Thoracic Aortic Aneurysm Surgery in Marfan Patients: a Perspective from the UK

Abstract

Objective: Cardiovascular complications in Marfan patients include progressive aortic root dilation which can precipitate acute aortic dissection, ruptured aorta, severe aortic regurgitation, or all the aforementioned. Such complications can be fatal and the cause of death prior to any surgical intervention. We set out to identify the Marfan population in England and Wales and present their surgical outcomes. Methods: A total of 306 patients with Marfan syndrome who underwent aortic root surgery were identified between April 2007 and March 2013 from NICOR database. We examined the perioperative characteristics of such cohort along with in-hospital outcomes and survival. Results: Root and ascending segment procedures on Marfan patients performed in 3.3% of the total cohort by NICOR root surgery patients. The median reported age was 40 years (IQR = 29-49 years) and 100 (32.7%) were female. Of the patients analysed, 17.3% were treated non-electively and 68.6% of them received concomitant valve procedure. The in-hospital mortality was 2.0%. Reoperation for bleeding was required in 8.2% of patients and 1.3% of them suffered a cerebrovascular accident (CVA). Mortality at 1 year was reported as 5.5%. Conclusion: The outcomes of surgery on the root and ascending aorta in Marfan patients in the United Kingdom are satisfactory; however, the overall complexities of this patient population are not well understood and would benefit from further investigations.

Marfan syndrome, aortic dissection, and pregnancy—the triple combination

“…Perhaps it would be encouraging to the authors that there is further research available which hold the same opinions, particularly when seeing improved results in mortality for valvular surgeries in those with Marfans. 2 Infact even some previous works have also corroborated with the safety of conception in Marfans provided the aortic root diameter remains under 45 mm. 3 However, whilst preconceptual counseling is paramount, in many cases this can be missed due to the syndrome not being diagnosed before the pregnancy, 4 and so having a prerequisite of early detection and following of any clinical guidelines would be vital in reducing the rate of adverse life-threatening results later on.…”

Aortic dissections in Marfan disease during pregnancy: Review article

“…surgery, and assesses outcomes. While it does not specifically address Marfans in pregnancy, it reported satisfactory outcomes for 306 Marfans patients, 100 of which were female, and suggests, as our paper does, that Aortic root surgery in this cohort remains an evolving but achievable challenge [1]. The call for an international registry to assess the work up and outcomes of this patient population is a worthy one which we would be happy to participate in.…”

Aortic Dissections in Marfan Disease During Pregnancy: Review Article

“…The recently released work by Dr. Harky identifies the Marfans population in England and Wales undergoing planned and non-planned Aortic root surgery, and assesses outcomes. While it does not specifically address Marfans in pregnancy, it reported satisfactory outcomes for 306 Marfans patients, 100 of which were female, and suggests, as our paper does, that Aortic root surgery in this cohort remains an evolving but achievable challenge [1]. The call for an international registry to assess the work up and outcomes of this patient population is a worthy one which we would be happy to participate in.…”