De Rubens FJ, García CA, González RC, Lieberman HE, Díaz GL

Language: Spanish
References: 22
Page: 286-293
PDF size: 373.58 Kb.

ABSTRACT

Objective: To describe our experience in the cardiovascular characteristics and clinical evolution of patients with MS, ED and LD.
Materials and Methods: Patient records from the last 10 years were reviewed in a cross-sectional, observational and retrospective study. The diagnosis was made with family history and Ghent clinical criteria. Cardiac characteristics, treatment and clinical criteria. Cardiac characteristics, treatment and clinical behavior were analyzed. Percentages and frequencies are made.
Results: Thirty patients with SM, 18 with ED and 3 with LD (51 patients) were studied. The male gender predominated and the age ranged between 1 and 17 years of age. Patients with SM had 50% of family history, with LD 66% and with ED 44%. Cardiovascular alterations occurred in patients with MS in 73%, the most frequent being dilation of the aortic root. 66.6% patients with LD had vascular disease. Treatment was based on losartan and propranolol. None were managed with cardiovascular surgery. Follow-up ranged from 6 months to 9 years. The majority had no cardiovascular
Conclusions: In patients with SM and LD, cardiovascular disease is common in children and adolescents. It is advisable to continue cardiovascular monitoring and early treatment.

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