Chapter 66 - Ventricular Tachycardia in Patients Following Surgery for Congenital Heart Disease

Abstract

Ventricular tachycardia, and the possible relation of ventricular tachycardia to sudden death, remains one of the most difficult management issues in patients who have previously undergone surgical repair of significant congenital heart defects. The occurrence of sudden death in this patient population is even more devastating when one considers that for the most part, the presenting life-threatening hemodynamic abnormalities in these patients have been successfully managed by surgery, often years or decades before the occurrence of a fatal arrhythmic event. Initially, attention had been focused on the possibility that the development of complete atrioventricular (AV) block might be a possible explanation for sudden death in such patients. However, although AV block is certainly the explanation in a few patients, it has not been implicated in most instances of sudden death. In patients who have undergone major atrial surgery, such as the Mustard or Senning repair for transposition of the great vessels or the Fontan procedure for various forms of single ventricle, atrial flutter with rapid conduction is certainly involved in the etiology of sudden death. 1 2 However, because of the frequent occurrence of premature ventricular complexes, nonsustained and sustained ventricular tachycardia in patients who have undergone complete repair of tetralogy of Fallot and related defects such as double outlet right ventricle, ventricular tachycardia has been implicated in the etiology of sudden death in this patient group. Indeed, it has been reported that postoperative tetralogy of Fallot is the single most common condition seen in children between the ages of 1 and 16 years who have experienced sudden death, 3 although the risk for sudden death is also increased in aortic stenosis, coarctation, and transposition. 4