Elsevier

Journal of Cardiac Failure

Volume 12, Issue 9, December 2006, Pages 726-733
Journal of Cardiac Failure

Clinical Investigation
Clinical Features of Isolated Ventricular Noncompaction in Adults Long-Term Clinical Course, Echocardiographic Properties, and Predictors of Left Ventricular Failure

https://doi.org/10.1016/j.cardfail.2006.08.002Get rights and content

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 and natural course of IVNC in adults diagnosed at our hospital.

Methods and 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.

Conclusion

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.

Section snippets

Study Population and Data Acquisition

Medical charts of IVNC patients diagnosed between February 2001 and September 2005 were reviewed retrospectively to document clinical presentations including symptoms and New York Heart Association classification (NYHA), physical examination findings including neurological examination, primary diagnosis, positive family history, 12-lead resting electrocardiogram (ECG), presence of arrhythmia and cerebrovascular events, hospitalization for heart failure, need for operation or interventional

Demographics and Clinical Information

Fifty-seven adult cases of IVNC identified based on ∼49000 echocardiograms performed on just over 42000 patients during this study period, and 10 adult relatives of those index cases diagnosed by family screening, were included in this cohort. Family screening had been performed in 36 patients through which a total of 15 new cases (10 adult and 5 children) from 12 families were diagnosed with IVNC (familial occurrence was 33%). Five children diagnosed with family screening were excluded from

Discussion

Over the last decade, IVNC has been recognized as a distinct form of cardiomyopathy with a distinct underlying cause and prognosis.1, 5, 9 This study reports one of the largest series of adults with IVNC from a single institution and compares the data with those previously reported.

Conclusion

IVNC is a form of cardiomyopathy with highly varying clinical manifestations ranging from no symptoms to severe CHF. The present study showed a higher prevalence than previously reported ones; however, determination of the actual prevalence requires population-based studies. Although our patients showed better prognosis with lower mortality and morbidity, ultimate outcome of this rare disease remains unclear. Age, ratio of NC/C, and number of affected segments are determinants of LV systolic

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