Noncompaction cardiomyopathy epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
This is a rare genetic disorder. It is hard to know the true prevalance of disease as the diagnosis may be missed on echocardiography, and studies of prevalance focus on patients referred to teriary referral centers with congestive heart failure. The prevalence may increase as imaging modalities improve. In the largest study to date, the prevalence was 0.014%[1].
Prevalence in Children
The incidence is estimated to be 0.12 cases per 100,000 children [2].
Prevalence in Infants
Others have estimated the rate to be as high as 0.81 per 100,000 infants per calender year.
Male Predominance
There is an excess incidence of the disease in males (56% to 82%). This has been taken as evidence that in some cases there may be x-linked inheritance.
Age
In the initial description of the disease, the median age at diagnosis was 7 with a range from 11 months to 22 years[3]. Other studies have confirmed the presence of NCC in all age groups including the elderly.
References
- ↑ Oechslin EN, Attenhofer Jost CH, Rojas JR, 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; 36: 493–500.
- ↑ Botto, LD. Left ventricular noncompaction. Orphanet encyclopedia. http://www.orpha.net/data/patho/GB/uk-LVNC.pdf
- ↑ Chin TK, Perloff JK, Williams RG, et al. Isolated noncompaction of left ventricular myocardium: a study of eight cases. Circulation. 1990; 82: 507–513.