AIMS: To better characterize patients with Marfan syndrome who have survived an acute aortic dissection and to estimate the risks of events in the descending aorta. Up until now, this portion of the aorta has not been well studied but is gaining importance due to improved patient survival. METHODS AND RESULTS: We report a retrospective cohort of 100 Marfan patients who survived an aortic dissection. Dissection occurred in either the ascending aorta (AscAo) (n = 37), the descending aorta (DescAo) (n = 20), or both (As + DescAo, n = 43). During a mean follow-up of 9.8 ± 6.0 years (complete for 88% of the patients), 17 patients died and 52 had a clinical event (new aortic dissection, surgery, ischaemia, haemorrhage), 60% of which involved the descending aorta. Event-free survival was similar whatever the location of the aortic dissection. However, a better event-free survival was observed when no dissected portion of the aorta remained after surgery, which was the case in 62% (23/37) of the AscAo patients (30% incurred an event vs. 86%; P = 0.008 by log-rank test). Interestingly, the diameter of the ascending aorta was below the surgical threshold in 60% of the patients who incurred a dissection of the descending aorta, and within the normal range in 25%. CONCLUSION: The descending aorta may dissect whatever the diameter of the ascending aorta. The descending aorta is the location of most late clinical events after any dissection of the aorta. The rate of clinical events is much lower when all the dissected aorta has been removed in patients with AscAo dissection.
AIMS: To better characterize patients with Marfan syndrome who have survived an acute aortic dissection and to estimate the risks of events in the descending aorta. Up until now, this portion of the aorta has not been well studied but is gaining importance due to improved patient survival. METHODS AND RESULTS: We report a retrospective cohort of 100 Marfan patients who survived an aortic dissection. Dissection occurred in either the ascending aorta (AscAo) (n = 37), the descending aorta (DescAo) (n = 20), or both (As + DescAo, n = 43). During a mean follow-up of 9.8 ± 6.0 years (complete for 88% of the patients), 17 patients died and 52 had a clinical event (new aortic dissection, surgery, ischaemia, haemorrhage), 60% of which involved the descending aorta. Event-free survival was similar whatever the location of the aortic dissection. However, a better event-free survival was observed when no dissected portion of the aorta remained after surgery, which was the case in 62% (23/37) of the AscAo patients (30% incurred an event vs. 86%; P = 0.008 by log-rank test). Interestingly, the diameter of the ascending aorta was below the surgical threshold in 60% of the patients who incurred a dissection of the descending aorta, and within the normal range in 25%. CONCLUSION: The descending aorta may dissect whatever the diameter of the ascending aorta. The descending aorta is the location of most late clinical events after any dissection of the aorta. The rate of clinical events is much lower when all the dissected aorta has been removed in patients with AscAo dissection.
Authors: Jonathan W Weinsaft; Richard B Devereux; Liliana R Preiss; Attila Feher; Mary J Roman; Craig T Basson; Alexi Geevarghese; William Ravekes; Harry C Dietz; Kathryn Holmes; Jennifer Habashi; Reed E Pyeritz; Joseph Bavaria; Karianna Milewski; Scott A LeMaire; Shaine Morris; Dianna M Milewicz; Siddharth Prakash; Cheryl Maslen; Howard K Song; G Michael Silberbach; Ralph V Shohet; Nazli McDonnell; Tabitha Hendershot; Kim A Eagle; Federico M Asch Journal: J Am Coll Cardiol Date: 2016-06-14 Impact factor: 24.094
Authors: Dianna M Milewicz; Alan C Braverman; Julie De Backer; Shaine A Morris; Catherine Boileau; Irene H Maumenee; Guillaume Jondeau; Arturo Evangelista; Reed E Pyeritz Journal: Nat Rev Dis Primers Date: 2021-09-02 Impact factor: 65.038
Authors: Lisa Q Rong; Alexandra J Lopes; Hannah W Mitlak; Maria C Palumbo; Stephanie Mick; Jiwon Kim; Robert A Levine; S Chiu Wong; Mark Reisman; Richard B Devereux; Mario Gaudino; Jonathan W Weinsaft Journal: J Cardiothorac Vasc Anesth Date: 2021-09-10 Impact factor: 2.894
Authors: Yskert von Kodolitsch; Julie De Backer; Helke Schüler; Peter Bannas; Cyrus Behzadi; Alexander M Bernhardt; Mathias Hillebrand; Bettina Fuisting; Sara Sheikhzadeh; Meike Rybczynski; Tilo Kölbel; Klaus Püschel; Stefan Blankenberg; Peter N Robinson Journal: Appl Clin Genet Date: 2015-06-16
Authors: R Franken; T Radonic; A W den Hartog; M Groenink; G Pals; M van Eijk; R Lutter; B J M Mulder; A H Zwinderman; V de Waard Journal: Neth Heart J Date: 2015-02 Impact factor: 2.380