Impaired right ventricular systolic function demonstrated by reduced atrioventricular plane displacement in adults with Marfan syndrome.
OBJECTIVE
The right ventricle (RV) ejects the same volume of blood at the same rate as the left ventricle (LV). Mild LV dysfunction has been demonstrated in Marfan syndrome (MFS). However, little attention has been paid to the functioning of the RV. The aim of this study was to assess RV function in unoperated adult MFS patients.


RESULTS
In 66 unoperated (15-58 years) MFS patients and 61 controls, rate of pressure rise (dp/dt) in RV, and tricuspid annular motion (TAM) were studied using conventional echocardiography and tissue Doppler imaging (TDI). When compared with controls, MFS patients showed impaired RV systolic function as expressed by a reduced dp/dt, TAM obtained by M-mode echocardiography, and peak TDI systolic velocities at the basal lateral wall (745.36+/-37.85 vs. 1103.30+/-27.30 mmHg, P<0.001; 2.2+/-0.05 vs. 2.5+/-0.05 cm, P<0.001; and 0.13+/-0.002 vs. 0.16+/-0.002 m/s, P<0.001, respectively).


CONCLUSIONS
This study demonstrated a primary impairment of RV systolic function in MFS. This is the first study to report RV dysfunction in MFS. Such data could prove valuable during the peri-operative and long-term medical management of MFS patients.
Kiotsekoglou
Anatoli
A
Department of Cardiac and Vascular Sciences, St George's, University of London, Cranmer Terrace, London SW17 0RE, UK.
Sutherland
George R
GR
Moggridge
James C
JC
Kapetanakis
Venedictos
V
Bajpai
Abhay
A
Bunce
Nicholas
N
Mullen
Michael J
MJ
Louridas
George
G
Nassiri
Dariush K
DK
Camm
John
J
Child
Anne H
AH
D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D000367:Age Factors; D001283:Atrioventricular Node / Q000000981:diagnostic imaging / Q000473:pathology*; D001783:Blood Flow Velocity; D016022:Case-Control Studies; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000503:physiopathology*; D015201:Meta-Analysis as Topic; D008875:Middle Aged; D013599:Systole; D014261:Tricuspid Valve / Q000473:pathology*; D018608:Ultrasonography, Doppler; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging / Q000503:physiopathology*; D016278:Ventricular Function, Right; D055815:Young Adult
D000319:Adrenergic beta-Antagonists