Clinical InvestigationClinical Features of Isolated Ventricular Noncompaction in Adults Long-Term Clinical Course, Echocardiographic Properties, and Predictors of Left Ventricular Failure
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
References (25)
- et al.
Noncompaction of ventricular myocardium: a study of twelve patients
J Am Soc Echocardiogr
(2002) - et al.
Isolated noncompaction of the myocardium in adults
Mayo Clin Proc
(1997) - et al.
Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis
J Am Coll Cardiol
(2000) - et al.
Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background
J Am Coll Cardiol
(1999) - et al.
Isolated ventricular noncompaction is associated with coronary microcirculatory dysfunction
J Am Coll Cardiol
(2002) Clinical features of isolated left ventricular noncompaction in children
Int J Cardiol
(2004)- et al.
Determinants of outcome in isolated ventricular noncompaction in childhood
Am J Cardiol
(2004) - et al.
Echocardiographic assessment of left ventricular hypertrophy: comparison to necropsy findings
Am J Cardiol
(1986) - et al.
Paradoxical contributions of non-compacted and compacted segments to global left ventricular dysfunction in isolated left ventricular noncompaction
Am J Cardiol
(2006) - et al.
Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the definition and classification of cardiomyopathies
Circulation
(1996)
Isolated noncompaction of left ventricular myocardium. A study of eight cases
Circulation
Persisting myocardial sinusoids of both ventricles as an isolated anomaly: echocardiographic, angiographic and pathologic anatomical findings
Cardiovasc Intervent Radiol
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