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Preexcitation Syndromes Associated with Congenital Heart Disease

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Cardiac Preexcitation Syndromes

Abstract

Although preexcitation is a congenital heart defect, the focus of this chapter will be on the congenital heart abnormalities associated with preexcitation. The simultaneous occurrence of preexcitation syndromes with tachyarrhythmias and certain types of congenital heart disease — for example, Ebstein’s malformation — is not unexpected, considering the developmental abnormalities and anatomic substrates involved. Each separate problem may affect the clinical presentation and the management of the other. Because structural anomalies and hemodynamic abnormalities result in dilatation and fibrosis, causing atrial and ventricular ectopy, episodes of tachycardia may be frequent and poorly tolerated. Conversely, frequent episodic or prolonged tachycardia (or both) may result in further deterioration of hemodynamics, in subendocardial ischemia, and in the development of additional structural abnormalities with further fibrosis and chamber dilatation. In addition to these interrelationships, treatment may be affected by the simultaneous occurrence of tachycardia and structural or hemodynamic abnormalities. For example, the use of betaadrenergic blockers, while helpful in treating many patients with preexcitation syndromes and supraventricular tachycardia, may aggravate or be contraindicated in patients with congenital heart disease and ventricular dysfunction.

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References

  1. Öhnell RF: Pre-excitation: a cardiac abnormality; patho-physiological, patho-anatomical and clinical studies of an excitatory spread phenomenon bearing upon the problem of the WPW (Wolff-Parkinson and White) electrocardiogram and paroxysmal tachycardia. Acta Med. Scand. [Suppl] 152: 1, 1944.

    Google Scholar 

  2. Durrer D: Electrical aspects of human cardiac activity: a clinical-physiological approach to excitation and stimulation. Cardiovasc. Res. 2: 1, 1968.

    Article  PubMed  CAS  Google Scholar 

  3. Gallagher JJ, Pritchett ELC, Sealy WC, Kasell J, Wallace AG: The preexcitation syndromes. Prog. Cardiovasc. Dis. 20: 285, 1978.

    Article  PubMed  CAS  Google Scholar 

  4. Anderson RH, Becker AE, Brechenmacher C Davies MJ, Rossi L: Ventricular preexcitation: a proposed nomenclature for its substrates. Eur. J. Cardiol. 3: 27, 1975.

    PubMed  CAS  Google Scholar 

  5. Lev M, Leffler WB, Langendorf R, Pick A: 22. Anatomic findings in a case of ventricular pre-excitation (WPW) terminating in complete atrioventricular block. Circulation 34: 718, 1966.

    PubMed  CAS  Google Scholar 

  6. James TN: Morphology of the human atrioven- 23. tricular node, with remarks pertinent to its electrophysiology. Am. Heart J. 62: 756, 1961.

    Article  PubMed  CAS  Google Scholar 

  7. Denes P, Wu D, Dhingra RC, Chuquimia R, Rosen KM: Demonstration of dual A-V nodal 24. pathways in patients with paroxysmal supra-ventricular tachycardia. Circulation 48: 549, 1973.

    PubMed  CAS  Google Scholar 

  8. Josephson ME, Kastor JA: Supraventricular 25. tachycardia in Lown-Ganong-Levine syndrome: atrionodal versus intranodal reentry. Am. J. Cardiol. 40: 521, 1977.

    Article  PubMed  CAS  Google Scholar 

  9. Davies MJ, Anderson RH, Becker AE: The 26. Conduction System of the Heart. London, Butterworth, 1983, pp. 181–202.

    Google Scholar 

  10. Anderson KR, Zuberbuhler JR, Anderson RH, 27. Becker AE, Lie JT: Morphologic spectrum of Ebstein’s anomaly of the heart: a review. Mayo Clin. Proc. 54: 174, 1979. 28.

    Google Scholar 

  11. Katz LN, Pick A: Cited by Schiebler GL, Adams P Jr, Anderson RC12

    Google Scholar 

  12. Schiebler GL, Adams P Jr, Anderson RC: The Wolff-Parkinson-White syndrome in infants and 29. children: a review and a report of 28 cases. Pediatrics 24: 585, 1959.

    PubMed  CAS  Google Scholar 

  13. Metianu C, de Balsac RH: Les troubles congénitaux de la conduction et du rythme. In Traité des Cardiopathies Congénitales, Donzelot E, 30. D’Allaines F (eds.). Paris, Masson, 1954, p. 1025.

