Ebstein's anomaly of the tricuspid valve
- Brojendra N Agarwala, MD
Brojendra N Agarwala, MD
- Professor of Pediatrics
- Pritzker School of Medicine of the University of Chicago
- Ziyad M Hijazi, MD, MPH, FAAP, FACC, MSCAI, FAHA
Ziyad M Hijazi, MD, MPH, FAAP, FACC, MSCAI, FAHA
- Professor of Pediatrics, Weill Cornell Medicine
- Chairman, Department of Pediatrics & Director, Cardiac Program
- Sidra Medical & Research Center
- Doha, Qatar
- Joseph Dearani, MD
Joseph Dearani, MD
- Chair, Division of Cardiovascular Surgery
- Professor of Surgery
- Mayo Clinic
Ebstein's anomaly is a congenital malformation that is characterized primarily by abnormalities of the tricuspid valve and right ventricle (figure 1). The estimated risk of Ebstein’s anomaly in the general population is 1 in 20,000 live births with no predilection for either gender [1-3]. For unknown reasons, it occurs with higher frequency in infants of mothers who take lithium during early pregnancy [4,5]. (See "Teratogenicity, pregnancy complications, and postnatal risks of antipsychotics, benzodiazepines, lithium, and electroconvulsive therapy", section on 'Lithium'.)
Tricuspid valve — The morphology of the tricuspid valve in Ebstein's anomaly, and consequently the clinical presentation, is highly variable. The tricuspid valve leaflets are malformed and partly attached to the fibrous tricuspid valve annulus and partly to the right ventricular endocardium (image 1).
●The anterior leaflet is the largest leaflet and is usually attached to the tricuspid valve annulus.
●The posterior and septal leaflets are vestigial or absent as there is failure of delamination (separation of the layer of valve tissue) from the myocardium. When present, the free edges are generally displaced posteriorly and downward from the atrioventricular junction and progressively toward the right ventricular outflow tract and apex.
The leaflets of the tricuspid valve may have free leading edges with chordae, or be directly attached to papillary muscle(s) or the underlying myocardium. The tricuspid valve is funnel-shaped ("sail-like"), incompetent, and rarely stenotic.
- Lupo PJ, Langlois PH, Mitchell LE. Epidemiology of Ebstein anomaly: prevalence and patterns in Texas, 1999-2005. Am J Med Genet A 2011; 155A:1007.
- Correa-Villaseñor A, Ferencz C, Neill CA, et al. Ebstein's malformation of the tricuspid valve: genetic and environmental factors. The Baltimore-Washington Infant Study Group. Teratology 1994; 50:137.
- Pradat P, Francannet C, Harris JA, Robert E. The epidemiology of cardiovascular defects, part I: a study based on data from three large registries of congenital malformations. Pediatr Cardiol 2003; 24:195.
- Allan LD, Desai G, Tynan MJ. Prenatal echocardiographic screening for Ebstein's anomaly for mothers on lithium therapy. Lancet 1982; 2:875.
- Attenhofer Jost CH, Connolly HM, Dearani JA, et al. Ebstein's anomaly. Circulation 2007; 115:277.
- Dearani JA, Danielson GK. Congenital Heart Surgery Nomenclature and Database Project: Ebstein's anomaly and tricuspid valve disease. Ann Thorac Surg 2000; 69:S106.
- BURCHELL HB, DUSHANE JW, KILBY RA, WOOD EH. Ebstein's malformation: a clinical and laboratory study. Medicine (Baltimore) 1956; 35:161.
- Attenhofer Jost CH, Connolly HM, O'Leary PW, et al. Left heart lesions in patients with Ebstein anomaly. Mayo Clin Proc 2005; 80:361.
- Watson H. Electrode catheters and the diagnosis of Ebstein's anomaly of the tricuspid valve. Br Heart J 1966; 28:161.
- SUMNER RG, JACOBY WJ Jr, TUCKER DH. EBSTEIN'S ANOMALY ASSOCIATED WITH CARDIOMYOPATHY AND PULMONARY HYPERTENSION. Circulation 1964; 30:578.
