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(Chest. 1998;113:1415-1417.)
© 1998 American College of Chest Physicians

Cardiomyopathic Lentiginosis/LEOPARD Syndrome

Presenting as Sudden Cardiac Arrest

Alexander Woywodt 1; Julia Welzel MD2; Henning Haase MD1; Anke Duerholz MD1; Uwe Wiegand MD1; Juergen Potratz MD1; and Abdolhamid Sheikhzadeh MD1

1 From the Department of Cardiology, University of Luebeck School of Medicine, Luebeck, Germany
2 From the Department of Dermatology, University of Luebeck School of Medicine, Luebeck, Germany

A 26-year-old apparently healthy man with numerous pigmented skin lesions collapsed during an evening party and was resuscitated from ventricular fibrillation. Hypertrophic cardiomyopathy and subaortic tunnel were disclosed by angiocardiography. A diagnosis of cardiomyopathic lentiginosis/lentigines (multiple), electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of the genitalia, retardation of growth, and deafness (sensorineural) syndrome was made. The patient then underwent treatment with an implantable pacer-cardioverter-defibrillator device. Further evaluation revealed several well-established features of the disorder. This is the first reported case of survival from ventricular fibrillation associated with this rare and little known multifaceted syndrome. Disseminated lentiginosis must prompt clinicians to evaluate such cases further since underlying disorders may be associated with considerable morbidity and, apparently, sudden death.

Key Words: cardiac arrest • cardiomyopathic lentiginosis • hypertrophic nonobstructive cardiomyopathy • implantable pacer-cardioverter-defibrillator • lentiginosis • LEOPARD syndrome • subaortic tunnel

Submitted on March 11, 1997
Accepted on September 17, 1997




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A J Bauer and C A Stratakis
The lentiginoses: cutaneous markers of systemic disease and a window to new aspects of tumourigenesis
J. Med. Genet., November 1, 2005; 42(11): 801 - 810.
[Abstract] [Full Text] [PDF]


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