    Google Scholar 

  14. Hecht HH (moderator): Anomalous atrioventricular excitation: panel discussion. Ann. N.Y. Acad. Sci. 65: 826, 1957.

    Article  Google Scholar 

  15. Giardina ACV, Ehlers KH, Engle MA: Wolff- Parkinson-White syndrome in infants and chil- 31. dren: a long-term follow-up study. Br. Heart J. 34: 839, 1972.

    Article  PubMed  CAS  Google Scholar 

  16. Pohlmann F: Beitrag zur Frage der verkürzten Vorhof-Kammerleitung. Ztschr. Klin. Med. 140: 1, 1941.

    Google Scholar 

  17. Kiss P von: “Paladino-Kent-Bündel” beim 32. Kinde. Arch. Kinderheilkd. 122:29, 1941.

    Google Scholar 

  18. Nâdrai A: Die Elektrokardiographie im Säuglings-und Kindesalter. Ergeb. Inn. Med. Kinderheilkd. 60: 688, 1941.

    Article  Google Scholar 

  19. Bodlander JW: The Wolff-Parkinson-White syndrome in association with congenital heart disease: coarctation of the aorta; report of a case. Am. Heart J. 31: 785, 1946. 33.

    Google Scholar 

  20. Moore RM, Dimond G: Case of coarctation of aorta (adult type) with Wolff-Parkinson-White syndrome. J. Indiana Med. Assoc. 39: 352, 1946. 34.

    Google Scholar 

  21. Segers M, Sanabria T, Lequime J, Denolin H: Lesyndrome de Wolff-Parkinson-White: mise en evidence d’une connexion A-V septale directe. Acta Cardiol. 2: 21, 1947.

    Google Scholar 

  22. Rosenberg HS, Klima T, McNamara DG, Leachman RD: Atrioventricular communication in the Wolff-Parkinson-White syndrome. Am. J. Clin. Pathol. 56: 79, 1971.

    PubMed  CAS  Google Scholar 

  23. Gillette PC, Garson A Jr, Kugler JD: WolffParkinson-White syndrome in children: electrophysiologic and pharmacologic characteristics. Circulation 60: 1487, 1979.

    PubMed  CAS  Google Scholar 

  24. Hejtmancik MR, Herrmann GR: The electrocardiographic syndrome of short P-R interval and broad QRS complexes: a clinical study of 80 cases. Am. Heart J. 54: 708, 1957.

    Article  PubMed  CAS  Google Scholar 

  25. El-Said G, Rosenberg HS, Mullins CE, Hallman GL, Cooley DA, McNamara DG: Dysrhythmias after Mustard’s operation for transposition of the great arteries. Am. J. Cardiol. 30: 526, 1972.

    Article  PubMed  CAS  Google Scholar 

  26. Neutze J, Kerr AR, Whitlock RML: Epicardial mapping in a variant of type A Wolff-ParkinsonWhite syndrome. Circulation 48: 662, 1973.

    PubMed  CAS  Google Scholar 

  27. Gomi H, Karasu K, Nishimaru I, Iikawa T, Kazama S: Case of surgery on Wolff-ParkinsonWhite syndrome. Kyobu. Geka. 27: 129, 1974. Mantakas ME, McCue CM, Miller WW: Natural history of Wolff-Parkinson-White syndrome discovered in infancy. Am. J. Cardiol. 41:1097, 1978.

    Google Scholar 

  28. Symons JC, Shinebourne EA, Joseph MC, Lincoln C, Ho Y, Anderson RH: Criss-cross heart with congenitally corrected transposition: report of a case with d-transposed aorta and ventricular preexcitation. Eur. J. Cardiol. 5: 493, 1977.

    PubMed  CAS  Google Scholar 

  29. Benson DW Jr, Gallagher JJ, Oldham HN, Sealy WC, Sterba R, Spach MS: Corrected transposition with severe intracardiac deformities with Wolff-Parkinson-White syndrome in a child: electrophysiologic investigation and surgical correction. Circulation 61: 1256, 1980.

    PubMed  Google Scholar 

  30. Dick M II, Behrendt DM, Byrum CJ, Sealy WC, Stern AM, Hees P, Rosenthal A: Tricuspid atre-sia and the Wolff-Parkinson-White syndrome: evaluation methodology and successful surgical treatment of the combined disorders. Am. Heart J. 101: 496, 1981.

    Article  PubMed  Google Scholar 

  31. Vanetti A, Donzeau-Gouge GP, Frank R, Fourati M, Evans J, Ismail MB, Daumet P: Surgical treatment for a complicated congenital aortic stenosis: the association of a three-level obstruction of the left ventricular outflow tract with mitral incompetence, coarctation of the aorta, and the Wolff-Parkinson-White syndrome. J. Thorac. Cardiovasc. Surg. 77: 230, 1979.

    PubMed  CAS  Google Scholar 

  32. Wellens HJJ, Gorgels AP: Mirror image dextrocardia and the Wolff-Parkinson-White syndrome. Chest 76: 91, 1979.

    Article  PubMed  CAS  Google Scholar 

  33. Hiejima K, Tsuchiya S, Sakamoto Y, Shiina S: Two cases of Marfan’s syndrome associated respectively with subacute bacterial endocarditis and the Wolff-Parkinson-White syndrome. Jpn. Heart J. 9: 208, 1968.

    Google Scholar 

  34. Schiebler GL, Adams P Jr, Anderson RC, Amplatz K, Lester RG: Clinical study of twenty-three cases of Ebstein’s anomaly of the tricuspid valve. Circulation 19: 165, 1959.

    PubMed  CAS  Google Scholar 

  35. Soulié P, Heulin A, Pauly-Laubry C, Degeorges

    Google Scholar 

  36. M: Maladie d’Ebstein etude clinique et évolution (a propos de 40 observations dont 9 chirurgicales). Arch. Mal Coeur 63: 615, 1970.

    Google Scholar 

  37. 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. 54: 163, 1979.

    PubMed  CAS  Google Scholar 

  38. Lev M, Gibson S, Miller RA: Ebstein’s disease with Wolff-Parkinson-White syndrome: report of a case with a histopathologic study of possible conduction pathways. Am. Heart J. 49: 724, 1955.

    Article  PubMed  CAS  Google Scholar 

  39. Friedman S, Wells CRE, Amiri S: The transient nature of Wolff-Parkinson-White anomaly in childhood. J. Pediatr. 74: 296, 1969.

    Article  PubMed  CAS  Google Scholar 

  40. Klein GJ, Gulamhusein SS: Intermittent preexcitation in the Wolff-Parkinson-White syndrome. Am. J. Cardiol. 52: 292, 1983.

    Article  PubMed  CAS  Google Scholar 

  41. Follath F, Hallidie-Smith KA: Unusual electrocardiographic changes in Ebstein’s anomaly. Br. Heart J. 34: 513, 1972.

    Article  PubMed  CAS  Google Scholar 

  42. Gallagher JJ, Smith WM, Kasell JH, Benson DW Jr, Sterba R, Grant AO: Role of Mahaim fibers in cardiac arrhythmias in man. Circulation 64: 176, 1981.

    Article  PubMed  CAS  Google Scholar 

  43. Holmes DR Jr, Osborn MJ, Gersh B, Maloney JD, Danielson GK: The Wolff-Parkinson-White syndrome: a surgical approach. Mayo Clin. Proc. 57: 345, 1982.

    PubMed  Google Scholar 

  44. Smith WM, Gallagher JJ, Kerr CR, Sealy WC, Kasell JH, Benson DW Jr, Reiter MJ, Sterba R, Grant AO: The electrophysiologic basis and management of symptomatic recurrent tachycardia in patients with Ebstein’s anomaly of the tricuspid valve. Am. J. Cardiol. 49: 1223, 1982.

    Article  PubMed  CAS  Google Scholar 

  45. Tonkin AM, Dugan FA, Svenson RH, Sealy WC, Wallace AG, Gallagher JJ: Coexistence of functional Kent and Mahaim-type tracts in the pre-excitation syndrome: demonstration by catheter techniques and epicardial mapping. Circulation 52: 193, 1975.

    PubMed  CAS  Google Scholar 

  46. Castellanos A, Agha AS, Befeler B, Myerburg RJ: Double accessory pathways in WolffParkinson-White syndrome. Circulation 51: 1020, 1975.

    PubMed  CAS  Google Scholar 

  47. Edvardsson N, Blomström P, Olsson SB: Identification of multiple pathways in the WolffParkinson-White syndrome: diagnostic problems and therapeutic implications. Cardiology 71: 20, 1984.

    Article  PubMed  CAS  Google Scholar 

  48. Inoue H, Matsuo H, Takayanagi K, Ishimitsu T, Murao S: Antidromic reciprocating tachycardia via a slow Kent bundle in Ebstein’s anomaly. Am. Heart J. 106: 147, 1983.

    Article  PubMed  CAS  Google Scholar 

  49. Smith WM, Broughton A, Reiter MJ, Benson DW Jr, Grant AO, Gallagher JJ: Bystander accessory pathway during AV node re-entrant tachycardia. PACE 6: 537, 1983.

