Inheritance of the mitral valve prolapse syndrome. Discussion of a three-dimensional penetrance model

Nancy V. Strahan, Edmond A. Murphy, Nicholas J. Fortuin, Patricia C. Come, J. O Neal Humphries

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The families of 12 probands with classic mural valve prolapse were studied for evidence of mitral valve prolapse. Seventy parents, sibs, and progeny were included in the analysis. Forty-seven percent (16 of 34) of progeny were affected compared with 30 percent (3 of 10) of parents. Thirty-eight percent (10 of 26) of sibs were affected. A three-compartmental penetrance model was devised to account for the variation in expression with age. This includes a latent stage (time before onset of signs), an affected stage, and a stage in which the subjects are withdrawn (because of treatment, regression, or death). The implications of this model are discussed.

Original languageEnglish (US)
Pages (from-to)967-972
Number of pages6
JournalAmerican Journal of Medicine
Issue number6
StatePublished - 1983
Externally publishedYes


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