Preexcitation Syndromes Associated with Congenital Heart Disease

  • Co-burn J. Porter
  • David R. HolmesJr.


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.


Congenital Heart Disease Tricuspid Valve Congenital Heart Defect Mitral Valve Prolapse Accessory Pathway 
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© Martinus Nijhoff Publishing 1986

Authors and Affiliations

  • Co-burn J. Porter
  • David R. HolmesJr.

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