The prospect of aortic surgery is a daunting one. But a clear, impartial understanding of the options and procedures can reduce the fear and anxiety. Here is our guide:
Aortic Surgery
At some point in the lives of many individuals with Marfan syndrome, aortic surgery will be required to keep them safe and prevent the life-threatening consequences of aortic dissection. Ideally this will be a planned operation which gives ample opportunity to talk to your surgeon and cardiologist. Every patient is different, and every aorta is different, there are also lots of different surgical techniques available which all have pros and cons.
Usually, people with Marfan or Loeys-Dietz syndrome will be under the care of a Cardiologist who will arrange their regular aortic surveillance scans and make sure they are on effective medications to try and reduce the rate of aortic dilatation. However, at some point surgery may be required and at this point your Cardiologist will refer you to one of their surgical colleagues. Cardiologists and Aortic surgeons work together in Aortic Teams and will still be available to answer questions you might have. After your surgery you may well be referred back to your Cardiologist for ongoing surveillance.
It can be very tempting to begin researching different surgical techniques on the internet, this can be a good way of finding out more about the options available and perhaps prompt questions you would like to ask when you see your surgeon. However, it is important to remember that not all operations are possible in all circumstances, and you need to see your surgeon with an open mind.
Any decision about surgery needs to be a collaboration between you and your surgeon, the basis of informed consent is that you have all the information required to make an informed decision. Your surgeon will make recommendations based on lots of different factors which include:
- Age
- Past medical history
- Your specific circumstances (are you a young woman who would still like to become pregnant)
- Previous surgery
- Aortic anatomy
- Heart function (including that of the heart valves)
These factors also allow your surgeon to give you detailed information about your risk of surgery and have a conversation about the potential risks vs. potential benefits of different surgeries.
Getting a Second Opinion
In the UK, you can ask your doctor for a second opinion and the vast majority will be happy to refer you to a colleague. The General Medical Council (GMC) states that all doctors should respect a patient’s right to seek a second opinion. Sometimes, a surgeon will proactively refer you to one of their colleagues if they feel that there is a treatment they do not offer that would be more suitable for you.
There are a few things to consider when seeking a second opinion:
- Be open and honest with your doctor, they will need to refer you on to a colleague and can provide all your medical records and test results that can save time
- You may receive the same advice from the second doctor
- Sometimes, asking additional questions of your doctor can negate the need for a second opinion. You can contact your surgeon via their secretary or via the specialist nurse team if there are things that are unclear. A simple explanation of the rationale for a surgeon’s recommendation can clear up misunderstanding.
- Getting a second opinion can delay treatment and in an urgent situation this may not always be feasible
- Aortic teams work collaboratively and will have regular multidisciplinary team (MDT) meetings to discuss surgical cases and decide upon the best options for treatment. These meetings bring together numerous specialists who will review complex cases and discuss the best, evidence based treatments for patients, taking into account their specific risk factors. Your surgeon may decide to refer your case to the MDT so they can discuss it with colleagues and ensure that you are being offered the right treatment for your specific situation. The results of any MDT discussion will always be communicated to you and your GP.
Below is a table with some of the operations that might be recommended to a patient with Marfan syndrome who requires intervention to their aorta.
Below is a table with some of the operations that might be recommended to a patientwith Marfan syndrome who requires intervention to their aorta.
Surgery
Description
Ascending Aorta Replacement
Depending on the location of the aortic aneurysm, your surgeon may need to replace other parts of the aorta including the ascending aorta (just above the aortic root) and parts of the aortic arch. Dacron grafts are used to replace the dilated portion of the aorta.
David Procedure
Valve sparing aortic root replacement, in this surgery the dilated portion of the aorta is removed and replaced with a Dacron graft (durable synthetic material). The patient’s own (native) aortic valve remains in place and the Dacron graft is stitched into place.
This type of operation would avoid the need for anticoagulation therapy with warfarin but is now suitable if the valve requires replacement.
Bentall Procedure
Aortic root replacement with valve replacement, in this surgery the aortic valve and the dilated portion of the aorta are removed. They are replaced with a Dacron graft (durable synthetic material) that has a mechanical valve already sewn onto one end. The graft is stitched into place by the surgeon.
This type of operation has been done for many years and the outcomes for patients are good. The mechanical valve replacement requires lifelong anticoagulation therapy (the patient must take warfarin to keep the blood slightly thinner and prevent clots forming on the valve).
Tissue Aortic Valve Replacement
Instead of using a mechanical aortic valve replacement a bioprosthetic valve can be implanted. This type of valve replacement does not require the patient to take anticoagulation therapy, but research has shown that bioprosthetic valve replacements may not last as long as mechanical valve replacement.
PEARS
Personalised external aortic root support. This is a relatively new type of surgery with the first procedure being done in 2004. Instead of removing the dilated portion of the aorta, a personalised 3D graft is made for each patient (using a specialised 3D CT scan), the graft is then ‘wrapped’ around the dilated aorta to hopefully prevent further dilatation.
Please get in touch via [email protected] if you have questions.
Disclaimer – we are unable to give individual recommendations, these need to come from your care provider who has access to all your medical reports and information.
We can help you understand tricky concepts, talk through the different types of operation that might be available and be a listening ear if you are feeling overwhelmed by the process.
