Thorac Cardiovasc Surg 2000; 48(4): 214-219
DOI: 10.1055/s-2000-6897
Original Cardiovascular
ORIGINALARBEIT
© Georg Thieme Verlag Stuttgart · New York

Ebstein's Anomaly in Adults.Arrhythmias: Diagnosis and Therapeutic Approach

J. Hebe
  • Department Cardiology, St. Georg Hospital, Hamburg, Germany
Further Information

Publication History

Publication Date:
31 December 2000 (online)

Patients with Ebstein's anomaly (M. E.) are known to have a high potential for developing arrhythmia, in the vast majority, of the tachycardia type. Most of these tachycardias are based on accessory pathways (AP) located along the anomalous atrioventricular valve, found in up to 30 % of this patient cohort. Next to this main representative for congenital arrhythmogenic substrates, various types of acquired tachycardia were found in patients with M. E., such as atrial ectopic tachycardia (AET), atrial flutter (A. Flutt.), atrial reentry tachycardia (ART), atrial fibrillation and ventricular tachyarrhythmia. Refractoriness to medical treatment and a higher potential for side effects in these patients resulted in an increasing referral to interventional electrophysiology recent years. A total of 37 patients with M. E. and tachycardia underwent an attempt for radiofrequency current (RFC) treatment at our institution, 30 of whom were adults aged 18 to 61 yrs (33 ± 12.2. yrs). Just 4 patients under went surgery for reconstruction of the anomalous valve and closure of an ASD (2 patients), in whom a total of 6 cases of atrial tachycardia were found, 3 common A. Flutt. and 3 atriotomy-scar related ART. Of the 26 unoperated patients, one presented with typical AV-nodal reentry tachycardia and the remaining patients with atrioventricular reentry tachycardia, based on a total of 39 APs. 27/39 Aps had bidirectional conduction properties, causing the WPW syndrome in 16 patients, 8 APs were concealed and the remaining 4 were Mahaim fibers. Within 39 sessions, 26 of the 30 patients were successfully treated by RFC ablation of the atrhythmogenic substrates. Mean session duration lasted for 313 ± 151 min (range 95 to 660 min), and a fluoroscopy time of 56.0 ± 38.4 min (2.2 to 146.8 min) was required. There were no severe acute complications. Conclusions: In patients with M. E. not only a high potential for the development of tachycardia, but also a tendency towards multiple arrhythmogenic substrates in the single patient can be ascertained. RFC ablation can be used safely and effectively for treatment for various types of tachycardias in patients with M. E.. Such therapy carries the potential for a definitive treatment and should be taken early into consideration as it meets the requirements this patient cohort.

