Diagnosis and options for reconstructive heart valve surgery in the Marfan syndrome

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Diagnosis and options for reconstructive heart valve surgery in the Marfan syndrome. / Bernhardt, Amj; Treede, H; Rybczynski, M; Sheikzadeh, S; Meinertz, T; Reichenspurner, H; Kodolitsch, Y.

In: KARDIOLOGIYA, Vol. 51, No. 8, 2011, p. 85-90.

Research output: SCORING: Contribution to journalSCORING: Review articleResearch

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@article{d300da4744864a8d9ed70068ca1fa0b3,
title = "Diagnosis and options for reconstructive heart valve surgery in the Marfan syndrome",
abstract = "Marfan syndrome is a disorder of the connective tissue that is inherited in an autosomal-dominant fashion and that is caused by mutations in the gene coding for fibrillin-1, FBN1. Although complications of the syndrome may involve the eye, the lung and the skeleton, the high mortality of untreated cases results almost exclusively from cardiovascular complications, including aortic dissection, rupture and mitral valve regurgitation. The multiorgan involvement of many of these syndromes requires multidisciplinary expert centers that can increase the average life expectancy of affected patients from only 32 years to over 60 years. The present article both reviews classical standards of managing cardiovascular manifestations and highlights the surgical approach for aortic and mitral valve surgery in Marfan patients.",
keywords = "Adrenergic beta-Antagonists/therapeutic use, Aneurysm, Dissecting/etiology, Aorta/pathology, Aortic Aneurysm/etiology, Aortic Rupture/etiology, Cardiovascular Surgical Procedures/methods, Combined Modality Therapy, Connective Tissue/pathology, Fibrillin-1, Fibrillins, Humans, Interdisciplinary Communication, Life Expectancy, Marfan Syndrome/complications, Microfilament Proteins/genetics, Mitral Valve Insufficiency/etiology, Mutation, Patient-Centered Care/standards, Practice Patterns, Physicians'/standards",
author = "Amj Bernhardt and H Treede and M Rybczynski and S Sheikzadeh and T Meinertz and H Reichenspurner and Y Kodolitsch",
year = "2011",
language = "English",
volume = "51",
pages = "85--90",
journal = "KARDIOLOGIYA",
issn = "0022-9040",
publisher = "Media Sfera",
number = "8",

}

RIS

TY - JOUR

T1 - Diagnosis and options for reconstructive heart valve surgery in the Marfan syndrome

AU - Bernhardt, Amj

AU - Treede, H

AU - Rybczynski, M

AU - Sheikzadeh, S

AU - Meinertz, T

AU - Reichenspurner, H

AU - Kodolitsch, Y

PY - 2011

Y1 - 2011

N2 - Marfan syndrome is a disorder of the connective tissue that is inherited in an autosomal-dominant fashion and that is caused by mutations in the gene coding for fibrillin-1, FBN1. Although complications of the syndrome may involve the eye, the lung and the skeleton, the high mortality of untreated cases results almost exclusively from cardiovascular complications, including aortic dissection, rupture and mitral valve regurgitation. The multiorgan involvement of many of these syndromes requires multidisciplinary expert centers that can increase the average life expectancy of affected patients from only 32 years to over 60 years. The present article both reviews classical standards of managing cardiovascular manifestations and highlights the surgical approach for aortic and mitral valve surgery in Marfan patients.

AB - Marfan syndrome is a disorder of the connective tissue that is inherited in an autosomal-dominant fashion and that is caused by mutations in the gene coding for fibrillin-1, FBN1. Although complications of the syndrome may involve the eye, the lung and the skeleton, the high mortality of untreated cases results almost exclusively from cardiovascular complications, including aortic dissection, rupture and mitral valve regurgitation. The multiorgan involvement of many of these syndromes requires multidisciplinary expert centers that can increase the average life expectancy of affected patients from only 32 years to over 60 years. The present article both reviews classical standards of managing cardiovascular manifestations and highlights the surgical approach for aortic and mitral valve surgery in Marfan patients.

KW - Adrenergic beta-Antagonists/therapeutic use

KW - Aneurysm, Dissecting/etiology

KW - Aorta/pathology

KW - Aortic Aneurysm/etiology

KW - Aortic Rupture/etiology

KW - Cardiovascular Surgical Procedures/methods

KW - Combined Modality Therapy

KW - Connective Tissue/pathology

KW - Fibrillin-1

KW - Fibrillins

KW - Humans

KW - Interdisciplinary Communication

KW - Life Expectancy

KW - Marfan Syndrome/complications

KW - Microfilament Proteins/genetics

KW - Mitral Valve Insufficiency/etiology

KW - Mutation

KW - Patient-Centered Care/standards

KW - Practice Patterns, Physicians'/standards

M3 - SCORING: Review article

C2 - 21942965

VL - 51

SP - 85

EP - 90

JO - KARDIOLOGIYA

JF - KARDIOLOGIYA

SN - 0022-9040

IS - 8

ER -