Outcome of Stanford type B dissection in patients with Marfan syndrome

Abstract

Objective To determine the outcome of Stanford type B aortic dissection in patients with Marfan syndrome and to evaluate aortic diameters at time of dissection as well as the impact of previous aortic root replacement. Methods Analysis of all patients with Marfan syndrome fulfilling Ghent criteria seen at this institution since 1995 until 2022. Results Thirty-six (19%) out of 188 patients with Marfan syndrome suffered from Stanford type B aortic dissection during the study period. Mean aortic diameter at time of dissection was 39.0 mm (95% CI: 35.6-42.3). Mean pre-dissection diameter (available in 25% of patients) was 32.1 mm (95% CI: 28.0-36.3) and mean expansion was 19% (95% CI: 11.9-26.2). There was no correlation between age and diameter at time of dissection (<20, 21-30, 31-40, 41-50, 51-60, <61 years; p = 0.78). Freedom-from-intervention after dissection was 53%, 44%, 33% at 1, 5 and 10 years. Aortic growth rate in those patients that had to undergo intervention within the 1st year after dissection was 10.2 mm/y (95% CI: 4.4-15.9) compared to 5.8 mm/y (95% CI: 3.3-8.3), p = 0.109 in those thereafter. Mean time between dissection and intervention was 1.8 years (95% CI: 0.6-3.0). While type B dissection seems more frequent after previous elective aortic repair (58% vs 42%), there was no difference between valve-sparing root replacement (VSRR) compared to Bentall procedures (HR for VSRR 0.78, 95% CI: 0.31–2.0, p-value = 0.61). Mean age of the entire population at end of follow-up was 42 years (95% CI: 39.2–44.7). Mean follow-up time was 9 years (95% CI: 7.8–10.4). Conclusions Stanford type B dissection in patients with Marfan syndrome occurs far below accepted thresholds for intervention. Risk for type B dissection is present throughout lifetime and two third of patients need an intervention after dissection. There is no difference in freedom from type B dissection between a Bentall procedure and a valve-sparing root replacement.