Concurrent End-Stage Cardiomyopathy and Aortic Disease in Patients with
Marfan Syndrome: A Case Report and Literature Review
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.