Abstract 15193: Sudden Arrhythmic Death in a Contemporary Cohort of Adults with Congenital Heart Disease
Objectives Sudden cardiac death (SCD) is a major cause of mortality in adults with congenital heart disease (CHD). Earlier studies showed that patients with left-sided outflow lesions, tetralogy of Fallot (TOF) and (congenitally corrected) transposition of great arteries (cc) TGA were at increased risk for SCD. However, as this population ages, the population at risk and the risk factors associated with SCD have changed. Therefore, the aim of this study was to determine the adults with CHD at risk for SCD and the clinical parameters associated with SCD.
Methods This was a retrospective multicenter case-controlled study. Adults who died suddenly due to proven or presumed arrhythmia were included (cases). Presumed arrhythmia was defined as death due to cardiovascular causes within one hour of onset of the symptoms (or significant worsening) or unwitnessed death during sleep. For each case, two controls matched on diagnosis, type of surgical intervention, age and gender were included.
Results Overall, 217 (18%) adults died suddenly of which 171 (64% male, mean age 36±15) were due to proven or presumed arrhythmia. The main cardiac lesions included septal defects (23%, mean age 44±18 years (y)), TOF (22%, mean age 35±15 y), (cc) TGA, 19%, mean age 31±10 y), single ventricle physiology (15%, mean age 31±12 y) and left-sided obstructive heart defects (13%, mean age 37±16 y). Of the 171 patients, 131 (77%) experienced an out of hospital cardiac arrest. SCD during exercise was less common (10%). Most cases of SCD occurred at rest (69%) or during sleep (11%). Overall, independent predictors of SCD were supraventricular tachycardia (OR 3.9, 95% confidence interval (CI) 1.4-10.5, P=0.008), moderate to severe systemic ventricular dysfunction (OR 4.3, 95% CI 1.2-15.6, P=0.025), dilated systemic ventricle (OR 2.5, 95% CI 1.0-6.5 P=0.051), prolonged QRS duration (OR 1.03, 95% CI 1.00-1.05, P=0.006) and increased QT dispersion (OR 1.02, 95% CI 1.00-1.03, P=0.006).
Conclusion In this large cohort of adults with CHD, the majority of SCD occurred at rest. While patients with TOF, (cc) TGA and left-sided outflow lesions have previously been identified as at risk for SCD, this study identifies adults with septal defects, and single ventricle physiology as being at comparable risk.
- © 2011 by American Heart Association, Inc.