Aortic Root Replacement and Berlin Heart EXCOR Implantation in Marfan Syndrome

Abstract

Aortic regurgitation secondary to aortic aneurysmal disease with subsequent long-term volume overload of the left ventricle is associated with left ventricular dysfunction. In Marfan syndrome, ventricular dysfunction may exist independent of valvular disease (Marfan cardiomyopathy). We report of a 20-year-old Marfan syndrome patient presenting with severe aortic regurgitation and severe heart failure. Aortic surgery with concurrent biventricular paracorporeal assist device implantation was performed to bridge the patient to heart transplantation, which was performed successfully 21 weeks after aortic surgery. While the optimal strategy for this situation has not been described, the approach applied here appears a valid option.