- Seward JB, Tajik AJ, Feist DJ, Smith HC. Ebstein's anomaly in an 85-year-old man. Mayo Clin Proc 1979; 54:193.
- Celermajer DS, Bull C, Till JA, et al. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 1994; 23:170.
- Watson H. Natural history of Ebstein's anomaly of tricuspid valve in childhood and adolescence. An international co-operative study of 505 cases. Br Heart J 1974; 36:417.
- LEV M, GIBSON S, MILLER RA. Ebstein's disease with Wolff-Parkinson-White syndrome; report of a case with a histopathologic study of possible conduction pathways. Am Heart J 1955; 49:724.
- Cappato R, Schlüter M, Weiss C, et al. Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein's anomaly. Circulation 1996; 94:376.
- Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008; 118:e714.
- VAN LINGEN B, BAUERSFELD SR. The electrocardiogram in Ebstein's anomaly of the tricuspid valve. Am Heart J 1955; 50:13.
- Shiina A, Seward JB, Edwards WD, et al. Two-dimensional echocardiographic spectrum of Ebstein's anomaly: detailed anatomic assessment. J Am Coll Cardiol 1984; 3:356.
- Gussenhoven EJ, Stewart PA, Becker AE, et al. "Offsetting" of the septal tricuspid leaflet in normal hearts and in hearts with Ebstein's anomaly. Anatomic and echographic correlation. Am J Cardiol 1984; 54:172.
- Bonow RO, Carabello BA, Chatterjee K, et al. 2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation 2008; 118:e523.
- Zoghbi WA, Enriquez-Sarano M, Foster E, et al. Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography. J Am Soc Echocardiogr 2003; 16:777.
- Graham TP Jr, Driscoll DJ, Gersony WM, et al. Task Force 2: congenital heart disease. J Am Coll Cardiol 2005; 45:1326.
- Knott-Craig CJ, Goldberg SP, Overholt ED, et al. Repair of neonates and young infants with Ebstein's anomaly and related disorders. Ann Thorac Surg 2007; 84:587.
- Knott-Craig CJ, Goldberg SP, Kirklin JK. Surgical strategy to prevent cardiac injury during reoperation in infants. J Cardiothorac Surg 2008; 3:10.
- Attenhofer Jost CH, Edmister WD, Julsrud PR, et al. Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein's anomaly. Int J Cardiovasc Imaging 2012; 28:1147.
- Wilson W, Taubert KA, Gewitz M, et al. Prevention of Infective Endocarditis. Guidelines From the American Heart Association. A Guideline From the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation 2007; 115 published online April 19, 2007. www.circ.ahajournals.org/cgi/reprint/CIRCULATIONAHA.106.183095v1 (Accessed on May 04, 2007).
- Reich JD, Auld D, Hulse E, et al. The Pediatric Radiofrequency Ablation Registry's experience with Ebstein's anomaly. Pediatric Electrophysiology Society. J Cardiovasc Electrophysiol 1998; 9:1370.
- Misaki T, Watanabe G, Iwa T, et al. Surgical treatment of patients with Wolff-Parkinson-White syndrome and associated Ebstein's anomaly. J Thorac Cardiovasc Surg 1995; 110:1702.
- Roten L, Lukac P, DE Groot N, et al. Catheter ablation of arrhythmias in ebstein's anomaly: a multicenter study. J Cardiovasc Electrophysiol 2011; 22:1391.
- Starnes VA, Pitlick PT, Bernstein D, et al. Ebstein's anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg 1991; 101:1082.
- Mair DD. Ebstein's anomaly: natural history and management. J Am Coll Cardiol 1992; 19:1047.
- Augustin N, Schmidt-Habelmann P, Wottke M, et al. Results after surgical repair of Ebstein's anomaly. Ann Thorac Surg 1997; 63:1650.
- Vargas FJ, Mengo G, Granja MA, et al. Tricuspid annuloplasty and ventricular plication for Ebstein's malformation. Ann Thorac Surg 1998; 65:1755.
- Danielson GK. Ebstein's anomaly of the tricuspid valve. In: Pediatric Cardiac Surgery, Mavroudas C, Backer CL (Eds), Mosby, St. Louis 1994. p.413.
