European Journal of Pediatrics

, Volume 172, Issue 11, pp 1491–1500 | Cite as

Dyssynchronous ventricular contraction in Wolff–Parkinson–White syndrome: a risk factor for the development of dilated cardiomyopathy

  • Chen-Cheng Dai
  • Bao-Jing Guo
  • Wen-Xiu Li
  • Yan-Yan Xiao
  • Mei Jin
  • Lin Han
  • Jing-Ping Sun
  • Cheuk-Man Yu
  • Jian-Zeng DongEmail author
Original Article


Emerging evidence suggests that significant left ventricular dysfunction may arise in right-sided septal or paraseptal accessory pathways (APs) with Wolff–Parkinson–White syndrome, even in the absence of recurrent or incessant tachycardia. During 1 year and 9 months, we identified four consecutive female children with median age of 8 years diagnosed as having dilated cardiomyopathy (DCM) combined with overt right-sided APs several years ago. Incessant or recurrent tachycardia as the cause of DCM could be excluded. Anti-heart failure chemotherapy did not produce satisfactory effects. The patients underwent radiofrequency ablations (RFCAs). This report describes the clinical and echocardiographic characteristics of the cases before and after the ablation. Dyssynchronous ventricular contraction was observed in all patients. The locations of the APs were the right-sided anteroseptum and the free wall (n = 2 each). All patients received successful RFCAs. Their physical activities and growth improved greatly, and the echocardiographic data demonstrated that their left ventricular (LV) contraction recovered to synchrony shortly after the ablation and that their LV function recovered to normal gradually during the follow-up. Conclusions: A causal relationship between overt ventricular preexcitation and the development of DCM is supported by the complete recovery of LV function and reversed LV remodeling after the loss of ventricular preexcitation. Preexcitation-related dyssynchrony was probably the crucial mechanism. Not only right-sided septal or paraseptal but also free wall overt APs may induce LV dysfunction and even DCM. AP-induced DCM is an indication for ablation with a good prognosis.


Wolff–Parkinson–White (WPW) syndrome Accessory pathway (AP) Dilated cardiomyopathy (DCM) Speckle tracking echocardiography Segmental dyskinesia 



Dr. Chen-Cheng Dai is financially supported by a Science Foundation from Dean of Capital Medical University affiliated Beijing Anzhen Hospital (project no. 2013Z09).


