Long-term outcome of patients with Marfan syndrome with previous aortic surgery but native aortic roots. (19th September 2020)

• Record Type: Journal Article
• Authors: Mosbahi, Selim; Yildiz, Murat; Heinisch, Paul-Philipp; Langhammer, Bettina; Jungi, Silvan; Carrel, Thierry P; Schoenhoff, Florian S
• Is Part Of: European journal of cardio-thoracic surgery. Volume 58:Number 6(2020)
• Page Start: 1289
• Page End: 1295
• Publication Date: 2020-09-19
• Subjects: Marfan syndrome -- Aortic root -- Aortic surgery -- Aortic dissection; Heart -- Surgery -- Periodicals; Chest -- Surgery -- Periodicals; 617.54
• DOI: 10.1093/ejcts/ezaa221
• Languages: English
• ISSNs: 1010-7940

Abstract

Abstract : Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder caused by mutations in the gene encoding for the extracellular matrix protein fibrillin-1 [1] leading to dysregulation of the transforming growth factor-beta signalling pathway [2]. Abstract: OBJECTIVES: The goal of this study was to report the long-term outcomes of patients with Marfan syndrome who had aortic surgery on any aortic segment except for the replacement of the aortic root itself. METHODS: An observational retrospective single-centre study was conducted with 115 Marfan syndrome patients who underwent 189 major aortic interventions from 1995 until 2018. Patients without aortic root replacement were identified and aortic root growth was analysed over time. RESULTS: Eleven of 115 patients (9.5%) did not have aortic root replacement during a follow-up of 10.5 [standard deviation (SD) 5.7] years and a mean age at last follow-up of 53.9 (SD 13.4) years. Patients without root replacement did not suffer less frequently from any type of acute aortic dissection (type A 27% vs 25%, P = 0.999; type B 36% vs 25%, P = 0.474). Patients with native aortic roots did not undergo fewer aortic interventions than those with aortic root replacement [12/11, mean 1.09 (SD 0.54) operations/patient vs 177/104, mean 1.7 (SD 1.3); P = 0.128]. Progression of the aortic root dimension was 0.5 (SD 0.3) mm/year in the group of patients with native aortic roots. CONCLUSIONS: Current data suggest that 10% of patients with Marfan syndrome with previous aortic surgery will be free from aortic root replacement until the sixth decade of life.