Key result
Orthotopic heart transplantation in patients with Marfan syndrome had no difference in mortality compared to patients without Marfan syndrome.
Why the study?
Many centers are reluctant to offer orthotopic heart transplantation for Marfan syndrome patients with concurrent aortic disease due to surgical complexity and perceived inferior outcomes compared to patients without Marfan syndrome.
Does orthotopic heart transplantation improve survival in patients with Marfan syndrome and concurrent end-stage cardiomyopathy and aortic disease compared to patients without MFS?
Case Report (n=1)
Does orthotopic heart transplantation improve survival in patients with Marfan syndrome and concurrent end-stage cardiomyopathy and aortic disease compared to patients without MFS?
Orthotopic heart transplantation is a viable, life-saving option for patients with Marfan syndrome and concurrent aortic disease, with mortality comparable to non-MFS patients.
Supports transplantation consideration in Marfan syndrome; leaves open confirmation in larger studies.
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.
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Smith et al. (2021) conducted a case report in Marfan syndrome with concurrent end-stage cardiomyopathy and aortic disease (n=1). Orthotopic heart transplantation vs. Patients without Marfan syndrome was evaluated on Mortality. Orthotopic heart transplantation in patients with Marfan syndrome had no difference in mortality compared to patients without Marfan syndrome.
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