Clinical features of isolated ventricular noncompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure.

摘要: Abstract Background Isolated ventricular non-compaction (IVNC) is a rare disorder characterized by prominent trabecular meshwork and deep recesses. We retrospectively assessed the clinical characteristics natural course of IVNC in adults diagnosed at our hospital. Methods Results Sixty-seven adult patients (44 male, mean age 41 ± 18 years) with diagnosis were evaluated this retrospective cohort. Its prevalence was found to be .14%. Forty-seven (70%) had class I/II functional capacity. Fifty-seven (85%) electrocardiographic abnormalities, most common one left (LV) hypertrophy (25%). LV systolic function depressed 44 (66%), median ejection fraction (EF) 35% (range: 20%–48%) diagnosis. Multiple regression analysis revealed that initial presentation, total number affected segments, ratio non-compaction/compaction (NC/C) independent predictors dysfunction. Familial occurrence 33%. During follow-up 30 months 9–50 months), major complications including tachycardia, heart failure requiring hospitalization, cerebrovascular events observed 36%, 34%, 9% patients, respectively. Ten (15%) died study. LVEF presentation capacity last visit mortality. Conclusion This study suggests form cardiomyopathy higher relatively better prognosis than previously reported. Age NC/C, segments seem determinants dysfunction, while predict mortality