    Article  PubMed  CAS  Google Scholar 

  50. Klein GJ, Bashore TM, Sellers TD, Pritchett ELC, Smith WM, Gallagher JJ: Ventricular fibrillation in the Wolff-Parkinson-White syndrome. N. Engl. J. Med. 301: 1080, 1979.

    Article  PubMed  CAS  Google Scholar 

  51. Mehta AV, Pickoff AS, Raptoulis A, Wolff GS, Garcia OL, Tamer DF, Ferrer PL, Gelband H: Atrial flutter and atrial fibrillation associated with Wolff-Parkinson-White syndrome in childhood. Pediatr. Cardiol. 1: 197, 1980.

    Article  Google Scholar 

  52. Kugler JD, Gillette PC, Duff DF, Cooley DA, McNamara DG: Elective mapping and surgical division of the bundle of Kent in a patient with Ebstein’s anomaly who required tricuspid valve replacement. Am. J. Cardiol. 41: 602, 1978.

    Article  PubMed  CAS  Google Scholar 

  53. Durrer D, Roos JP: Epicardial excitation of the ventricles in a patient with Wolff-ParkinsonWhite syndrome (type B). Circulation 35: 15, 1967.

    PubMed  CAS  Google Scholar 

  54. Burchell HB, Frye RL, Anderson MW, McGoon DC: Atrioventricular and ventriculoatrial excitation in Wolff-Parkinson-White syndrome (type B): temporary ablation at surgery. Circulation 36: 663, 1967.

    PubMed  CAS  Google Scholar 

  55. Danielson GK, Fuster V: Surgical repair of Ebstein’s anomaly. Ann. Surg. 196: 499, 1982.

    Article  PubMed  CAS  Google Scholar 

  56. Westaby S, Karp RB, Kirklin JW, Waldo AL, Blackstone EH: Surgical treatment in Ebstein’s malformation. Ann. Thorac. Surg. 34: 388, 1982.

    Article  PubMed  CAS  Google Scholar 

  57. Sealy WC, Gallagher JJ, Pritchett ELC, Wallace AG: Surgical treatment of tachyarrhythmias in patients with both an Ebstein anomaly and a Kent bundle. J. Thorac. Cardiovasc. Surg. 75: 847, 1978.

    PubMed  CAS  Google Scholar 

  58. Sealy WC, Gallagher JJ, Pritchett ELC: The surgical anatomy of Kent bundles based on electrophysiological mapping and surgical exploration. J. Thorac. Cardiovasc. Surg. 76: 804, 1978.

    PubMed  CAS  Google Scholar 

  59. Iwa T, Magara T, Watanabe Y, Kawasuji M, Misaki T: Interruption of multiple accessory conduction pathways in the Wolff-ParkinsonWhite syndrome. Ann. Thorac. Surg. 30: 313, 1980.

    Article  PubMed  CAS  Google Scholar 

  60. Devereux RB, Perloff JK, Reichek N, Josephson ME: Mitral valve prolapse. Circulation 54: 3, 1976.

    PubMed  CAS  Google Scholar 

  61. Procacci PM, Savran SV, Schreiter SL, Bryson AL: Prevalence of clinical mitral-valve prolapse in 1169 young women. N. Engl. J. Med. 294: 1086, 1976.

    Article  PubMed  CAS  Google Scholar 

  62. Josephson Me, Horowitz LN, Kastor JA: Paroxysmal supraventricular tachycardia in patients with mitral valve prolapse. Circulation 57: 111, 1978.

    Google Scholar 

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Authors
  1. Co-burn J. Porter
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  2. David R. Holmes Jr.
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Editor information

Editors and Affiliations

  1. Section of Cardiology, University of Minnesota Medical School, Mayo Memorial Building, Box 341, 55455, Minneapolis, Minnesota, USA

    David G. Benditt M.D. (Associate Professor of Medicine) (Associate Professor of Medicine)

  2. Pediatric Cardiology, University of Minnesota Medical School, Mayo Memorial Building, Box 94, 55455, Minneapolis, Minnesota, USA

    D. Woodrow Benson Jr. M.D., Ph.D. (Professor of Pediatrics) (Professor of Pediatrics)

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© 1986 Martinus Nijhoff Publishing

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Porter, Cb.J., Holmes, D.R. (1986). Preexcitation Syndromes Associated with Congenital Heart Disease. In: Benditt, D.G., Benson, D.W. (eds) Cardiac Preexcitation Syndromes. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-7526-5_14

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  • DOI: https://doi.org/10.1007/978-1-4684-7526-5_14

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-7528-9

  • Online ISBN: 978-1-4684-7526-5

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