Implantable Cardioverter Defibrillators in Adults with Congenital Heart Disease:

Sudden death is the most frequent cause of late mortality in adults with congenital heart disease. This study reviews the experience of an Adult Congenital Heart Center with the use of implantable cardioverter defibrillators (ICDs). The charts of adults with congenital heart disease who had an ICD implantation were reviewed for diagnosis, residual lesions, reoperations, reason for implantation, complications, and recurrence of arrhythmias. Since 1995, 13 patients (mean age 43 years) had an ICD implantation for aborted sudden death (4), and spontaneous (6), or induced (3) ventricular tachycardia. Diagnosis were repaired (6) or palliated (1) tetralogy of Fallot, operated pulmonary stenosis (2), palliated complex pulmonary atresia (1), congenitally corrected transposition (1), operated ventricular (1), and atrial (1) septal defects. Significant residual lesions included severe pulmonary regurgitation (2), systemic ventricular dysfunction (2), and severe pulmonary hypertension (1). Five patients had a QRS > 180 ms, four had a QRS < 180 ms, four had a paced rhythm. Overall, four (31%) patients had no ventricular dysfunction, no residual lesion, and QRS < 180 ms. During a mean follow-up of 29 months, seven patients had recurrent ventricular tachycardia, three with normal ventricular function, no residual lesion, and QRS < 180 ms. ICD implantation is an important adjunct in the management of adults with congenital heart disease. As malignant arrhythmias occur even in patients with no residual lesion, no QRS prolongation and no ventricular dysfunction, the recognition of those who would benefit from an ICD remains a clinical challenge.