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, Jahrgang 51, Nr. 8, 2011, S. 85-90.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Review › Forschung
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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 -