Left ventricular diastolic dysfunction in children and young adults with Marfan syndrome

B. B. Das, A. L. Taylor, Angela T Yetman

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

Adults with Marfan syndrome (MFS) demonstrate abnormal aortic elastic properties manifest by decreased aortic distensibility and increased aortic stiffness. Left ventricular (LV) diastolic dysfunction has been reported in adults with MFS. The objective of this study was to assess the frequency of LV diastolic dysfunction in a group of children and young adults with MFS and to determine whether diastolic dysfunction is associated with hemodynamic alterations of the aorta. Review of echocardiographic findings in 40 patients with MFS was performed to assess LV size, systolic function, isovolumic relaxation time (IVRT), mitral inflow velocities, deceleration time (DT) of mitral E wave, and aortic root dimension. No patient had significant valvar disease or was on any cardiac medication at the time of study. A group of 40 age and sex-matched healthy subjects undergoing echocardiography served as controls. Significant differences in LV diastolic function were found between MFS patients and controls. MFS patients had prolonged DT and IVRT and decreased mitral E/A ratio, suggesting impaired LV relaxation. No relationship between aortic root dimension and diastolic performance was identified. Left ventricular diastolic dysfunction may be an early marker of myocardial involvement in young MFS patients occurring independently of aortic root dilatation.

Original languageEnglish (US)
Pages (from-to)256-258
Number of pages3
JournalPediatric Cardiology
Volume27
Issue number2
DOIs
StatePublished - Apr 1 2006

Fingerprint

Marfan Syndrome
Left Ventricular Dysfunction
Young Adult
Deceleration
Vascular Stiffness
Time and Motion Studies
Left Ventricular Function
Echocardiography
Aorta
Dilatation
Healthy Volunteers
Age Groups
Hemodynamics

Keywords

  • Diastolic dysfunction
  • Marfan syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

Cite this

Left ventricular diastolic dysfunction in children and young adults with Marfan syndrome. / Das, B. B.; Taylor, A. L.; Yetman, Angela T.

In: Pediatric Cardiology, Vol. 27, No. 2, 01.04.2006, p. 256-258.

Research output: Contribution to journalArticle

@article{4cb2a0e52c7147ddbcf2d834e36430bd,
title = "Left ventricular diastolic dysfunction in children and young adults with Marfan syndrome",
abstract = "Adults with Marfan syndrome (MFS) demonstrate abnormal aortic elastic properties manifest by decreased aortic distensibility and increased aortic stiffness. Left ventricular (LV) diastolic dysfunction has been reported in adults with MFS. The objective of this study was to assess the frequency of LV diastolic dysfunction in a group of children and young adults with MFS and to determine whether diastolic dysfunction is associated with hemodynamic alterations of the aorta. Review of echocardiographic findings in 40 patients with MFS was performed to assess LV size, systolic function, isovolumic relaxation time (IVRT), mitral inflow velocities, deceleration time (DT) of mitral E wave, and aortic root dimension. No patient had significant valvar disease or was on any cardiac medication at the time of study. A group of 40 age and sex-matched healthy subjects undergoing echocardiography served as controls. Significant differences in LV diastolic function were found between MFS patients and controls. MFS patients had prolonged DT and IVRT and decreased mitral E/A ratio, suggesting impaired LV relaxation. No relationship between aortic root dimension and diastolic performance was identified. Left ventricular diastolic dysfunction may be an early marker of myocardial involvement in young MFS patients occurring independently of aortic root dilatation.",
keywords = "Diastolic dysfunction, Marfan syndrome",
author = "Das, {B. B.} and Taylor, {A. L.} and Yetman, {Angela T}",
year = "2006",
month = "4",
day = "1",
doi = "10.1007/s00246-005-1139-5",
language = "English (US)",
volume = "27",
pages = "256--258",
journal = "Pediatric Cardiology",
issn = "0172-0643",
publisher = "Springer New York",
number = "2",

}

TY - JOUR

T1 - Left ventricular diastolic dysfunction in children and young adults with Marfan syndrome

AU - Das, B. B.

AU - Taylor, A. L.

AU - Yetman, Angela T

PY - 2006/4/1

Y1 - 2006/4/1

N2 - Adults with Marfan syndrome (MFS) demonstrate abnormal aortic elastic properties manifest by decreased aortic distensibility and increased aortic stiffness. Left ventricular (LV) diastolic dysfunction has been reported in adults with MFS. The objective of this study was to assess the frequency of LV diastolic dysfunction in a group of children and young adults with MFS and to determine whether diastolic dysfunction is associated with hemodynamic alterations of the aorta. Review of echocardiographic findings in 40 patients with MFS was performed to assess LV size, systolic function, isovolumic relaxation time (IVRT), mitral inflow velocities, deceleration time (DT) of mitral E wave, and aortic root dimension. No patient had significant valvar disease or was on any cardiac medication at the time of study. A group of 40 age and sex-matched healthy subjects undergoing echocardiography served as controls. Significant differences in LV diastolic function were found between MFS patients and controls. MFS patients had prolonged DT and IVRT and decreased mitral E/A ratio, suggesting impaired LV relaxation. No relationship between aortic root dimension and diastolic performance was identified. Left ventricular diastolic dysfunction may be an early marker of myocardial involvement in young MFS patients occurring independently of aortic root dilatation.

AB - Adults with Marfan syndrome (MFS) demonstrate abnormal aortic elastic properties manifest by decreased aortic distensibility and increased aortic stiffness. Left ventricular (LV) diastolic dysfunction has been reported in adults with MFS. The objective of this study was to assess the frequency of LV diastolic dysfunction in a group of children and young adults with MFS and to determine whether diastolic dysfunction is associated with hemodynamic alterations of the aorta. Review of echocardiographic findings in 40 patients with MFS was performed to assess LV size, systolic function, isovolumic relaxation time (IVRT), mitral inflow velocities, deceleration time (DT) of mitral E wave, and aortic root dimension. No patient had significant valvar disease or was on any cardiac medication at the time of study. A group of 40 age and sex-matched healthy subjects undergoing echocardiography served as controls. Significant differences in LV diastolic function were found between MFS patients and controls. MFS patients had prolonged DT and IVRT and decreased mitral E/A ratio, suggesting impaired LV relaxation. No relationship between aortic root dimension and diastolic performance was identified. Left ventricular diastolic dysfunction may be an early marker of myocardial involvement in young MFS patients occurring independently of aortic root dilatation.

KW - Diastolic dysfunction

KW - Marfan syndrome

UR - http://www.scopus.com/inward/record.url?scp=33645123434&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33645123434&partnerID=8YFLogxK

U2 - 10.1007/s00246-005-1139-5

DO - 10.1007/s00246-005-1139-5

M3 - Article

VL - 27

SP - 256

EP - 258

JO - Pediatric Cardiology

JF - Pediatric Cardiology

SN - 0172-0643

IS - 2

ER -