Acute Aortic Dissection Determines the Fate of Initially Untreated Aortic Segments in Marfan Syndrome
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- Florian S. Schoenhoff
- From the Department of Cardiovascular Surgery, University Hospital Berne, Berne, Switzerland (F.S.S., S.J., M.C., E.R., D.R., J.S., A.K., T.C.); the Division of Metabolism and Molecular Pediatrics, University Children’s Hospital Zurich, Zurich, Switzerland (G.M., B.S.); the Institute of Medical Genetics, Division of Medical Molecular Genetics and Gene Diagnostics, University of Zurich, Zurich, Switzerland (G.M.); and the Center for Cardiovascular Genetics and Gene Diagnostics, Zurich, Switzerland (G...
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- Silvan Jungi
- From the Department of Cardiovascular Surgery, University Hospital Berne, Berne, Switzerland (F.S.S., S.J., M.C., E.R., D.R., J.S., A.K., T.C.); the Division of Metabolism and Molecular Pediatrics, University Children’s Hospital Zurich, Zurich, Switzerland (G.M., B.S.); the Institute of Medical Genetics, Division of Medical Molecular Genetics and Gene Diagnostics, University of Zurich, Zurich, Switzerland (G.M.); and the Center for Cardiovascular Genetics and Gene Diagnostics, Zurich, Switzerland (G...
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- Martin Czerny
- From the Department of Cardiovascular Surgery, University Hospital Berne, Berne, Switzerland (F.S.S., S.J., M.C., E.R., D.R., J.S., A.K., T.C.); the Division of Metabolism and Molecular Pediatrics, University Children’s Hospital Zurich, Zurich, Switzerland (G.M., B.S.); the Institute of Medical Genetics, Division of Medical Molecular Genetics and Gene Diagnostics, University of Zurich, Zurich, Switzerland (G.M.); and the Center for Cardiovascular Genetics and Gene Diagnostics, Zurich, Switzerland (G...
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- Eva Roost
- From the Department of Cardiovascular Surgery, University Hospital Berne, Berne, Switzerland (F.S.S., S.J., M.C., E.R., D.R., J.S., A.K., T.C.); the Division of Metabolism and Molecular Pediatrics, University Children’s Hospital Zurich, Zurich, Switzerland (G.M., B.S.); the Institute of Medical Genetics, Division of Medical Molecular Genetics and Gene Diagnostics, University of Zurich, Zurich, Switzerland (G.M.); and the Center for Cardiovascular Genetics and Gene Diagnostics, Zurich, Switzerland (G...
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- David Reineke
- From the Department of Cardiovascular Surgery, University Hospital Berne, Berne, Switzerland (F.S.S., S.J., M.C., E.R., D.R., J.S., A.K., T.C.); the Division of Metabolism and Molecular Pediatrics, University Children’s Hospital Zurich, Zurich, Switzerland (G.M., B.S.); the Institute of Medical Genetics, Division of Medical Molecular Genetics and Gene Diagnostics, University of Zurich, Zurich, Switzerland (G.M.); and the Center for Cardiovascular Genetics and Gene Diagnostics, Zurich, Switzerland (G...
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- Gabor Matyas
- From the Department of Cardiovascular Surgery, University Hospital Berne, Berne, Switzerland (F.S.S., S.J., M.C., E.R., D.R., J.S., A.K., T.C.); the Division of Metabolism and Molecular Pediatrics, University Children’s Hospital Zurich, Zurich, Switzerland (G.M., B.S.); the Institute of Medical Genetics, Division of Medical Molecular Genetics and Gene Diagnostics, University of Zurich, Zurich, Switzerland (G.M.); and the Center for Cardiovascular Genetics and Gene Diagnostics, Zurich, Switzerland (G...
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- Beat Steinmann
- From the Department of Cardiovascular Surgery, University Hospital Berne, Berne, Switzerland (F.S.S., S.J., M.C., E.R., D.R., J.S., A.K., T.C.); the Division of Metabolism and Molecular Pediatrics, University Children’s Hospital Zurich, Zurich, Switzerland (G.M., B.S.); the Institute of Medical Genetics, Division of Medical Molecular Genetics and Gene Diagnostics, University of Zurich, Zurich, Switzerland (G.M.); and the Center for Cardiovascular Genetics and Gene Diagnostics, Zurich, Switzerland (G...