- Danielson GK, Driscoll DJ, Mair DD, et al. Operative treatment of Ebstein's anomaly. J Thorac Cardiovasc Surg 1992; 104:1195.
- Carpentier A, Chauvaud S, Macé L, et al. A new reconstructive operation for Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988; 96:92.
- da Silva JP, Baumgratz JF, da Fonseca L, et al. The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg 2007; 133:215.
- Quaegebeur JM, Sreeram N, Fraser AG, et al. Surgery for Ebstein's anomaly: the clinical and echocardiographic evaluation of a new technique. J Am Coll Cardiol 1991; 17:722.
- Hetzer R, Nagdyman N, Ewert P, et al. A modified repair technique for tricuspid incompetence in Ebstein's anomaly. J Thorac Cardiovasc Surg 1998; 115:857.
- Marianeschi SM, McElhinney DB, Reddy VM, et al. Alternative approach to the repair of Ebstein's malformation: intracardiac repair with ventricular unloading. Ann Thorac Surg 1998; 66:1546.
- Wu Q, Huang Z. A new procedure for Ebstein's anomaly. Ann Thorac Surg 2004; 77:470.
- Bove EL, Kirsh MM. Valve replacement for Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1979; 78:229.
- Kiziltan HT, Theodoro DA, Warnes CA, et al. Late results of bioprosthetic tricuspid valve replacement in Ebstein's anomaly. Ann Thorac Surg 1998; 66:1539.
- van Son JA, Falk V, Black MD, et al. Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia. Eur J Cardiothorac Surg 1998; 13:280.
- Kaulitz R, Ziemer G. Modified Fontan procedure for Ebstein's anomaly of the tricuspid valve--an alternative surgical approach preserving Ebstein's anatomy. Thorac Cardiovasc Surg 1995; 43:275.
- Quinonez LG, Dearani JA, Puga FJ, et al. Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle. J Thorac Cardiovasc Surg 2007; 133:1303.
- Malhotra SP, Petrossian E, Reddy VM, et al. Selective right ventricular unloading and novel technical concepts in Ebstein's anomaly. Ann Thorac Surg 2009; 88:1975.
- Kenny D, Hijazi ZM, Walsh KP. Transcatheter tricuspid valve replacement with the Edwards SAPIEN valve. Catheter Cardiovasc Interv 2011; 78:267.
- Roberts PA, Boudjemline Y, Cheatham JP, et al. Percutaneous tricuspid valve replacement in congenital and acquired heart disease. J Am Coll Cardiol 2011; 58:117.
- Freud LR, Escobar-Diaz MC, Kalish BT, et al. Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study. Circulation 2015; 132:481.
- Dearani JA, Bacha E, da Silva JP. Cone Reconstruction of the Tricuspid Valve for Ebstein's Anomaly: Anatomic Repair. Op Tech Thorac Cardiovasc Surg 2008; :109.
- Perloff, JK. The Clinical Recognition of Congenital Heart Disease, 6th ed, Elsevier/Saunders, Philadelphia 2012.
- Connolly HM, Warnes CA. Ebstein's anomaly: outcome of pregnancy. J Am Coll Cardiol 1994; 23:1194.
- Waickman LA, Skorton DJ, Varner MW, et al. Ebstein's anomaly and pregnancy. Am J Cardiol 1984; 53:357.
- Kounis NG, Zavras GM, Papadaki PJ, et al. Pregnancy-induced increase of supraventricular arrhythmias in Wolff-Parkinson-White syndrome. Clin Cardiol 1995; 18:137.
- Donnelly JE, Brown JM, Radford DJ. Pregnancy outcome and Ebstein's anomaly. Br Heart J 1991; 66:368.
- Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol 2007; 49:2303.
- Tricuspid valve
- Right ventricle
- Associated cardiac defects
- CLINICAL PRESENTATION
- Physical examination
- Differential diagnosis
- Chest radiograph
- Arrhythmia evaluation
- Other studies
- Medical therapy
- - Arrhythmia treatment
- Repaired Ebstein's anomaly
- PARTICIPATION IN ATHLETICS
- SUMMARY AND RECOMMENDATIONS