Primary Cardiac Impairment in Patients With Marfan Syndrome Undergoing a Bentall Procedure

Abstract

Background: In this study we sought to verify the existence of primary cardiac impairment in patients with Marfan syndrome (MFS) undergoing a Bentall procedure and to explore its clinical significance.

Methods: Between October 2010 and April 2019, 164 patients with MFS and 748 patients without MFS with aortic root aneurysms underwent a Bentall procedure. Clinical characteristics and perioperative cardiac structure and function between both groups were compared after propensity score matching. Multivariate regression analysis was performed to identify factors that influenced the postoperative left ventricular ejection fraction and any adverse cardiogenic events. The midterm survival between these 2 groups were compared.

Results: After propensity score matching both groups had similar preoperative baseline characteristics. The MFS patients presented a higher preoperative New York Heart Association classification, larger left ventricular size, and poorer postoperative cardiac function compared with non-MFS patients. In our multivariate regression analysis MFS was associated with a lower postoperative left ventricular ejection fraction. Furthermore MFS was an independent risk factor for adverse postoperative cardiogenic events during patients' hospital stay (odds ratio, 6.243; 95% confidence interval, 1.71-22.75; P = .006). There was no difference in midterm survival between the 2 groups (P = .772).

Conclusions: Clinical manifestations observed in MFS patients supported the existence of primary cardiac impairment. Because of a higher rate of postoperative cardiogenic adverse events in MFS patients, the potential impact of primary cardiac impairment in MFS should be carefully considered in cardiovascular surgery.