Stanton, Christopher

Introduction Left ventricular non-compaction (LVNC) is a rare cardiomyopathy thought to result from intrauterine arrest of myocardial compaction which has been associated with and increased risk of sudden cardiac death (SCD). However, appropriate identification of at-risk individuals and risk-stratification is lacking, particularly in patients diagnosed during adulthood. We sought to identify high-risk patients wtih LVNC in order to guide therapy. Hypothesis That patients with decreased left ventricular ejection fraction (LVEF) would have worse outcomes than those with preserved left ventricular function. Methods We reviewed outcomes in all patients diagnosed with LVNC at Mayo Clinic between 2001 through 2006. Data were prospectively entered into a centralized database and retrospectively analyzed. Mortality was compared to age and sex matched population rates. Results A total of 80 patients were diagnosed with LVNC during this interval. After exclusion of pediatric patients (n = 21), 59 adult patients (62% male; age 46 ± 19 years, range 18–83) were analyzed. There were 4 deaths in this group (none due to stroke, mortality 18% at 2 years) during follow-up (mean 13.0 ± 13.5 months, range 0–52). Overall survival of patients with LVNC was worse than age and sex matched controls (Minnesota population p < 0.001). This was also noted in patients with LVEF > 35% (n = 35, p = 0.02). Overall LVEF was 41 ± 16% but was lower in patients who died compared to survivors (26 ± 10% vs. 42 ± 15%; Hazard ratio = 2.0 for a decrease in ejection fraction of 10%, p = 0.12). There were no deaths in patients with LVEF > 50% (n = 17). Conclusion Survival of adults with LVNC is worse than expected for age and sex. A decrease in ejection fraction is associated with increased risk of death in this population. These data suggest that patients diagnosed with LVNC and reduced LVEF may benefit from ICD implantation, whereas patients with a normal LVEF may be observed.