Surgical management of aortic root disease in Marfan syndrome and other congenital disorders associated with aortic root aneurysms

Elective root replacement in Marfan syndrome has improved life expectancy in affected patients. Three forms of surgery are now available: total root replacement (TRR) with a valved conduit, valve sparing root replacement (VSRR) and personalised external aortic root support (PEARS) with a macroporous mesh sleeve. TRR can be performed irrespective of aortic dimensions and a mechanical replacement valve is a secure and near certain means of correcting aortic valve regurgitation but has thromboembolic and bleeding risks. VSRR offers freedom from anticoagulation and attendant risks of bleeding but reoperation for aortic regurgitation runs at 1.3% per annum. A prospective multi-institutional study has found this to be an underestimate of the true rate of valve-related adverse events. PEARS conserves the aortic root anatomy and optimises the chance of maintaining valve function but average follow-up is under 5 years and so the long-term results are yet to be determined. Patients are on average in their 30s and so the cumulative lifetime need for reoperation, and of any valve-related complications, are consequently substantial. With lowering surgical risk of prophylactic root replacement, the threshold for intervention has reduced progressively over 30 years to 4.5 cm and so an increasing number of patients who are not destined to have a dissection are now having root replacement. In evaluation of these three forms of surgery, the number needed to treat to prevent dissection and the balance of net benefit and harm in future patients must be considered

A 'compare and contrast' exercise: wrapping versus personalised external aortic root support (PEARS)

Wrapping of the aorta and personalised external aortic root support (PEARS) both have the purpose of preventing further expansion of the ascending aorta in order to reduce the risk of aortic dissection and to spare the patient the disastrous consequences of aortic rupture. For the first time, Plonek and colleagues have reported systematically the CT appearances of a series of cases of wrapping. They illustrate the important finding that there are residual spaces between the aorta and the wrap. PEARS by contrast is intimately in contact with the aorta due to its personalised design and is fully incorporated due it construction from a porous mesh. A limitation of PEARS is that it is, of its nature, a planned and elective operation while wrapping can be undertaken during an emergency operation and can be used without prior planning as an intraoperative decision