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Published in: International Journal of Arrhythmia 1/2023

Open Access 01-12-2023 | AV Reentrant Tachycardia | Case Report

Accessory pathways in monozygotic twins with different clinical phenotypes: a case report

Authors: Halim Marzak, Simon Fitouchi, Thomas Cardi, Mohamad Kanso, Alexandre Schatz, Laurence Jesel

Published in: International Journal of Arrhythmia | Issue 1/2023

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Abstract

Background

The atrioventricular reentrant tachycardia (AVRT) is the most common tachycardia associated with accessory pathways (APs). Although sporadic Wolff-Parkinson-White (WPW) syndrome has been well-described, AP occurrence in identical twins with WPW syndrome remains rarely reported.

Case presentation

We report a case of 14-year-old monozygotic twin brothers referred for an electrophysiology (EP) study. Twin A presented with recurrent symptomatic narrow complex tachycardia after exercise, noted for 3 years. His 12-lead surface electrocardiogram (ECG) did not show ventricular pre-excitation. However, an orthodromic AVRT utilizing a concealed right posteroseptal AP was found and successfully ablated. AVRT did not recur 12 months after the procedure. Twin B was asymptomatic. During his medical examination for firefighter volunteerism, his 12-lead ECG showed a spontaneous ventricular pre-excitation. EP study revealed a short anterograde right midseptal AP, which was then successfully eliminated by catheter ablation. His 12-lead ECG showed no ventricular pre-excitation recurrence 12 months after the procedure.

