Time to Rethink about Prophylactic Aortic Root Surgery in Patients with Marfan's Syndrome

Should We Move Up the Clock on Preventive Intervention in Marfan's Disease?

In a Marfan patient without risk factors, guidelines, updated once in a while, express a relatively conservative attitude with regard to the maximum ascending aortic diameter limit, so far generally accepted up to 5 cm.[1] However, the prospect of a dilating ascending aorta remains a matter of concern and therefore a “moving target” for geneticists, physicians, and especially for surgeons. Driven by (bad) experience, some of our pioneers sought very early for reasonable prophylactic surgery, not infrequently ending up in truly unnecessary aortic valve replacement. Only one of them (at least in the seventies of the last century) suggested (and still suggests!) a kind of less radical intervention by external reinforcement of the ascending aorta accompanied by valve replacement or valve preservation.[2] Here, Francis Robicsek again underlines his early quest, reasoned by own experience, reaching back almost 50 years now.[3] To those who believe that this suggestion is outdated in times of advanced valve-preserving ascending aortic replacement surgery, listen and read carefully! The tailors of Gloucester around John Pepper and Tom Treasure, together with the “PEARS” (Personalized External Aortic Root Support) investigators, whistle a similar tune, remastered by the aid of recent three-dimensional printing technology. They concluded a couple of weeks ago that PEARS is effective in stabilizing the aortic root and preventing its dilatation.[4] It may be anticipated that Dr. Robicsek is a strong supporter of PEARS as he gives the advice to reinforce the ascending aorta of a patient with Marfan's syndrome once it reaches a diameter of 3.5 cm.

Therefore, what shall we do with PEARS and Robicsek's plea? Keep on waiting from 3.5 cm to at least 4.5 cm and perform an elective David operation with zero mortality[5]? Travel to the United Kingdom and adopt PEARS? Refer the patient to the United Kingdom? Not so easy (not only because of the Brexit)! It is always right, however, to sit with your patient and talk. Talk about diameters and respective risks. Talk about guidelines and elective, valve-preserving surgery. Then you will get an idea of the patient's perception of his/her ascending aorta. If he/she is fine with the current guidelines, you may be too. If the discussion drifts toward PEARS, be open and consider the option(s)! Why not?

• References

• 1 Erbel R, Aboyans V, Boileau C. , et al; ESC Committee for Practice Guidelines; The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. Eur Heart J 2014; 35 (41) 2873-2926
• 2 Robicsek F, Daugherty HK, Mullen DC. External grafting of aortic aneurysms. J Thorac Cardiovasc Surg 1971; 61 (01) 131-134
• 3 Robicsek F. Should we move up the clock on preventive intervention in Marfan’s disease?. J Thorac Cardiovasc Surg 2019. doi: 10.1055/s-0039-1678680
• 4 Izgi C, Newsome S, Alpendurada F. , et al. External aortic root support to prevent aortic dilatation in patients with Marfan syndrome. J Am Coll Cardiol 2018; 72 (10) 1095-1105
• 5 Kallenbach K, Baraki H, Khaladj N. , et al. Aortic valve-sparing operation in Marfan syndrome: what do we know after a decade?. Ann Thorac Surg 2007; 83 (02) S764-S768 , discussion S785–S790