  1. 1.
    Cadrin-Tourigny J, Fournier A, Andelfinger G, Khairy P (2008) Severe left ventricular dysfunction in infants with ventricular preexcitation. Hear Rhythm 5:1320–1322. doi: 10.1016/j.hrthm.2008.05.022 CrossRefGoogle Scholar
  2. 2.
    Cheng A, Helm RH, Abraham TP (2009) Pathophysiological mechanisms underlying ventricular dyssynchrony. Europace 11(Suppl):v10–v14. doi: 10.1093/europace/eup272 PubMedCrossRefGoogle Scholar
  3. 3.
    De Boeck BW, Teske AJ, Leenders GE, Mohamed Hoesein FA, Loh P, van Driel VJ, Doevendans PA, Prinzen FW, Cramer MJ (2010) Detection and quantification by deformation imaging of the functional impact of septal compared to free wall preexcitation in the Wolff–Parkinson–White syndrome. Am J Cardiol 106:539–546. doi: 10.1016/j.amjcard.2010.03.066 PubMedCrossRefGoogle Scholar
  4. 4.
    DeMaria AN, Vera Z, Neumann A, Mason DT (1976) Alterations in ventricular contraction pattern in the Wolff–Parkinson–White syndrome: detection by echocardiography. Circulation 53:249–257. doi: 10.1161/01.CIR.53.2.249 PubMedCrossRefGoogle Scholar
  5. 5.
    Emmel M, Balaji S, Sreeram N (2004) Ventricular preexcitation associated with dilated cardiomyopathy: a causal relationship? Cardiol Young 14(6):594–599. doi: 10.1017/S1047951104006031 PubMedCrossRefGoogle Scholar
  6. 6.
    Fazio G, Mongiovi' M, Sutera L, Novo G, Novo S, Pipitone S (2008) Segmental dyskinesia in Wolff–Parkinson–White syndrome: a possible cause of dilatative cardiomyopathy. Int J Cardiol 123:e31–e34. doi: 10.1016/j.ijcard.2006.11.109 PubMedCrossRefGoogle Scholar
  7. 7.
    Fukunaga H, Akimoto K, Furukawa T, Takahashi K, Kishiro M, Shimizu T, Kamiyama H, Sumitomo N (2013) Improvement in non-tachycardia-induced cardiac failure after radiofrequency catheter ablation in a child with a right-sided accessory pathway. Heart Vessels. doi: 10.1007/s00380-013-0322-5 PubMedGoogle Scholar
  8. 8.
    Gorcsan J 3rd, Abraham T, Agler DA, Bax JJ, Derumeaux G, Grimm RA, Martin R, Steinberg JS, Sutton MS, Yu CM (2008) Echocardiography for cardiac resynchronization therapy: recommendations for performance and reporting—a report from the American Society of Echocardiography Dyssynchrony Writing Group endorsed by the Heart Rhythm Society. J Am Soc Echocardiogr 21:191–213. doi: 10.1097/HCO.0b013e3282f2e260 PubMedCrossRefGoogle Scholar
  9. 9.
    Hishida H, Sotobata I, Koike Y, Okumura M, Mizuno Y (1976) Echocardiographic patterns of ventricular contraction in the Wolff–Parkinson–White syndrome. Circulation 54:567–570. doi: 10.1161/01.CIR.54.4.567 PubMedCrossRefGoogle Scholar
  10. 10.
    Iwasaku T, Hirooka K, Taniguchi T, Hamano G, Utsunomiya Y, Nakagawa A, Koide M, Ishizu T, Yamato M, Sasaki N, Yamamoto H, Kawaguchi Y, Mizuno H, Koretsune Y, Kusuoka H, Yasumura Y (2009) Successful catheter ablation to accessory atrioventricular pathway as cardiac resynchronization therapy in a patient with dilated cardiomyopathy. Europace 11:121–123. doi: 10.1093/europace/eun318 PubMedCrossRefGoogle Scholar
  11. 11.
    Ko J (2012) Left ventricular dysfunction and dilated cardiomyopathy in infants and children with Wolff–Parkinson–White syndrome in the absence of tachyarrhythmias. Korean Circ J 42(12):803–808. doi: 10.4070/kcj.2012.42.12.803 PubMedCrossRefGoogle Scholar
  12. 12.
    Kwon BS, Bee EJ, Kim GB, Noh CN, Choi JY, Yun YS (2010) Septal dyskinesia and global left ventricular dysfunction in pediatric Wolff–Parkinson–White syndrome with septal accessory pathway. J Cardiovasc Electrophysiol 21:290–295PubMedCrossRefGoogle Scholar
  13. 13.
    Modesto KM, Cauduro S, Dispenzieri A, Khandheria B, Belohlavek M, Lysyansky P, Friedman Z, Gertz M, Abraham TP (2006) Two-dimensional acoustic pattern derived strain parameters closely correlate with one-dimensional tissue Doppler derived strain measurements. Eur J Echocardiogr 7:315–321. doi: 10.1016/j.euje.2005.07.003 PubMedCrossRefGoogle Scholar
  14. 14.
