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Concurrent End-Stage Cardiomyopathy and Aortic Disease in Patients with Marfan Syndrome: A Case Report and Literature Review
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  • Timothy Smith,
  • Jose Sleiman,
  • Nikita Zadneulitca,
  • Cedric Sheffield,
  • Viviana Navas,
  • Jaime Hernandez-Montfort,
  • Mauricio Velez,
  • Nicolas Brozzi
Timothy Smith
Cleveland Clinic Florida
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Jose Sleiman
Cleveland Clinic Florida
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Nikita Zadneulitca
Cleveland Clinic Florida
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Cedric Sheffield
Cleveland Clinic Florida
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Viviana Navas
Cleveland Clinic Florida
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Jaime Hernandez-Montfort
Cleveland Clinic Florida
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Mauricio Velez
Cleveland Clinic Florida
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Nicolas Brozzi
Cleveland Clinic Foundation
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Abstract

Abstract Background: Marfan syndrome (MFS) is a connective tissue disorder that can lead to aortic disease, arrhythmias and heart failure. Many centers are reluctant to offer orthotopic heart transplantation (OHT) for patients with MFS with concurrent aortic disease due to complexity of the surgery and perceived inferior results when compared to patients without MFS. Methods: We present a case of a patient with MFS with previous Bentall procedure who underwent successful OHT, accompanied by a literature review on OHT performed for patients with MFS. Results and Conclusions: Patients with MFS who underwent OHT had no difference in mortality compared to patients without MFS. Even though OHT is technically more challenging when combined with concurrent intervention for aortic disease, it should be considered as a life-saving operation for patients with MFS.

Peer review status:UNDER REVIEW

26 May 2021Submitted to Journal of Cardiac Surgery
02 Jun 2021Assigned to Editor
02 Jun 2021Submission Checks Completed
02 Jun 2021Reviewer(s) Assigned