Document Type : Case Report


1 Professor, Adult Congenital Heart Diseases, Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Iran

2 Assistant Professor, Adult Congenital Heart Diseases, Cardiovascular Research Center, Institute of Basic and Clinical Physiology Sciences, Kerman University of Medical Sciences, Kerman, Iran


BACKGROUND: Ebstein’s anomaly is one of the challenging congenital heart diseases (CHDs) that is presented with different anatomical and clinical symptoms. For this reason, patients with this complication require exact diagnostic methods and appropriate treatment approaches. In addition, multiple cardiac defects accompany this anomaly.CASE REPORT: In this study, we describe a rare associated defect in an adult patient with Ebstein’s anomaly and proper surgical methods performed for her.CONCLUSION: The most important issue in adult patients with Ebstein’s anomaly is to pay close attention to the presence of associated anomalies, in which careful examination and use of para-clinical methods is very helpful. Furthermore, accurate diagnosis of the associated defects determines the treatment and surgery of patients.


  1. Dearani JA, Danielson GK. Surgical management of Ebstein's anomaly in the adult. Semin Thorac Cardiovasc Surg 2005; 17(2): 148-54.
  2. Makous N, Vander Veer JB. Ebstein's anomaly and life expectancy. Report of a survival to over age 79. Am J Cardiol 1966; 18(1): 100-4.
  3. Attenhofer Jost CH, Connolly HM, Edwards WD, Hayes D, Warnes CA, Danielson GK. Ebstein's anomaly - review of a multifaceted congenital cardiac condition. Swiss Med Wkly 2005; 135(19-20): 269-81.
  4. Nihoyannopoulos P, McKenna WJ, Smith G, Foale R. Echocardiographic assessment of the right ventricle in Ebstein's anomaly: Relation to clinical outcome. J Am Coll Cardiol 1986; 8(3): 627-35.
  5. Attenhofer Jost CH, Connolly HM, O'Leary PW, Warnes CA, Tajik AJ, Seward JB. Left heart lesions in patients with Ebstein anomaly. Mayo Clin Proc 2005; 80(3): 361-8.
  6. Khajali Z, Maleki M, Mohebbi B, Aliramezany M. Coarctation stenting in a rare case with congenitally corrected transposition of the great arteries and the bicuspid aortic valve. J Tehran Heart Cent 2020; 15(1): 27-30.
  7. Giuliani ER, Fuster V, Brandenburg RO, Mair DD. Ebstein's anomaly: the clinical features and natural history of Ebstein's anomaly of the tricuspid valve. Mayo Clin Proc 1979; 54(3): 163-73.
  8. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein's anomaly. Circulation 2007; 115(2): 277-85.
  9. Eustace S, Kruskal JB, Hartnell GG. Ebstein's anomaly presenting in adulthood: the role of cine magnetic resonance imaging in diagnosis. Clin Radiol 1994; 49(10): 690-2.
  10. Knott-Craig CJ, Overholt ED, Ward KE, Ringewald JM, Baker SS, Razook JD. Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up. Ann Thorac Surg 2002; 73(6): 1786-92.