open access

Vol 23, No 1 (2016)
Original articles
Published online: 2015-10-21
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A marked decrease in heart rate variability in Marfan syndrome patients with confirmed FBN1 mutations

Andriy Cherkas, Rustam Zhuraev
DOI: 10.5603/CJ.a2015.0076
·
Pubmed: 26503076
·
Cardiol J 2016;23(1):23-33.

open access

Vol 23, No 1 (2016)
Original articles
Published online: 2015-10-21

Abstract

Background: The studies on heart rate variability (HRV), a key predictor of all-cause mortality, in Marfan syndrome (MS), up to now have not been reported, especially in patients with FBN1 mutations.

Methods: Among 18 MS patients with the phenotype of MS meeting inclusion criteria 15 have had a FBN1 gene mutation. Short electrocardiography records were taken in the supine position and during orthostatic tests. The control group consisted of 30 apparently healthy nonathletes matched by age and gender.

Results: Heart rates in MS patients with the FBN1 mutation were increased in both the supine position and orthostatic test (p < 0.001). Most of the time-domain (standard deviation, pNN50) and frequency-domain (total power, very low, low, and high frequency) parameters of HRV were significantly reduced in the MS patients (p < 0.001).

Conclusions: A marked decrease in HRV, documented in the study, may be an important clinical feature in MS patients with confirmed FBN1 gene mutations.  

Abstract

Background: The studies on heart rate variability (HRV), a key predictor of all-cause mortality, in Marfan syndrome (MS), up to now have not been reported, especially in patients with FBN1 mutations.

Methods: Among 18 MS patients with the phenotype of MS meeting inclusion criteria 15 have had a FBN1 gene mutation. Short electrocardiography records were taken in the supine position and during orthostatic tests. The control group consisted of 30 apparently healthy nonathletes matched by age and gender.

Results: Heart rates in MS patients with the FBN1 mutation were increased in both the supine position and orthostatic test (p < 0.001). Most of the time-domain (standard deviation, pNN50) and frequency-domain (total power, very low, low, and high frequency) parameters of HRV were significantly reduced in the MS patients (p < 0.001).

Conclusions: A marked decrease in HRV, documented in the study, may be an important clinical feature in MS patients with confirmed FBN1 gene mutations.  

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Keywords

Marfan syndrome, FBN1 mutation, heart rate variability, heart rate, autonomic control, transforming growth factor beta, metabolism

About this article
Title

A marked decrease in heart rate variability in Marfan syndrome patients with confirmed FBN1 mutations

Journal

Cardiology Journal

Issue

Vol 23, No 1 (2016)

Pages

23-33

Published online

2015-10-21

DOI

10.5603/CJ.a2015.0076

Pubmed

26503076

Bibliographic record

Cardiol J 2016;23(1):23-33.

Keywords

Marfan syndrome
FBN1 mutation
heart rate variability
heart rate
autonomic control
transforming growth factor beta
metabolism

Authors

Andriy Cherkas
Rustam Zhuraev

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