Chest
Volume 113, Issue 5, May 1998, Pages 1415-1417
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Selected Reports
Cardiomyopathic Lentiginosis/LEOPARD Syndrome Presenting as Sudden Cardiac Arrest

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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.

Section snippets

CASE REPORT

An apparently healthy 26-year-old man was admitted to the ICU after successful resuscitation from documented ventricular fibrillation sustained during dancing the polka. A comprehensive past medical history could be obtained from his mother: “moles” had erupted during childhood; a dermatologist had diagnosed nevi and had recommended surveillance. In 1980, an operation was performed for an undescended testicle; an aneurysm of the left femoral vein was excised in 1988. In 1991, ultrasound studies

COMMENT

Sudden ventricular fibrillation is uncommon in young adults. Unlike in the elderly, coronary heart disease is rarely the cause. Instead, myocarditis, cardiomyopathy, right ventricular dysplasia, use of illicit drugs, and anomalies of the QT interval prevail. Hypertrophic cardiomyopathy is a key feature of cardiomyopathic lentiginosis.6 Interestingly, sudden death has been mentioned in previous reports of the disorder.7

In this case, cardiomyopathy was revealed as the most likely cause and

CONCLUSION

Lentiginosis must prompt thorough evaluation since it may be part of a multifaceted syndrome that cannot only be associated with considerable morbidity but may even place patients at risk for sudden death. Although identification of patients in need of prophylactic treatment will remain difficult, pacer-cardioverter-defibrillator device therapy is believed to be indicated and beneficial in survivors of out-of-hospital ventricular fibrillation.

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