Clinical features of isolated ventricular noncompaction in adults long-term clinical course, echocardiographic properties, and predictors of left ventricular failure.
BACKGROUND
Isolated ventricular non-compaction (IVNC) is a rare disorder characterized by prominent trabecular meshwork and deep recesses. We retrospectively assessed the clinical characteristics and natural course of IVNC in adults diagnosed at our hospital.


RESULTS
Sixty-seven adult patients (44 male, mean age 41 +/- 18 years) with the diagnosis of IVNC were evaluated in this retrospective cohort. Its prevalence was found to be .14%. Forty-seven patients (70%) had class I/II functional capacity. Fifty-seven patients (85%) had electrocardiographic abnormalities, and the most common one was left ventricular (LV) hypertrophy (25%). LV systolic function was depressed in 44 patients (66%), with a median ejection fraction (EF) of 35% (range: 20%-48%) at diagnosis. Multiple regression analysis revealed that age at initial presentation, the total number of affected segments, and the ratio of non-compaction/compaction (NC/C) were the independent predictors of LV systolic dysfunction. Familial occurrence of IVNC was 33%. During a mean follow-up of 30 months (range: 9-50 months), major complications including ventricular tachycardia, heart failure requiring hospitalization, and cerebrovascular events were observed in 36%, 34%, and 9% of the patients, respectively. Ten patients (15%) with IVNC died in this study. LVEF at initial presentation and functional capacity at last visit were found to be independent predictors of mortality.


CONCLUSIONS
This study suggests that IVNC is a form of cardiomyopathy with higher prevalence and relatively better prognosis than previously reported. Age at initial presentation, ratio of NC/C, and number of affected segments seem to be major determinants of LV systolic dysfunction, while initial LVEF and last functional capacity predict mortality in this cohort.
Aras
Dursun
D
Turkiye Yuksek Ihtisas Hospital, Department of Cardiology, Ankara, Turkey.
Tufekcioglu
Omac
O
Ergun
Kumral
K
Ozeke
Ozcan
O
Yildiz
Ali
A
Topaloglu
Serkan
S
Deveci
Bulent
B
Sahin
Onur
O
Kisacik
Halil Lutfi
HL
Korkmaz
Sule
S
D000328:Adult; D006328:Cardiac Catheterization; D009202:Cardiomyopathies / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology*; D015331:Cohort Studies; D004452:Echocardiography; D004562:Electrocardiography; D015716:Electrocardiography, Ambulatory; D005260:Female; D006333:Heart Failure / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D015995:Prevalence; D012189:Retrospective Studies; D018487:Ventricular Dysfunction, Left / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology