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

Treasure T, Takkenberg JJ, Pepper J - Heart (2014)

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

ABSTRACT
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.

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Related in: MedlinePlus

Aortic root diameter over a 20-year period in a woman with Marfan syndrome; the diameter continued to enlarge during a first pregnancy and thereafter to reach the accepted threshold of 45 mm.28 She requested referral for personalised external aortic root support (PEARS). Her aortic dimensions were reduced as was seen in eight of the first 10 PEARS operations.20
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HEARTJNL2013305132F6: Aortic root diameter over a 20-year period in a woman with Marfan syndrome; the diameter continued to enlarge during a first pregnancy and thereafter to reach the accepted threshold of 45 mm.28 She requested referral for personalised external aortic root support (PEARS). Her aortic dimensions were reduced as was seen in eight of the first 10 PEARS operations.20

Mentions: This swings the pendulum towards the ‘workmanship of certainty’: the surgeon is provided with a device which is made to measure for that individual's aorta and which will hold the aortic root at its existing shape and size. The process allows for a measured reduction in its diameter20 (figure 6). A formal evaluation of whether aortic regurgitation can be reduced or eliminated is yet to be done but has been noted anecdotally.


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

Treasure T, Takkenberg JJ, Pepper J - Heart (2014)

Aortic root diameter over a 20-year period in a woman with Marfan syndrome; the diameter continued to enlarge during a first pregnancy and thereafter to reach the accepted threshold of 45 mm.28 She requested referral for personalised external aortic root support (PEARS). Her aortic dimensions were reduced as was seen in eight of the first 10 PEARS operations.20
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4215278&req=5

HEARTJNL2013305132F6: Aortic root diameter over a 20-year period in a woman with Marfan syndrome; the diameter continued to enlarge during a first pregnancy and thereafter to reach the accepted threshold of 45 mm.28 She requested referral for personalised external aortic root support (PEARS). Her aortic dimensions were reduced as was seen in eight of the first 10 PEARS operations.20
Mentions: This swings the pendulum towards the ‘workmanship of certainty’: the surgeon is provided with a device which is made to measure for that individual's aorta and which will hold the aortic root at its existing shape and size. The process allows for a measured reduction in its diameter20 (figure 6). A formal evaluation of whether aortic regurgitation can be reduced or eliminated is yet to be done but has been noted anecdotally.

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

ABSTRACT
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.

Show MeSH
Related in: MedlinePlus