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- Juerg Schmidli
- From the Department of Cardiovascular Surgery, University Hospital Berne, Berne, Switzerland (F.S.S., S.J., M.C., E.R., D.R., J.S., A.K., T.C.); the Division of Metabolism and Molecular Pediatrics, University Children’s Hospital Zurich, Zurich, Switzerland (G.M., B.S.); the Institute of Medical Genetics, Division of Medical Molecular Genetics and Gene Diagnostics, University of Zurich, Zurich, Switzerland (G.M.); and the Center for Cardiovascular Genetics and Gene Diagnostics, Zurich, Switzerland (G...
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- Alexander Kadner
- From the Department of Cardiovascular Surgery, University Hospital Berne, Berne, Switzerland (F.S.S., S.J., M.C., E.R., D.R., J.S., A.K., T.C.); the Division of Metabolism and Molecular Pediatrics, University Children’s Hospital Zurich, Zurich, Switzerland (G.M., B.S.); the Institute of Medical Genetics, Division of Medical Molecular Genetics and Gene Diagnostics, University of Zurich, Zurich, Switzerland (G.M.); and the Center for Cardiovascular Genetics and Gene Diagnostics, Zurich, Switzerland (G...
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- Thierry Carrel
- From the Department of Cardiovascular Surgery, University Hospital Berne, Berne, Switzerland (F.S.S., S.J., M.C., E.R., D.R., J.S., A.K., T.C.); the Division of Metabolism and Molecular Pediatrics, University Children’s Hospital Zurich, Zurich, Switzerland (G.M., B.S.); the Institute of Medical Genetics, Division of Medical Molecular Genetics and Gene Diagnostics, University of Zurich, Zurich, Switzerland (G.M.); and the Center for Cardiovascular Genetics and Gene Diagnostics, Zurich, Switzerland (G...
説明
<jats:sec> <jats:title>Background—</jats:title> <jats:p>The aim of the current study was to investigate incidence and causes of surgical interventions in primarily nontreated aortic segments after previous aortic repair in patients with Marfan syndrome.</jats:p> </jats:sec> <jats:sec> <jats:title>Methods and Results—</jats:title> <jats:p> Retrospective analysis of 86 consecutive Marfan syndrome patients fulfilling Ghent criteria that underwent 136 aortic surgeries and were followed at this institution in the past 15 years. Mean follow-up was 8.8±6.8 y. Thirty-day, 6-month, 1-year, and overall mortality was 3.5%, 5.8%, 7.0%, and 12.8%, respectively. Ninety-two percent of patients initially presented with aortic root, ascending aortic or arch lesions, whereas 8% presented with descending aortic or thoraco-abdominal lesions. Primary presentation was acute aortic dissection (AAD) in 36% (77% type A, 23% type B) and aneurismal disease in 64%. Secondary complete arch replacement had to be performed in only 6% of patients without AAD, but in 36% with AAD ( <jats:italic>P</jats:italic> =0.0005). In patients without AAD, 11% required surgery on primarily nontreated aortic segments (5 of 6 patients experienced type B dissection during follow-up), whereas in patients after AAD, 48% underwent surgery of initially nontreated aortic segments (42% of patients with type A and 86% of those with type B dissection; <jats:italic>P</jats:italic> =0.0002). </jats:p> </jats:sec> <jats:sec> <jats:title>Conclusions—</jats:title> <jats:p>The need for surgery in primarily nontreated aortic segments is precipitated by an initial presentation with AAD. Early elective surgery is associated with low mortality and reintervention rates. Type B dissection in patients with Marfan syndrome is associated with a high need for extensive aortic repair, even if the dissection is being considered uncomplicated by conventional criteria.</jats:p> </jats:sec>
収録刊行物
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- Circulation
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Circulation 127 (15), 1569-1575, 2013-04-16
Ovid Technologies (Wolters Kluwer Health)
