Abstract

Introduction: Conservative treatment is the treatment of choice in patients (pts) with non- complicated acute type B aortic dissection (TBAAD). This strategy has not been validated in patients with Marfan Syndrome (MFS) presenting with non-complicated TBAAD. Methods: Our study evaluated 586 medically managed patients with non-complicated TBAAD enrolled in IRAD between 1996 and 2011. The patients were divided into groups based on the presence (N=15, 2.6%) or absence (N=571, 97.4%) of MFS. Categorical variables were analyzed using chi-square test and Fischer’s exact test where applicable. Continuous variables were analyzed using student’s t-test for normally distributed data, and test of medians for non-normally distributed data. Results: The mean age was significantly lower for MFS patients (41.3±9.4 years vs 65±13.5 years [p<0.001]). Significantly more patients without MFS had history of hypertension (61.5% vs 40% [p<0.001]). Additionally, those with MFS had significantly higher incidence of prior aortic dissection (64.3% vs 5.8% [p<0.001]), and family history of aortic disease (75% vs 9.5% [p<0.001]). Significantly more non-MFS patients presented with hypertension (70% vs 33.3% [p<0.008]). Abdominal vessels involvement was more frequent in patients with MFS (53.3% vs 22.8% [p=0.011]). During the hospitalization, more patients with MFS had extension of their dissection (20% vs 4% [p=0.024]). None of the patients with MFS died during the index hospitalization, nor did they have new neurological or visceral ischemia. Kaplan-Meyer survival curve showed no significant difference in survival between the two groups from admission to five years follow-up. Conclusion: The presence of dissection extension in patients with MFS warrants special in-hospital attention. However, our results indicate that, similar to patients without MFS, conservative treatment may be safe in Marfan Syndrome patients presenting with non-complicated acute type B aortic dissection. Given the limited study population, more research is needed to definitively analyze whether non-complicated TBAAD MFS patients would benefit from conservative versus more aggressive dissection management.

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