References

  • 1 Anderson R H, Ho S Y. Anatomy of the atrioventricular junctions with regard to ventricular preexitation.  Pacing Clin Electrophysiol. 1997;  20 ((8/2)) 2072-2076
  • 2 Attie F, Casanova J M. et al . Ebstein's anomaly. Clinical profile in 174 patients.  Arch Inst Cardiol Mex. 1999;  69 ((1)) 17-25
  • 3 Behl P R, Blesocsky A. Ebstein's anomaly: sixteen years experience with valve replacement without plication of the right ventricle.  Thorax. 1984;  39 8
  • 4 Brugada J, Martinez Sanchez J. Radiofrequency catheter ablation of atriofascicular accessory pathways guided by discrete electrical potentials recorded at the tricuspid annulus.  Pacing Clin Electrophysiol. 1995;  18 ((7)) 1388-1194
  • 5 Calkins H, Sousa J. et al . Diagnosis and cure of the Wolff-Parkinson-White syndrome paroxysmal supraventricular tachycardias during a single electrophysiologic test.  N Engl J Med. 1991;  324 1612-1618
  • 6 Cosio F G, Arribas F, Lopez-Gil M, Gonzales H D. Radiofrequency ablation of atrial flutter.  J Cardiovasc Electrophysiol. 1996;  7 60-70
  • 7 Hebe J. Radiofrequency Catheter Ablation of Tachycardia in Patients with Congenital Heart Disease. Ped. Cardiol. in press
  • 8 Hebe J, Antz M. et al . Radiofrequency current ablation of supraventricular tachycardias in congenital heart disease.  Herz. 1998;  23 231-250
  • 9 Hellerstrand K J, Nathan A W. et al . Electrophysiologic effects of flecainide acetate on sinus node function, anomalous atrioventricular connections, and pacemaker thresholds.  Am J Cardiol. 1984;  53 30 B
  • 10 Jackman W M, Wang X. et al . Catheter ablation of accessory atrioventricular pathways (Wolff-Parkinson-White syndrome) by radiofrequency current.  N Engl J Med. 1991;  324 1605-1611
  • 11 Kalman J M, VanHare G F, Olgin J E, Saxon L A, Stark S I, Lesh M. Ablation of “incisional” reentrant atrial tachycardia complicating surgery for congenital heart disease. Use of entrainment to define a critical isthmus of conduction.  Circulation. 1996;  93(3) 502-512
  • 12 Kastor J A, Goldreyer B N. et al . Electrophysiologic characteristics of Ebstein's anomaly of the tricuspid valve.  Circulation. 1975;  52 987
  • 13 Klein G J, Guerdon G M. et al . “Nodoventricular O¿ accessory pathway: evidence for a distinct accessory atrioventricular pathway with atrioventricular node-like properties.  J Am CollCardiol. 1988;  11 1035
  • 14 Kuck K-H, Schlüter M. et al . Radiofrequency current catheter ablation of accessory atrioventricular pathways.  Lancet. 1991;  337 1557-1561
  • 15 Kumar A E, Fyler D C. et al . Ebstein's anomaly: clinical profile and natural history.  Am J Cardiol. 1971;  28 84
  • 16 Nakagawa H, Lazzara R, Khastgir T. et al . Role of the tricuspid annulus and the eustachian valve/ridge on atrial flutter.  Circulation. 1996;  94 407-424
  • 17 Oh J K, Holmers D R. et al . Cardiac arrhythmias in patients with surgical repair of Ebstein's anomaly.  J Am Cardiol. 1985;  6 1351
  • 18 Perloff J K. The Clinical Recognition of Congenital Heart Disease. Philadelphia; W.B. Saunders Co 3rd ed. 1987
  • 19 Pressley J C, Wharton J M. et al . Effect of Ebstein's anomaly on short- and long-term outcome of surgically treated patients with Wolff-Parkinson-White syndrome.  Circulation. 1992 Oct;  4 1147-1155
  • 20 Price J E, Amsterdam E A. et al . Ebsteins disease associated with complete atrioventricular block.  Chest. 1978;  73 524
  • 21 Reich J D, Auld D. et al . The Pediatric Radiofrequency Ablation Registry's experience with Ebstein's anomaly. Pediatric Electrophysiology Society.  J Cardiovasc Electrophysiol. 1998;  9 ((12)) 1370-1377
  • 22 Rossi L, Thiene G. Mild Ebstein's anomaly associated with supraventricular tachycardia and sudden death: clinicomorphologic features in 3 patients.  Am J Cardiol. 1984;  53 332
  • 23 Schoen W J, Fujimura O. Variant preexitation syndrome: a true nodoventricular mahaim fiber or an accessory atrioventricular pathway with decremental properties?.  J Cardiovasc Electrophysiol. 1995;  6 ((12)) 1117-1123
  • 24 Smith W M, Gallagher J J. et al . The electrophysiologic basis and management of symptomatic recurrent tachycardia in patients with Ebstein's anomaly of the tricuspid valve.  Am J Cardiol. 1982;  49 1223
  • 25 Tchou P, Lehmann M H. et al . Atriofascicular connection or a nodoventricular fiber Mahaim fiber? Electrophysiologic elucidation of the pathway and associated reentrant circuit.  Circulation. 1988;  77 837
  • 26 Triedman J K, Jenkins K J, Colan S D, Saul J P, Walsh E P. Intra-atrial reentrant tachycardia after palliation of congenital heart disease: characterization of multiple macroreentrant circuits using fluoroscopically based three-dimensional endocardial mapping.  J Cardiovasc Electrophysiol. 1997;  8 (3) 259-270
  • 27 Van Hare G, Lesh M D. et al . Radiofrequency current ablation of supraventricular arrhythmias in patients with congenital heart disease: results and technical considerations.  JACC. 1993;  22 (No. 3) 883-890
  • 28 Watson H. Natural History of Ebstein's anomaly of the tricuspid valve. in childhood and adolescense: an intemational co-operative study of 505 cases.  Br. Heart J. 1974;  36 417
  • 29 Ward D E, Camm J. et al . Ebstein's anomaly in association with anomalous nodoventricular conduction: Pre-operative an intra-operative electrophysiological studies.  J. Electrocardiol. 1979;  12 227

Dr. med. Joachim Hebe

Kardiologie, Rhythmologie/Angeborene Herzfehler Allgemeines Krankenhaus St. Georg

Lohmühlenstraße 5

20099 Hamburg

Germany

Phone: + 49 040 2890-3616

Fax: + 49 040 2890-4435

Email: JOBAHEBE@AOL.COM