Frequency and age-related course of mitral valve dysfunction in the Marfan syndrome
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Frequency and age-related course of mitral valve dysfunction in the Marfan syndrome. / Rybczynski, Meike; Mir, Thomas S; Sheikhzadeh, Sara; Bernhardt, Alexander M J; Schad, Claudia; Treede, Hendrik; Veldhoen, Simon; Groene, Eike F; Kühne, Kristin; Koschyk, Dietmar; Robinson, Peter N; Berger, Jürgen; Reichenspurner, Hermann; Meinertz, Thomas; von Kodolitsch, Yskert.
in: AM J CARDIOL, Jahrgang 106, Nr. 7, 7, 01.10.2010, S. 1048-1053.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Frequency and age-related course of mitral valve dysfunction in the Marfan syndrome
AU - Rybczynski, Meike
AU - Mir, Thomas S
AU - Sheikhzadeh, Sara
AU - Bernhardt, Alexander M J
AU - Schad, Claudia
AU - Treede, Hendrik
AU - Veldhoen, Simon
AU - Groene, Eike F
AU - Kühne, Kristin
AU - Koschyk, Dietmar
AU - Robinson, Peter N
AU - Berger, Jürgen
AU - Reichenspurner, Hermann
AU - Meinertz, Thomas
AU - von Kodolitsch, Yskert
N1 - Copyright © 2010 Elsevier Inc. All rights reserved.
PY - 2010/10/1
Y1 - 2010/10/1
N2 - Mitral valve (MV) prolapse (MVP) has a high prevalence of 2% to 3% in the general population and thus constitutes the most common cause of severe nonischemic MV regurgitation (MVR). MVP is also common in persons with the Marfan syndrome. However, to date, a large-scale population-based cohort study using modern echocardiographic techniques has not been performed, and the frequency of MVP and the relation of MV dysfunction and age have not been investigated. Therefore, we conducted a population-based cohort study of 204 patients (108 males and 96 females, aged 31.2 ± 16.4 years) with classic Marfan syndrome. We performed echocardiographic follow-up of 174 patients for a mean of 4.4 ± 4.3 years. On the initial or subsequent echocardiographic scan, MVP was present in 82 patients (40%), severe MVR in 25 (12%), and MV endocarditis in 5 patients (2.5%). At 30 years of age, the Weibull cumulative distribution was 42.6% (95% confidence interval [CI] 36% to 50%) for MVP, 56.5% (95% CI 49.3% to 64%) for MVR of any degree, 6.7% (95% CI 3.9% to 11.3%) for severe MVR, and 0.92% (95% CI 0.21% to 3.91%) for MV endocarditis. The cumulative hazard for severe MVR and MV endocarditis was estimated to increase with age. MVP was associated with dural ectasia (p = 0.01), ectopia lentis (p = 0.02), and skeletal involvement (p
AB - Mitral valve (MV) prolapse (MVP) has a high prevalence of 2% to 3% in the general population and thus constitutes the most common cause of severe nonischemic MV regurgitation (MVR). MVP is also common in persons with the Marfan syndrome. However, to date, a large-scale population-based cohort study using modern echocardiographic techniques has not been performed, and the frequency of MVP and the relation of MV dysfunction and age have not been investigated. Therefore, we conducted a population-based cohort study of 204 patients (108 males and 96 females, aged 31.2 ± 16.4 years) with classic Marfan syndrome. We performed echocardiographic follow-up of 174 patients for a mean of 4.4 ± 4.3 years. On the initial or subsequent echocardiographic scan, MVP was present in 82 patients (40%), severe MVR in 25 (12%), and MV endocarditis in 5 patients (2.5%). At 30 years of age, the Weibull cumulative distribution was 42.6% (95% confidence interval [CI] 36% to 50%) for MVP, 56.5% (95% CI 49.3% to 64%) for MVR of any degree, 6.7% (95% CI 3.9% to 11.3%) for severe MVR, and 0.92% (95% CI 0.21% to 3.91%) for MV endocarditis. The cumulative hazard for severe MVR and MV endocarditis was estimated to increase with age. MVP was associated with dural ectasia (p = 0.01), ectopia lentis (p = 0.02), and skeletal involvement (p
KW - Adult
KW - Age Factors
KW - Female
KW - Humans
KW - Male
KW - Marfan Syndrome/complications
KW - Middle Aged
KW - Mitral Valve Prolapse/complications
KW - Ultrasonography
U2 - 10.1016/j.amjcard.2010.05.038
DO - 10.1016/j.amjcard.2010.05.038
M3 - SCORING: Journal article
C2 - 20854973
VL - 106
SP - 1048
EP - 1053
JO - AM J CARDIOL
JF - AM J CARDIOL
SN - 0002-9149
IS - 7
M1 - 7
ER -