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.
Key words:
Ebstein's anomaly - Adult Anomaly - Arrhythmia - Treatment - RFC-Ablation - Congenital
Heart Disease - Tachycardia
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