Conclusions

These identical twin brothers had a right-side AP in almost the same place but showed completely different phenotypes. This case clearly illustrates the difficulty in understanding genetic contribution in the origin of atrioventricular APs. Environmental exposure could play a role in their clinical presentations and AP electrophysiological properties.
Literature
1.
go back to reference Kuck KH, Friday KJ, Kunze KP, Schluter M, Lazzara R, Jackman WM. Sites of conduction block in accessory atrioventricular pathways Basis for concealed accessory pathways. Circulation. 1990;82:407–17.CrossRefPubMed Kuck KH, Friday KJ, Kunze KP, Schluter M, Lazzara R, Jackman WM. Sites of conduction block in accessory atrioventricular pathways Basis for concealed accessory pathways. Circulation. 1990;82:407–17.CrossRefPubMed
2.
go back to reference Gilette PC, Freed D, McNamara DG. A proposed autosomal dominant method of inheritance of the Wolff-Parkinson-White syndrome and supraventricular tachycardia. J Pediatr. 1978;93:257–8.CrossRef Gilette PC, Freed D, McNamara DG. A proposed autosomal dominant method of inheritance of the Wolff-Parkinson-White syndrome and supraventricular tachycardia. J Pediatr. 1978;93:257–8.CrossRef
3.
go back to reference Vidaillet HJ, Pressley JC, Henke E, Harrell FE, German LD. Familial occurrence of accessory atrioventricular pathways (preexcitation syndrome). N Engl J Med. 1987;317:65–9.CrossRefPubMed Vidaillet HJ, Pressley JC, Henke E, Harrell FE, German LD. Familial occurrence of accessory atrioventricular pathways (preexcitation syndrome). N Engl J Med. 1987;317:65–9.CrossRefPubMed
4.
go back to reference Ahmad F, Arad M, Musi N, He H, Wolf C, Branco D, et al. Increased alpha2 subunit-associated AMPK activity and PRKAG2 cardiomyopathy. Circulation. 2005;112:3140–8.CrossRefPubMed Ahmad F, Arad M, Musi N, He H, Wolf C, Branco D, et al. Increased alpha2 subunit-associated AMPK activity and PRKAG2 cardiomyopathy. Circulation. 2005;112:3140–8.CrossRefPubMed
5.
go back to reference Arad M, Moskowitz IP, Patel VV, Ahmad F, Perez-Atayde AR, Sawyer DB, et al. Transgenic mice overexpressing mutant PRKAG2 define the cause of Wolff-Parkinson-White syndrome in glycogen storage cardiomyopathy. Circulation. 2003;107:2850–6.CrossRefPubMed Arad M, Moskowitz IP, Patel VV, Ahmad F, Perez-Atayde AR, Sawyer DB, et al. Transgenic mice overexpressing mutant PRKAG2 define the cause of Wolff-Parkinson-White syndrome in glycogen storage cardiomyopathy. Circulation. 2003;107:2850–6.CrossRefPubMed
6.
go back to reference Wolf CM, Arad M, Ahmad F, Sanbe A, Bernstein SA, Toka O, et al. Reversibility of PRKAG2 glycogen-storage cardiomyopathy and electrophysiological manifestations. Circulation. 2008;117:144–54.CrossRefPubMed Wolf CM, Arad M, Ahmad F, Sanbe A, Bernstein SA, Toka O, et al. Reversibility of PRKAG2 glycogen-storage cardiomyopathy and electrophysiological manifestations. Circulation. 2008;117:144–54.CrossRefPubMed
7.
go back to reference Caldararu C, Alexandru R, Bartos D, Vatasescu RG. Identical anatomical location of accessory pathway in a family with Wolff-Parkinson-White syndrome. Europace. 2010;12:582–3.CrossRefPubMed Caldararu C, Alexandru R, Bartos D, Vatasescu RG. Identical anatomical location of accessory pathway in a family with Wolff-Parkinson-White syndrome. Europace. 2010;12:582–3.CrossRefPubMed
8.
go back to reference Ehtisham J, Watkins H. Is Wolff-Parkinson-White syndrome a genetic disease ? J Cardiovasc Electrophysiol. 2005;16:1258–62.CrossRefPubMed Ehtisham J, Watkins H. Is Wolff-Parkinson-White syndrome a genetic disease ? J Cardiovasc Electrophysiol. 2005;16:1258–62.CrossRefPubMed
9.
go back to reference Vaughan CJ, Hom Y, Okin DA, McDermott DA, Lerman BB, Basson CT. Molecular genetic analysis of PRKAG2 in sporadic Wolff-Parkinson-White syndrome. J Cardiovasc Electrophysiol. 2003;14:263–8.CrossRefPubMed Vaughan CJ, Hom Y, Okin DA, McDermott DA, Lerman BB, Basson CT. Molecular genetic analysis of PRKAG2 in sporadic Wolff-Parkinson-White syndrome. J Cardiovasc Electrophysiol. 2003;14:263–8.CrossRefPubMed
10.
go back to reference Coban-Akdemir ZH, Charng WL, Azamian M, Paine IS, Punetha J, Grochowski CM, et al. Wolff-Parkinson-White syndrome: de novo variants and evidence for mutational burden in genes associated with atrial fibrillation. Am J Med Genet A. 2020;182:1387–99.CrossRefPubMedPubMedCentral Coban-Akdemir ZH, Charng WL, Azamian M, Paine IS, Punetha J, Grochowski CM, et al. Wolff-Parkinson-White syndrome: de novo variants and evidence for mutational burden in genes associated with atrial fibrillation. Am J Med Genet A. 2020;182:1387–99.CrossRefPubMedPubMedCentral
11.
go back to reference Harnischfeger WW. Hereditary occurrence of the pre-excitation (Wolff-Parkinson-White) syndrome with re-entry mechanism and concealed conduction. Circulation. 1959;19:28–40.CrossRefPubMed Harnischfeger WW. Hereditary occurrence of the pre-excitation (Wolff-Parkinson-White) syndrome with re-entry mechanism and concealed conduction. Circulation. 1959;19:28–40.CrossRefPubMed
12.
go back to reference Field ME, Laffin JJ, Langberg JJ, Von Bergen NH. Isolated Wolff-Parkinson-White syndrome in identical twins. Heart Rhythm Case Reports. 2018;4:138–40.CrossRefPubMedPubMedCentral Field ME, Laffin JJ, Langberg JJ, Von Bergen NH. Isolated Wolff-Parkinson-White syndrome in identical twins. Heart Rhythm Case Reports. 2018;4:138–40.CrossRefPubMedPubMedCentral
13.
go back to reference Lu CW, Wu MH, Chu SH. Paroxysmal supraventricular tachycardia in identical twins with the same left lateral accessory pathways and innocent dual atrioventricular pathways. Pacing Clin Electrophysiol. 2000;23:1564–6.CrossRefPubMed Lu CW, Wu MH, Chu SH. Paroxysmal supraventricular tachycardia in identical twins with the same left lateral accessory pathways and innocent dual atrioventricular pathways. Pacing Clin Electrophysiol. 2000;23:1564–6.CrossRefPubMed
Metadata
Title
Accessory pathways in monozygotic twins with different clinical phenotypes: a case report
Authors
Halim Marzak
Simon Fitouchi
Thomas Cardi
Mohamad Kanso
Alexandre Schatz
Laurence Jesel
Publication date
01-12-2023
Publisher
BioMed Central
Published in
International Journal of Arrhythmia / Issue 1/2023
Electronic ISSN: 2466-1171
DOI
https://doi.org/10.1186/s42444-023-00091-5

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