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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

Cardiac magnetic resonance images. Dark blood spin-echo image (left) and a diastolic frame from complete cine acquisition (right) of severe aortic root dilatation of typical Marfan morphology with type A dissection and functional severe eccentric AR and LV dilatation. The aortic root diameter is 8 cm.
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HEARTJNL2013305132F3: Cardiac magnetic resonance images. Dark blood spin-echo image (left) and a diastolic frame from complete cine acquisition (right) of severe aortic root dilatation of typical Marfan morphology with type A dissection and functional severe eccentric AR and LV dilatation. The aortic root diameter is 8 cm.

Mentions: The most common form of aortic root aneurysm is that seen in Marfan syndrome. We recognise a characteristic morphology originating and predominately affecting the sinuses of Valsalva, in which the expansion of the aorta includes the wall of the sinuses proximal to the coronary orifices (figure 3). In current practice, this form of aneurysm is amenable to confident echo diagnosis.


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)

Cardiac magnetic resonance images. Dark blood spin-echo image (left) and a diastolic frame from complete cine acquisition (right) of severe aortic root dilatation of typical Marfan morphology with type A dissection and functional severe eccentric AR and LV dilatation. The aortic root diameter is 8 cm.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

HEARTJNL2013305132F3: Cardiac magnetic resonance images. Dark blood spin-echo image (left) and a diastolic frame from complete cine acquisition (right) of severe aortic root dilatation of typical Marfan morphology with type A dissection and functional severe eccentric AR and LV dilatation. The aortic root diameter is 8 cm.
Mentions: The most common form of aortic root aneurysm is that seen in Marfan syndrome. We recognise a characteristic morphology originating and predominately affecting the sinuses of Valsalva, in which the expansion of the aorta includes the wall of the sinuses proximal to the coronary orifices (figure 3). In current practice, this form of aneurysm is amenable to confident echo diagnosis.

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