Skip to main content Accessibility help

Surgical treatment of Ebstein's malformation: state of the art in 2006

  • Joseph A. Dearani (a1), Patrick W. O'Leary (a2) and Gordon K. Danielson (a1)


Ebstein's malformation is a congenital anomaly of the tricuspid valve and right ventricle that is characterized by several features, each of which can exhibit a spectrum of malformation. The first is adherence of the leaflets of the tricuspid valve to the underlying myocardium, this representing failure of delamination during development. This feature involves the septal and inferior leaflets, but rarely the anterior leaflet (Fig. 1). The second feature is anterior and apical rotational displacement of the functional annulus (Fig. 2). The third abnormality is dilation of the “atrialized” portion of the right ventricle, with variable degrees of hypertrophy and thinning of the wall. The fourth finding is redundancy, fenestrations, and tethering of the anterior leaflet. A fifth abnormality is dilation of the right atrioventricular junction, this being the true tricuspid valvar annulus. The final feature is variable ventricular myocardial dysfunction. Each heart with Ebstein's malformation is different, and there is an infinite variability that can occur with the above mentioned characteristics. These anatomical and functional abnormalities cause important tricuspid regurgitation, which results in right atrial and right ventricular dilation, and atrial and ventricular arrhythmias.


Corresponding author

Correspondence to: Joseph A. Dearani MD, 200 First Street, SW, Rochester, Minnesota 55905, United States of America. Tel: +1 507 255 2034; Fax: +1 507 255 7378; E-mail:


Hide All


Leung MP, Baker EJ, Anderson RH, Zuberbuhler JR. Cineangiographic spectrum of Ebstein's malformation: its relevance to clinical presentation and outcome. J Am Coll Cardiol 1988; 11: 154161.
Anderson KR, Lie JT. The right ventricular myocardium in Ebstein's anomaly: a morphometric histopathologic study. Mayo Clinic Proc 1979; 54: 181184.
Danielson GK, Maloney JD, Devloo RAE. Surgical repair of Ebstein's anomaly. Mayo Clinic Proc 1979; 54: 185192.
Hunter SW, Lillehei W. Ebstein's malformation of the tricuspid valve: study of a case together with suggestion of a new form of surgical therapy. Dis Chest 1958; 33: 297304.
Dearani JA, Danielson GK. Surgical management of Ebstein's anomaly in the adult. Semin Thorac Cardiovasc Surg 2005; 17: 148154.
Kiziltan HT, Theodoro DA, Warnes CA, O'Leary PW, Anderson BJ, Danielson GK. Late results of bioprosthetic tricuspid valve replacement for Ebstein's anomaly. Ann Thorac Surg 1998; 66: 15391545.
Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC. Operative treatment of Ebstein's anomaly. J Thorac Cardiovasc Surg 1992; 104: 11951202.
Boston US, Dearani JA, O'Leary PW, Driscoll DJ, Danielson GK. Tricuspid valve repair for Ebstein's anomaly in young children: a 30-year experience. Ann Thorac Surg 2006; 81: 690696.
Theodoro DA, Danielson GK, Porter CJ, Warnes CA. Right-sided maze procedure for right atrial arrhythmias in congenital heart disease. Ann Thorac Surg 1998; 65: 149154.
Greason KL, Dearani JA, Theodoro DA, Porter CB, Warnes CA, Danielson GK. Surgical management of atrial tachyarrhythmias associated with congenital cardiac anomalies: Mayo Clinic Experience. Ped Cardiac Surg Annual Semin Thorac and Cardiovasc Surg 2003; 6: 5971.
Khositseth A, Danielson GK, Dearani JA, Munger TM, Porter CJ. Supraventricular tachyarrhythmias in Ebstein's anomaly: management and outcome. J Thorac Cardiovasc Surg 2004; 128: 826833.
Theodoro DA, Danielson GK, Kiziltan HT, et al. Surgical management of Ebstein's anomaly: a 25-year experience. Circulation Suppl 1997; 96: I507.
Dearani JA, Danielson GK. Congenital Heart Surgery Nomenclature and Database Project: Ebstein's anomaly and tricuspid valve disease. Ann Thorac Surg 2000; 69: S106S117.
Driscoll DJ, Mottram CD, Danielson GK. Spectrum of exercise intolerance in 45 patients with Ebstein's anomaly and observations on exercise tolerance in 11 patients after surgical repair. J Am Coll Cardiol 1988; 11: 831836.
Schreiber C, Cook A, Ho SY, Augustin N, Anderson RH. Morphologic spectrum of Ebstein's malformation: revisitation relative to surgical repair. J Thorac Cardiovasc Surg 1999; 117: 148155.


Surgical treatment of Ebstein's malformation: state of the art in 2006

  • Joseph A. Dearani (a1), Patrick W. O'Leary (a2) and Gordon K. Danielson (a1)


Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed