Abstract 16256: Lesion-Specific Prevalence of Non-Compaction in Association with Congenital Heart Disease
Aims : Abnormal ventricular compaction increases cardiovascular mortality due to risks of arrhythmia, cardiomyopathy and systemic thromboembolism. Due to increased detection by cardiac MRI in our cohort of patients with congenital heart disease (CHD), we sought to define the relative lesion specific prevalence of compaction abnormalities and its effect on cardiac hemodynamics. Methods:We conducted a retrospective review of 326 consecutive cardiac MRI scans of patients with complex adult congenital heart disease performed from 2008-2012. Non-compaction (NC) was defined by the ratioof non-compacted to compacted myocardium >2.3 in end diastole. Cardiac MRIs of children with Tetralogy of Fallot (TOF) were also analyzed to assess the effect of age on the prevalence of NC.
Results : The highest prevalence of NC (77%) was in patients with univentricular physiology (20/26 patients; 9 with morphological left ventricle); followed by repaired transposition of the great arteries (TGA) (17/38; 16 with a systemic right ventricle) and TOF with a prevalence of 22% (25/123). The lowest prevalence was seen with congenital pulmonary stenosis (6%). In patients with univentricular physiology, NC did not correlate with ejection fraction (Mean EF =47+/-15%; p=0.27). A morphologic right ventricle as systemic ventricle was significantly associated with NC (p=0.02). NC in TGA patients correlated with age, (Median age 20; (IQR 15-30) p<0.001), systemic right ventricle (p<0.01) and was significantly associated with a lower EF (NC EF 55+/-11; compacted EF 64+/-7; p=0.01). In patients with TOF, there was no correlation of NC with age, ventricular volumes or ejection fraction.
Conclusions : Abnormal compaction is more prevalent in association with particular CHDs, up to 77% in “single ventricle” hearts. This may influence ventricular performance and warrants further investigation.
- © 2012 by American Heart Association, Inc.