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Concurrent End-Stage Cardiomyopathy and Aortic Disease in Patients with Marfan Syndrome
Timothy Smith1, Jose Sleiman2, Nikita Zadneulitca3, Cedric Sheffield3, Viviana Navas2, Jaime Hernandez-Montfort2, Mauricio Velez2, Nicolas Brozzi3.
1Ross University School of medicine, Bridgetown, Barbados, 2Cleveland Clinic Florida Department of Cardiology, Weston, FL, USA, 3Cleveland Clinic Florida Department of Cardiothoracic Surgery, Weston, FL, USA.

Background: Marfan syndrome (MFS) is a connective tissue disorder that can lead to aortic disease, arrhythmias and heart failure. Many centers historically have been 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, non-ischemic chronic biventricular systolic heart failure (Ejection fraction = 10%), ascending aortic aneurysm repaired by Bentall procedure 1 month prior, who had an out of hospital cardiac arrest followed by multiple episodes of ventricular arrhythmias due to end-stage cardiomyopathy, who underwent a heart transplant at our facility.Results and Conclusions: Our patient had a full recovery and is now 6 months post transplants with no complications or readmissions. Literature review showed 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.


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