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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

Acute aortic dissection in a man in his 50s from a family with Marfan syndrome and a history of several dissections and root replacements. One of the kidneys is not being perfused.
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HEARTJNL2013305132F1: Acute aortic dissection in a man in his 50s from a family with Marfan syndrome and a history of several dissections and root replacements. One of the kidneys is not being perfused.

Mentions: Aortic dissection is by far the commonest cause of death in the natural history of Marfan syndrome and other genetically defined aortopathies (figure 1). When there is an aortic root aneurysm, prophylactic surgery on the aortic root intended to prevent aortic dissection is the most important life prolonging treatment for individuals with these conditions and will therefore be given the most attention within the limitations of this clinical review.


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)

Acute aortic dissection in a man in his 50s from a family with Marfan syndrome and a history of several dissections and root replacements. One of the kidneys is not being perfused.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

HEARTJNL2013305132F1: Acute aortic dissection in a man in his 50s from a family with Marfan syndrome and a history of several dissections and root replacements. One of the kidneys is not being perfused.
Mentions: Aortic dissection is by far the commonest cause of death in the natural history of Marfan syndrome and other genetically defined aortopathies (figure 1). When there is an aortic root aneurysm, prophylactic surgery on the aortic root intended to prevent aortic dissection is the most important life prolonging treatment for individuals with these conditions and will therefore be given the most attention within the limitations of this clinical review.

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