    Prinzen FW, Cheriex EM, Delhaas T, van Oosterhout MF, Arts T, Wellens HJ, Reneman RS (1995) Asymmetric thickness of the left ventricular wall resulting from asynchronous electrical activation: a study in patients with left bundle branch block in dogs with ventricular pacing. Am Heart J 130:1045–1053. doi: 10.1016/0002-8703(95)90207-4 PubMedCrossRefGoogle Scholar
  15. 15.
    Sasse L (1977) Interventricular septal motion in Wolff–Parkinson–White syndrome. Am J Cardiol 39:469–470. doi: 10.1016/S0002-9149(77)80108-2 PubMedCrossRefGoogle Scholar
  16. 16.
    Suffoletto MS, Dohi K, Cannesson M, Saba S, Gorcsan J 3rd (2006) Novel speckle-tracking radial strain from routine black-and-white echocardiographic images to quantify dyssynchrony and predict response to cardiac resynchronization therapy. Circulation 113:960–968. doi: 10.1161/CIRCULATIONAHA.105.571455 PubMedCrossRefGoogle Scholar
  17. 17.
    Szmit S, Stec S, Szymański P, Opolski G (2008) Improvement of cardiopulmonary exercise capacity after radiofrequency ablation in patient with preexcitation during sinus rhythm: a new definition of symptomatic preexcitation? Hear Rhythm 5:1323–1326. doi: 10.1016/j.hrthm.2008.07.003 CrossRefGoogle Scholar
  18. 18.
    Tantengco MV, Thomas RL, Karpawich PP (2001) Left ventricular dysfunction after long-term right ventricular apical pacing in the young. J Am Coll Cardiol 37:2093–2100. doi: 10.1016/S0735-1097(01)01302-X PubMedCrossRefGoogle Scholar
  19. 19.
    Thambo JB, Bordachar P, Garrigue S, Lafitte S, Sanders P, Reuter S, Girardot R, Crepin D, Reant P, Roudaut R, Jaïs P, Haïssaguerre M, Clementy J, Jimenez M (2004) Detrimental ventricular remodeling in patients with congenital complete heart block and chronic right ventricular apical pacing. Circulation 310:3766–3772. doi: 10.1161/01.CIR.0000150336.86033.8D CrossRefGoogle Scholar
  20. 20.
    Tomaske M, Janousek J, Rázek V, Gebauer RA, Tomek V, Hindricks G, Knirsch W, Bauersfeld U (2008) Adverse effects of Wolff–Parkinson–White syndrome with right septal or posteroseptal accessory pathways on cardiac function. Europace 10:181–189. doi: 10.1093/europace/eun005 PubMedCrossRefGoogle Scholar
  21. 21.
    Udink ten Cate FE, Kruessell MA, Wagner K, Trieschmann U, Emmel M, Brockmeier K, Sreeram N (2010) Dilated cardiomyopathy in children with ventricular preexcitation: the location of the accessory pathway is predictive of this association. J Electrocardiol 43:146–154. doi: 10.1016/j.jelectrocard.2009.09.007 PubMedCrossRefGoogle Scholar
  22. 22.
    Udink ten Cate FE, Wiesner N, Trieschmann U, Khalil M, Sreeram N (2010) Dyssynchronous ventricular activation in asymptomatic Wolff–Parkinson–White syndrome: a risk factor for development of dilated cardiomyopathy. Indian Pacing Electrophysiol J 10:248–56PubMedGoogle Scholar
  23. 23.
    Winter S, Meyer C, Martinek M, Pürerfellner H, Nesser HJ (2011) Cardiac resynchronization therapy by ablation of right-anterolateral accessory pathway. Echocardiography 28(6):E108–E111. doi: 10.1111/j.1540-8175.2010.01364.x PubMedCrossRefGoogle Scholar
  24. 24.
    Yamanak S, Shirayama T, Inoue K, Kawata K, Yagi T, Azuma A, Inoue D, Nakagawa M (1998) Improved cardiac function after catheter ablation in a patient with type B Wollf–Parkinson–White syndrome with an old myocardial infarction. Jpn Circ J 62:860–862. doi: 10.1253/jcj62.860 CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Chen-Cheng Dai
    • 1
  • Bao-Jing Guo
    • 1
  • Wen-Xiu Li
    • 1
  • Yan-Yan Xiao
    • 1
  • Mei Jin
    • 1
  • Lin Han
    • 1
  • Jing-Ping Sun
    • 2
  • Cheuk-Man Yu
    • 2
  • Jian-Zeng Dong
    • 3
    Email author
  1. 1.Department of Pediatric CardiologyCapital Medical University, Beijing Anzhen HospitalBeijingChina
  2. 2.Department of CardiologyPrince of Wales Hospital, The Chinese University of Hong KongHong KongChina
  3. 3.Department of CardiologyCapital Medical University, Beijing Anzhen HospitalBeijingChina

Personalised recommendations