Endovascular Repair in Marfan Patients
At the bottom of the page, you'll find a glossary where highlighted words are explained in more detail.
Due to a mutation in the fibrillin-1 gene, individuals with Marfan Syndrome (MFS) have a fragile aorta. This can lead to the occurrence of aortic dissections (Fig 1) and rupture. Normally, patients would undergo open heart surgery. However, younger patients often do not present with many complications related to thoracoabdominal aortic aneurysm (TAAA), therefore open heart surgery bears a lot of risks for this patient group.
Thoracic endovascular aortic repair (TEVAR)
Endovascular aortic repair is widely used to treat aortic dissections. Thoracic endovascular aortic repair (TEVAR) (Fig 2) has fewer short-term complications compared to open surgery, especially when it comes to older patients who tend to have more health problems. However, this procedure is less durable than open surgery. Late complications of TEVAR include: endoleak, rapid aortic expansion (uncommon), stent-graft induced new entry, and retrograde type A aortic dissection.
For MFS patients, the utility of endovascular aortic repair is unclear. TEVAR is generally superior to open surgery when it comes to short-term complications. However, it is less durable and reintervention rates are highest in patients with chronic aortic dissection (MFS patients included). For this reason, along with the tendency for younger patients, durability of the procedure is a very important factor to consider. That is why MFS patients seeking definitive aortic repair are suggested open repair surgeries.
Can TEVAR be incorporated into the standard open surgery repair or be used to improve operational risks?
Yes, it may be a possibility. TEVAR can:
- be used as a bridge to aid open repair in patients with MFS;
- be used in emergency situations, TEVAR can be used to help stabilise patients until elective repair can be performed;
- be considered for treatment for late complications that arise from previous multiple aortic reoperations.
Want to know more about TEVAR?
Here are some short visual animations to show how it works:
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Check out the concept list
Aortic dissection | An aortic dissection, or tear in the aorta, typically occurs when the inner layer of the artery's wall weakens. If left untreated, the tear can enlarge. When this happens, blood can pass through the tear into the middle layer of the wall, causing the layers to separate from one another, or dissect.
Chronic aortic dissection | If the symptoms or dissection occur within the last 14 days, the dissection is termed acute aortic dissection. If the symptoms or dissection occur after the 14th day, the dissection is termed chronic aortic dissection.
Complications | A secondary disease or condition aggravating an already existing one
Elective repair | Elective surgery or elective procedure is surgery that is scheduled in advance because it does not involve a medical emergency.
Endoleak | Persistent blood flow between the excluded aneurysm sac and the stent graft.
Fibrillin-1 gene | The gene with the code for the fibrillin protein. The fibrillin protein is a specific protein that is used to built elastic fibres and found in connective tissue
Mutation | A change in the DNA sequence that can alter the structure or function of a gene, potentially leading to disease, disorders and syndromes
Open surgery | Cutting the skin and tissues so that the surgeon has a full view of the structures or organs involved.
Reintervention rates | Secondary procedures. Defined as any endovascular or surgical procedure done after the first intervention.
Reoperations | A duplication or repetition of a surgical operation, usually undertaken due to lack of success of the first attempt
Retrograde | Reverting to an earlier and inferior condition.
Retrograde type A aortic dissection (RTAAD) | When a type B aortic dissection (descending part) is treated with TEVAR, it may be possible that it extends the dissection into the upper aorta. For patients who do not have a dissection, a SINE tear might develop and extend towards the upper aorta. Regardless of how, this is a life-threatening event and must be treated by open surgery; furthermore, it can occur during the procedure or months to years later.
SINE (stent-graft induced new entry) tears | Caused by the stent graft itself. The most important risk factor is fitting an oversized stent graft in the landing zone in the aortic tissue; this is especially a problem for Marfan patients, who have an inherent weakness of the aortic wall
Thoracic endovascular aortic repair (TEVAR) | A minimally invasive procedure used to repair a thoracic aortic aneurysm or dissection.
Thoracoabdominal aortic aneurysm (TAAA) | Type of aortic aneurysm, a condition in which the wall of the aorta, the largest blood vessel in the body, weakens and bulges.
Are you curious about the scientific publication used to write this article? Check out the original study!
Steinmetz LM, Coselli JS. Endovascular repair in patients with Marfan Syndrome: Concerns amid controversy. Annals of Vascular Surgery. 2023 Aug;94:1–7.