Korean J Med.  2003 Jul;65(1):99-103.

A case of LEOPARD syndrome with cor triatriatum

Affiliations
  • 1Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea. yookd@catholic.ac.kr

Abstract

The LEOPARD syndrome is an acronym and serves as a mnemonic for the features of this autosomal dominant syndrome : L - lentigines (multiple), E - electrocardiographic conduction abnormalities, O - ocular hypertelorism, P - pulmonary stenosis, A - abnormalities of genitalia, R - retardation of growth, and D - deafness (sensoryneural). The main features of the syndrome are multiple lentigines in combination with congenital heart malformations. These frequently accompanied cardiac abnormalities are pulmonary stenosis, hypertrophic cardiomyopathy, and various ECG abnormalities. It is advisable to make cardiac evaluation in a patient with LEOPARD syndrome in spite of no clinical symptoms or signs, since cardiac dysfunction may be progressive or developed later. We experienced a case of this syndrome in a 31 year-old female, presenting multiple lentigines, ocular hypertelorism, and congenital cardiac abnormalities of incomplete right bundle branch block and cor triatriatum. We report the case with brief literature review.

Keyword

LEOPARD syndrome; Lentigo; Cor triatriatum

MeSH Terms

Adult
Bundle-Branch Block
Cardiomyopathy, Hypertrophic
Cor Triatriatum*
Deafness
Electrocardiography
Female
Genitalia
Heart
Humans
Hypertelorism
Lentigo
LEOPARD Syndrome*
Panthera*
Pulmonary Valve Stenosis
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