Cardiomyopathy, Dilated, with Hypergonadotropic Hypogonadism

Alternative Names

  • Malouf Syndrome
  • Cardiomyopathy, Congestive, with Hypergonadotropic Hypogonadism
  • Cardiomyopathy, Dilated, with Premature Ovarian Failure
  • Cardiomyopathy with Primary Testicular Failure
  • Najjar Syndrome
  • Genital Anomaly with Cardiomyopathy
  • Cardiogenital Syndrome
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WHO-ICD-10 version:2010

Diseases of the circulatory system

Other forms of heart disease

OMIM Number

212112

Mode of Inheritance

Autosomal dominant

Gene Map Locus

1q22

Description

This syndrome is characterized by the association of dilated cardiomyopathy and hypergonadotropic hypogonadism (DCM-HH). [From Orphanet]

Epidemiology in the Arab World

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Other Reports

Lebanon

Najjar et al (1973) were the first to report a syndrome of incompletely developed external genitals, mental retardation, and an ill-defined cardiomyopathy in three brothers born to second cousin Lebanese parents. Later, Najjar et al (1984) described another pair of siblings with a more severe phenotype, with a more severe genital anomaly and an earlier onset of cardiomyopathy. Intellectual disability was not found in this family. 

Malouf et al (1985) described two lebanese sisters, born to first cousin consanguineous parents, who presneted with primary hypergonadotropic hypogonadism and congestive cardiomyopathy. 

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