DR IMRAN JAVED,

MBBS, FCPS.

INTERNATIONAL FELLOW

DR IMRAN JAVED,

MBBS, FCPS.

INTERNATIONAL FELLOW

Manufacturing and placing a bespoke support for the

Marfan aortic root

Manufacturing and placing a bespoke support for the

Marfan aortic root

INTRODUCTIONINTRODUCTIONDissection of the ascending aorta is a

characteristic manifestation of Marfan syndrome.

It is the commonest cause of death, killing as many as 70%, often in their twenties or thirties.

The strongest predictors of likelihood of dissection are aortic root dimension and its rate of change.

Dissection of the ascending aorta is a characteristic manifestation of Marfan syndrome.

It is the commonest cause of death, killing as many as 70%, often in their twenties or thirties.

The strongest predictors of likelihood of dissection are aortic root dimension and its rate of change.

TREATMENT TREATMENT Currently,

prevention relies on replacing the ascending aorta before it dissects and ruptures.

Currently, prevention relies on replacing the ascending aorta before it dissects and ruptures.

PITFALLSPITFALLSIf a mechanical valve is incorporated the patient is committed to life-long anticoagulation. Valve sparing operations have been devised and modified but these remain exacting to perform and prone to failure. Bioprosthetic replacement is a less technically exacting means of reducing thromboembolic risk but at the price of likely eventual valve failure with the need for reoperation.

If a mechanical valve is incorporated the patient is committed to life-long anticoagulation. Valve sparing operations have been devised and modified but these remain exacting to perform and prone to failure. Bioprosthetic replacement is a less technically exacting means of reducing thromboembolic risk but at the price of likely eventual valve failure with the need for reoperation.

INNOVATIONINNOVATIONThe development of

a bespoke external aortic root support (EARS) using imaging and computer aided design to fit the individual patient's ascending aorta has been achieved

The development of a bespoke external aortic root support (EARS) using imaging and computer aided design to fit the individual patient's ascending aorta has been achieved

SURGICAL PRODECURESURGICAL PRODECUREThe chest was opened through median

sternotomy.

The aorta was completely dissected from the aorto-ventricular junction to the origin of the brachiocephalic artery.

Cardio-pulmonary bypass was available.

The EARS was brought to the operating table on the former, a model of the patient's own aorta.

The chest was opened through median sternotomy.

The aorta was completely dissected from the aorto-ventricular junction to the origin of the brachiocephalic artery.

Cardio-pulmonary bypass was available.

The EARS was brought to the operating table on the former, a model of the patient's own aorta.

TECHNICAL DETAILSTECHNICAL DETAILSThe

longitudinal seam at the front is opened.

The

longitudinal seam at the front is opened.

TECHNICAL DETAILSTECHNICAL DETAILSThe support is placed around the

aorta.

Note that the material extends proximal to the coronary arteries to the aorto-ventricular junction.

It is engineered to have high hoop strength preventing ‘annular’ dilatation.

The support is placed around the aorta.

Note that the material extends proximal to the coronary arteries to the aorto-ventricular junction.

It is engineered to have high hoop strength preventing ‘annular’ dilatation.

Copyright ©2010 The European Association for Cardio-thoracic Surgery

MRI image at the level of the aortic valve closure with aortic

diameters measured by the radiologist superposed along with

the traced outline

MRI image at the level of the aortic valve closure with aortic

diameters measured by the radiologist superposed along with

the traced outline

Diagnosis & Follow upDiagnosis & Follow upMRI image shows a cross-section of the aorta at

the level of closure of the aortic valve cusps.

It ensures that the levels are comparable within each patient and at the same point in the cardiac cycle (diastole).

An image is selected of the aortic arch to show a maximum transverse diameter at diastole because a frequent question is whether supporting the ascending aorta shifts the point of maximal stress to beyond the supported segment.

MRI image shows a cross-section of the aorta at the level of closure of the aortic valve cusps.

It ensures that the levels are comparable within each patient and at the same point in the cardiac cycle (diastole).

An image is selected of the aortic arch to show a maximum transverse diameter at diastole because a frequent question is whether supporting the ascending aorta shifts the point of maximal stress to beyond the supported segment.

CRITERIA FOR AORTIC ROOT REPLACEMENT

CRITERIA FOR AORTIC ROOT REPLACEMENT

Prophylactic aortic root replacement is recommended to patients with MFS when the root size reaches 50 mm.

The cumulative risk for dissection or rupture increases four times when the aneurysm size exceeds 60 mm.

Prophylactic surgery is advised when the aorta is less than 50 mm for patients with rapid aneurysm growth, family history of early dissection or sudden death, and moderate-to-severe aortic insufficiency.

Prophylactic aortic root replacement is recommended to patients with MFS when the root size reaches 50 mm.

The cumulative risk for dissection or rupture increases four times when the aneurysm size exceeds 60 mm.

Prophylactic surgery is advised when the aorta is less than 50 mm for patients with rapid aneurysm growth, family history of early dissection or sudden death, and moderate-to-severe aortic insufficiency.

ENDOVASCULAR APPROACHENDOVASCULAR APPROACHQUESTION OF THE DAY????

OPINION OF EXPERTS.

ONLY CASE REPORTS ARE AVAILABLE IN LITERATURE FOR TYPE A DISSECTIONS.

QUESTION OF THE DAY????

OPINION OF EXPERTS.

ONLY CASE REPORTS ARE AVAILABLE IN LITERATURE FOR TYPE A DISSECTIONS.