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The Management of Cardiovascular Abnormalities in Patient With LEOPARD Syndrome

LEOPARD syndrome (LS) is a rare hereditary disorder in Asian countries. This syndrome consists of multiple systemic abnormalities. In particular, characteristic cardiovascular effects in LS may include variable clinical manifestations from benign to life-threatening courses. The cardiac effects of t...

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Detalles Bibliográficos
Autores principales: Lee, Ki-Jun, Choi, Hyunmin, Choi, Won Ho, Kwon, Sung Uk, Doh, Joon Hyung, Namgung, June, Lee, Sung Yun, Lee, Won Ro
Formato: Texto
Lenguaje:English
Publicado: The Korean Society of Cardiology 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2910291/
https://www.ncbi.nlm.nih.gov/pubmed/20664743
http://dx.doi.org/10.4070/kcj.2010.40.7.339
Descripción
Sumario:LEOPARD syndrome (LS) is a rare hereditary disorder in Asian countries. This syndrome consists of multiple systemic abnormalities. In particular, characteristic cardiovascular effects in LS may include variable clinical manifestations from benign to life-threatening courses. The cardiac effects of this syndrome consist of left ventricular hypertrophy (LVH), pulmonary stenosis (PS), coronary artery dilatation and electrocardiogram(ECG) abnormalities. Since there are few LS patients who have undergone a complete cardiovascular evaluation, the nature and clinical prognosis of cardiovascular abnormalities in this syndrome remain uncertain. Also, there have been few reports on therapeutic strategies for cardiovascular abnormalities in LS. Here we describe a case of LS who presented with multiple cardiovascular problems and underwent successful surgical and medical treatment.