Primary cardiac impairment in patients with Marfan syndrome undergoing a Bentall procedure.

2020 
Abstract Background In this study, we sought to verify the existence of primary cardiac impairment in Marfan patients undergoing a Bentall procedure and to explore its clinical significance. Methods Between October 2010 and April 2019, 164 patients with Marfan syndrome, and, 748 non-Marfan patients with aortic root aneurysms underwent a Bentall procedure. The clinical characteristics and perioperative cardiac structure and function between two 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 mid-term survival between these two groups were compared. Results After propensity score matching, the two groups had similar preoperative baseline characteristics. The Marfan patients presented a higher preoperative New York Heart Association classification, larger left ventricular size and poorer postoperative cardiac function compared to the non-Marfan patients. In our multivariate regression analysis, Marfan syndrome was associated with a lower postoperative left ventricular ejection fraction. Furthermore, Marfan syndrome was an independent risk factor for adverse postoperative cardiogenic events during their hospital stay (odds ratio, 6.243,95% Confidence interval, 1.71-22.75, P=0.006). There was no difference in midterm survival between two groups (P=0.772). Conclusions Clinical manifestations observed in Marfan patients supported the existence of primary cardiac impairment. Due to a higher rate of postoperative cardiogenic adverse events in Marfan patients, the potential impact of primary cardiac impairment in Marfan syndrome should be carefully considered in cardiovascular surgery.
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