Arquivos Brasileiros de Cardiologia

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Volume 110, Nº 6, June 2018




Behavior of Blood Pressure Variables in Children and Adolescents with Duchenne Muscular Dystrophy

Fabiane R. R. H. Marui

Henrique Tria Bianco

Maria Teresa N. Bombig

Natascha G. F. Palmeira

José M. Thalenberg

Fernando Focaccia Povoa

Maria Cristina de O. Izar

Francisco Antonio H. Fonseca

Acary S. B. de Oliveira

Rui M. S. Povoa


Background: Duchenne muscular dystrophy is an X-chromosome-linked genetic disorder (locus Xp21). Involvement of the cardiovascular system is characterized by fibrous degeneration/replacement of myocytes with consequent ventricular hypertrophy and arterial hypertension.

Objective: To assess, by using 24-hour ambulatory blood pressure monitoring, the behavior of blood pressure variables in children and adolescents with a confirmed diagnosis of Duchenne muscular dystrophy.

Methods: Prospective observational cohort study, which selected 46 patients followed up on an outpatient basis, divided according to age groups. Blood pressure was classified according to the age percentile. The monitoring interpretation includes systolic and diastolic blood pressure means, systolic and diastolic blood pressure loads, and nocturnal dipping. The blood pressure means were calculated for the 24-hour, wakefulness and sleep periods. Nocturnal dipping was defined as a drop in blood pressure means during sleep greater than 10%. The significance level adopted was p < 0.05.

Results: Nocturnal dipping for systolic blood pressure was present in 29.9% of the participants. Approximately 53% of them had attenuated nocturnal dipping, and 15%, reverse nocturnal dipping. The age groups of 9-11 years and 6-8 years had the greatest percentage of attenuation, 19.1% and 14.9%, respectively. Regarding diastolic blood pressure, nocturnal dipping was identified in 53.2% of the children, being extreme in 27.7% of those in the age group of 6-11 years.

Conclusions: The early diagnosis of blood pressure changes can allow the appropriate and specific therapy, aimed at increasing the life expectancy of patients with Duchenne muscular dystrophy. (Arq Bras Cardiol. 2018; 110(6):551-557)

Keywords: Cardiovascular Diseases / genetics; Muscular Dystrophy, Duchenne / genetics; Hypertension; Child; Male.