Resumo

SERRANO RICARDO, Giselle; MARCANO SANZ, Luis Enrique  e  BACALLAO CARRIL, Dayamí. Cardiovascular considerations about Marfan's syndrome at pediatric ages. Rev Cubana Pediatr [online]. 2012, vol.84, n.2, pp. 176-187. ISSN 0034-7531.

Marfan syndrome is a hereditary disease of the connective tissue caused by mutation of type 1 fibrillin glycoprotein-coding gene in children and adults. This disease affects organs and systems, mainly cardiovascular, skeletal, ophthalmologic systems, skin and teguments. The review of the most current aspects of diagnosis, and the multidisciplinary care to reduce morbidity and mortality of pediatric patients were presented. It was concluded that the early use of betablockers and angiotensin II AT-1 receptor blocker (losarfan) are the fundamental pillars of drug therapy, since they reduce the frequency of cardiovascular complications that determine the disease prognosis. The scheduled surgery of the aortic root, particularly valve preservation, allows improving the life expectancies because it prevents high mortality from acute events. Hybrid procedures and endovascular interventions are promising alternatives for the management of the disease.

Palavras-chave : Marfan syndrome; genetic diseases; pediatrics.

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