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

Abstract

BackgroundDuchenne 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.ObjectiveTo 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.MethodsProspective 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.ResultsNocturnal 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.ConclusionsThe 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.

Social stress is lethal in the mdx model of Duchenne muscular dystrophy

“…Recent clinical studies have called attention to a critical role for low blood pressure in DMD [47À50]. Specifically, low blood pressure in an ambulatory setting emerged as a distinctive feature of DMD patients [47,49,50]. In a retrospective study of 43 DMD patients [48], Cheeran and coworkers identified low systolic pressure as one of the prognostic factors that predicts a worse outcome and death.…”

Hemodynamic Consideration in Intraoperative Neurophysiological Monitoring in Neuromuscular Scoliosis Surgery

“…Previously, hemodynamic instability in neuromuscular diseases was observed. BP was lower in patients with Duchenne muscular dystrophy (DMD), a representative neuromuscular disease, than in healthy patients, and this was thought to be due to decreased cardiac function [ 28 , 29 ]. In addition, it is reported that an increase in prothrombin time and depletion of factor VII is more common in NMS than AIS, which can lead to an increase in the bleeding amount [ 30 ].…”

Acute hemodynamics of cardiac sympathetic denervation

“…In a patient population of 29 patients they have demonstrated a significant drop in systolic BP, diastolic BP and HR both at rest and on exercise after a period of one month after the surgery. There are also a few older animal and human studies analysing the hemodynamic response of unilateral and bilateral upper thoracic sympathetic denervation [ [32] , [33] , [34] , [35] , [36] , [37] ]. They demonstrated that right and left thoracic sympathetic denervation may produce opposite effects on cardiac arrhythmias in experimental animals and in humans, with left sided sympathectomy producing more drop in BP, shortening of QTc and decrease in HR.…”