Outcome of aortic dissection affecting the descending aorta in Marfan Syndrome
Authors: Calvo-Barcelo M , Garcia-Duran A , Lopez-Sainz A , Limeres-Freire J , Gutierrez-Garcia-Moreno L , Galian-Gay L , Casas-Masnou G , Gonzalez-Alujas T , Fernandez-Galera R , Valente F , Olivero-Soldevila R , Rodriguez-Palomares J , Ferreira-Gonzalez I , Evangelista-Masip A , Teixido-Tura G 
Published: October 2022

Introduction

Marfan syndrome (MFS) is a connective tissue disorder related to FBN1 mutations. The main cardiac feature of this syndrome is the enlargement and the dissection of the ascending aorta. Close monitoring of the aortic root diameters and its preventive replacement has allowed improving life expectancy of these patients in the last decades. However, there is paucity of data about the outcome of aortic dissection affecting the descending aorta and its markers of poor evolution.

Purpose

To characterize patients with MFS and dissection involving the descending aorta. To compare baseline characteristics and the clinical outcome between patients with isolated type B dissection (Group B) and patients with type A dissection with residual B dissection (Group A).

Methods

Retrospective longitudinal analysis of patients that attended our Marfan unit between 2001 and 2021 with an aortic event involving the descending aorta. Clinical data were collected from electronic health records.

Results

From the global cohort of adults with Marfan syndrome seen at our centre, we identified a total of 37 adults (11.5% of the overall cohort) that had an acute aortic event with involvement of the descending aorta. 20 (54.1%) were type A aortic events and 17 (45.9%) were isolated type B dissections. Cardiovascular risk factors were similarly distributed in both groups but there was a significant higher percentage of women in Group B (35.0% vs 76.5%, p=0.012) (Figure 1).

Acute management of the aortic event implied surgery in 17 (85.0%) patients in Group A and in 5 (29.4%) patients in Group B (p=0.001). In Group B, interventions were 3 TEVAR and 2 open surgeries.

2 (5.4%) patient died during the acute phase, 1 (5.0%) in Group A and 1 (5.9%) in Group B (p=ns).The remaining 35 patients (19 in Group A, 16 in Group B) were followed-up for 10.4±6.7 years. 10 patients died during follow-up (6 in Group A, 4 in Group B). In Group A, 2 patients died of sudden death, 2 patients died in the perioperative setting of aortic surgery, and 2 patients died of an aortic rupture. In Group B, 3 patients died in the perioperative setting of aortic surgery and 1 of an intracranial hemorrhage.

The proportions of aortic events during follow-up (death, distal redissection, proximal dissection, distal intervention or aortic root surgery) in both groups are described in Figure 2. Median survival free of reintervention or death (non-related to initial event) was 7.5 years in the overall cohort; 8.2 years in Group A and 2.2 years in Group B (log rank p=0.04) (Figure 2).

Conclusions

Patients with Marfan syndrome and aortic dissection involving the descending aorta have a poor clinical outcome, with high rates of reintervention, redissection or death. Although Group B required less often surgery during the acute phase, worst survival free of reintervention or death was observed during follow-up.

Funding Acknowledgement

Type of funding sources: None.