998. link00961-8) 74 Reich JD, Auld D, Hulse E, Sullivan K, Campbell R. The Pediatric Radiofrequency Ablation Registry's experience with Ebstein's anomaly. Pediatric Electrophysiology Society. Journal of cardiovascular electrophysiology 1998. link 75 Marianeschi SM, McElhinney DB, Reddy VM, Silverman NH, Hanley FL. Alternative approach to the repair of Ebstein's malformation: intracardiac repair with ventricular unloading. The Annals of thoracic surgery 1998. link00897-2) 76 van Son JA, Kinzel P, Mohr FW. Pericardial patch augmentation of anterior tricuspid leaflet in Ebstein's anomaly. The Annals of thoracic surgery 1998. link00747-4) 77 Okishige K, Azegami K, Goseki Y, Ohira H, Sasano T, Yamashita K et al.. Radiofrequency ablation of tachyarrhythmias in patients with Ebstein's anomaly. International journal of cardiology 1997. link00089-2) 78 Daliento L, Angelini A, Ho SY, Frescura C, Turrini P, Baratella MC et al.. Angiographic and morphologic features of the left ventricle in Ebstein's malformation. The American journal of cardiology 1997. link00602-4) 79 Yung MC, Chang Y, Yu TJ, Weng ZC, Shih CC, Wang JS et al.. Surgical repair of Ebstein's anomaly. Zhonghua yi xue za zhi = Chinese medical journal; Free China ed 1997. link 80 Ohata T, Harada K, Tamura M, Ito T, Watanabe Y, Takada G. An infant case of Ebstein's anomaly of the tricuspid valve with abnormal diastolic filling pattern of the left ventricle. The Tohoku journal of experimental medicine 1996. link 81 Cappato R, Schlüter M, Weiss C, Antz M, Koschyk DH, Hofmann T et al.. Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein's anomaly. Circulation 1996. link 82 Isobe M, Tanaka M, Sekiguchi M. Subaortic stenosis due to accessory tissue of the mitral valve associated with Ebstein's anomaly in an adult. International journal of cardiology 1996. link02825-2) 83 Senni M, Chauvaud S, Crupi G, Procopio A, Bianchi T. Early and intermediate term results of Carpentier's repair for Ebstein's anomaly. Giornale italiano di cardiologia 1996. link 84 Kaulitz R, Ziemer G. Modified Fontan procedure for Ebstein's anomaly of the tricuspid valve--an alternative surgical approach preserving Ebstein's anatomy. The Thoracic and cardiovascular surgeon 1995. link 85 Eustace S, Kruskal JB, Hartnell GG. Ebstein's anomaly presenting in adulthood: the role of cine magnetic resonance imaging in diagnosis. Clinical radiology 1994. link82661-3) 86 Fang BR, Chiang CW, Lee YS. Echocardiographic study of Ebstein's anomaly. Changgeng yi xue za zhi 1994. link 87 Jaiswal PK, Balakrishnan KG, Saha A, Venkitachalam CG, Tharakan J, Titus T. Clinical profile and natural history of Ebstein's anomaly of tricuspid valve. International journal of cardiology 1994. link90031-0) 88 Correa-Villaseñor A, Ferencz C, Neill CA, Wilson PD, Boughman JA. Ebstein's malformation of the tricuspid valve: genetic and environmental factors. The Baltimore-Washington Infant Study Group. Teratology 1994. link 89 Yamazaki K, Hoshino S, Tagusari O, Fujino S, Takemura T, Koyanagi H. A new technique of tricuspid valve replacement for Ebstein's anomaly. Cardiovascular surgery (London, England) 1993. link 90 Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC. Operative treatment of Ebstein's anomaly. The Journal of thoracic and cardiovascular surgery 1992. link 91 Leung MP, Lee J, Lo RN, Mok CK. Modified Fontan procedure for severe Ebstein's malformation with predominant tricuspid stenosis. The Annals of thoracic surgery 1992. link90447-c) 92 Rusconi PG, Zuberbuhler JR, Anderson RH, Rigby ML. Morphologic-echocardiographic correlates of Ebstein's malformation. European heart journal 1991. link 93 Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein's anomaly appearing in the neonate. A new surgical approach. The Journal of thoracic and cardiovascular surgery 1991. link 94 Jacob JL, da Silveira LC, Braile DM. Echocardiographic and angiographic diagnosis of Ebstein's anomaly of the mitral valve. British heart journal 1991. link 95 Balaji S, Dennis NR, Keeton BR. Familial Ebstein's anomaly: a report of six cases in two generations associated with mild skeletal abnormalities. British heart journal 1991. link 96 Milgalter E, Laks H. Use of a pericardial patch to bridge the conduction tissue during tricuspid valve replacement. The Annals of thoracic surgery 1991. link90028-o) 97 Casta A. Echocardiographic assessment of the right atrioventricular junction in Ebstein's malformation. Puerto Rico health sciences journal 1990. link 98 Cromme-Dijkhuis AH, Hamer JP. Two-dimensional colour-coded echocardiography in Ebstein's anomaly. European heart journal 1990. link 99 Krishnamurthy SN, Paulose KO. Vocal cord paralysis with Ebstein's anomaly. The Journal of laryngology and otology 1989. link 100 Tuzcu EM, Moodie DS, Ghazi F, Sterba R, Gill CC, Stewart R et al.. Ebstein's anomaly: natural and unnatural history. Cleveland Clinic journal of medicine 1989. link