Limits...
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.

Show MeSH

Related in: MedlinePlus

Aortic roots with the characteristic morphology and distal displacement of the coronary orifices but without gross enlargement. The CT image (left) is a volume rendered image of an ECG-gated scan of the thoracic aorta. The CMR image (right) is a thin section from a 3D steady-state in free precession sequence acquired in the left anterior oblique projection.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4215278&req=5

HEARTJNL2013305132F4: Aortic roots with the characteristic morphology and distal displacement of the coronary orifices but without gross enlargement. The CT image (left) is a volume rendered image of an ECG-gated scan of the thoracic aorta. The CMR image (right) is a thin section from a 3D steady-state in free precession sequence acquired in the left anterior oblique projection.

Mentions: The shape of the aortic root merits close attention (figure 4). The portion of the aortic wall proximal to the coronary orifices is characteristically elongated. This is not seen in ascending aortic aneurysms associated with bicuspid aortic valve disease for example. The diameter at the level of closure of the aortic leaflets and its rate of change are the measurements used to make recommendations to the patient with respect to timing of aortic root surgery. Increasingly, the clinical diagnosis will be confirmed and augmented by knowledge of an individual's genome5 but this should not distract attention from the hazard represented by the aortic root disease. We argue here that patients with other aortopathies such as Ehlers–Danlos syndrome and Loeys–Dietz syndrome should generally be considered for aortic root surgery on similar imaging criteria, but in the latter perhaps at smaller size.


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 roots with the characteristic morphology and distal displacement of the coronary orifices but without gross enlargement. The CT image (left) is a volume rendered image of an ECG-gated scan of the thoracic aorta. The CMR image (right) is a thin section from a 3D steady-state in free precession sequence acquired in the left anterior oblique projection.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

HEARTJNL2013305132F4: Aortic roots with the characteristic morphology and distal displacement of the coronary orifices but without gross enlargement. The CT image (left) is a volume rendered image of an ECG-gated scan of the thoracic aorta. The CMR image (right) is a thin section from a 3D steady-state in free precession sequence acquired in the left anterior oblique projection.
Mentions: The shape of the aortic root merits close attention (figure 4). The portion of the aortic wall proximal to the coronary orifices is characteristically elongated. This is not seen in ascending aortic aneurysms associated with bicuspid aortic valve disease for example. The diameter at the level of closure of the aortic leaflets and its rate of change are the measurements used to make recommendations to the patient with respect to timing of aortic root surgery. Increasingly, the clinical diagnosis will be confirmed and augmented by knowledge of an individual's genome5 but this should not distract attention from the hazard represented by the aortic root disease. We argue here that patients with other aortopathies such as Ehlers–Danlos syndrome and Loeys–Dietz syndrome should generally be considered for aortic root surgery on similar imaging criteria, but in the latter perhaps at smaller size.

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