Abstract 14427: Long-Term Mortality and Risk Factors for Sudden Arrhythmic Death in Adults With Congenital Heart Disease Repaired Before 20 Years Old.
Background: Long-term mortality (M) of congenital heart patients (CHD) has previously been assessed in population based-studies and multicenter registries which did not discern either infant from adult M or operated from non-repaired CHD.
Methods: Since January 1990, 3,197 CHD have been evaluated at our adult CHD unit. Surgical or percutaneous intervention < 20 years of age had been performed in 1,289 (40%). Follow up was complete in 936 patients (73%) of this cohort. We sought to determine the overall M, the modes of death and risk factors for overall and sudden arrhythmic (proven or presumed) death (SD).
Results: Mean age at last examination was 30 ± 8 years and 478 (51%) were males. During a follow-up of 8,387 patients-years, 50 (6/1,000 pt-yrs) died: 15/99 with transposition of the great arteries (16/1,000 pt-yrs); 8/55 with single ventricle (15/1,000 pt-yrs); 10/216 with tetralogy of Fallot (5/1,000 pt-yrs); 10/242 with aortic stenosis/coarctation (4/1,000 pt-yrs); and 7/324 with other CHD (3/1,000 pt-yrs). Mode of death was SD in 21 (42%), cardiac surgery in 10 (20%), heart failure in 8 (16%), other cardiovascular death in 5 (10%), non cardiac cause in 2 (4%) and 4 patients underwent transplantation (8%). Univariate analysis showed that neither gender, age, age at repair and pulmonary or tricuspid valve dysfunction were related to overall M or SD. Multivariate analysis showed that severe ventricular (systemic or subpulmonary) dysfunction (p<0.001 in both) and pulmonary hypertension (p=0.001) were independent predictors of overall M whereas subaortic (right, left or single) ventricular dysfunction was the only independent predictor of SD (relative risk 48; 95% confidence interval 17-132; p<0.001). SD occurred in 27% (15 out of the 56) of patients with severe subaortic ventricular dysfunction compared with 0.7% (6 out of the 851) in patients without it (p<0.001). Previously documented atrial or ventricular arrhythmias and functional class were not independently related with SD.
Conclusions: Severe subaortic ventricular dysfunction is the main predictor of sudden arrhyihmic death in adults patients with congenital heart disease repaired in infancy or childhood and provides a rational basis for considering implantable cardioverter defibrillators.
- © 2010 by American Heart Association, Inc.