Preparing for Surgery

surgeons

People with Loeys-Dietz syndrome (LDS) may need to undergo surgery. This page offers a description of several types of surgery and information on how to prepare for surgery as a patient and parent.

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What is Vascular Surgery?

Vascular surgery is performed in individuals with LDS to repair circulatory system manifestations.

It is widely recommended as a preventative surgery for LDS patients with: 

  • Rapidly enlarging arteries (blood vessels that carry blood from the heart to the rest of the body)
  • Rapidly enlarging aorta (a candy cane-shaped artery that starts at the top of the heart and ends in the abdomen)
  • Pronounced family history of arterial dissection

As vascular tissue is not especially weak or frail in people with LDS, there are many cases of successful aneurysmal repair using a range of surgical techniques.

Preventative or Prophylactic Vascular Surgery

Preventative or prophylactic vascular surgery aims to prevent arterial dilations/aneurysms (abnormal bulges in arteries) from growing larger and dissecting (growing large enough to tear the artery).

Vascular surgery is also performed in emergency situations, such as in the case of arterial dissections. 

Type A aortic dissection takes place in the ascending aorta, the section of aorta between the heart and aortic arch, but can also extend to the arch or descending aorta.

Type B aortic dissection occurs only in the descending aorta, located between the arch and abdomen.

Timing of Preventative Surgery

The timing of preventative surgery depends on factors including aneurysm size and growth rate, arterial function, and family history.

For aortic root aneurysms in children, surgery is generally considered once the aortic root exceeds 1.8-2.0 cm and the 99th percentile, or once the Z-score is greater than 3.5.

In adolescents and adults, surgery is generally recommended once the maximum aortic root dimension reaches 4.0 cm. As LDS affects each individual differently, the surgical timing must be determined by a medical professional on a case-by-case basis.

Minimally Invasive or Open Surgery

Surgery may be minimally invasive or open.

Minimally invasive means that surgeons gain access to the inside of the body through one or several small incisions and use these openings to visualize and access an area of interest.

In comparison, open surgery uses large incisions to directly view and access the area. 

Types of Vascular Surgery

Aortic root replacement (ARR) is also known as the Bentall procedure. This surgery treats aneurysms (abnormal bulges) in the section of the aorta attached to the heart, the aortic root. The aortic root is replaced with a graft (synthetic tube) and the aortic valve is replaced with a biological or mechanical valve. A biological valve consists of human or animal tissue while a mechanical valve is made of metal or carbon.  

Like aortic root replacement surgery, valve-sparing aortic root repair/replacement (VSRR) treats aneurysms (abnormal bulges) in the section of the aorta attached to the heart, the aortic root, and replaces the aortic root with a graft (synthetic tube). However, in VSSR, the aortic valve is not replaced. There are two types of VSRR: the Yacoub procedure and the David procedure.

Endovascular aneurysm repair (EVAR) is a minimally invasive surgery used to repair aneurysms (abnormal bulges) in the abdominal portion of the aorta. An artificial tube (graft) is inserted through an artery in the groin, guided through the body, and placed at the site of the aneurysm. The graft creates a new lining for the artery and helps prevent a rupture or dissection. 

Thoracic endovascular aortic repair (TEVAR) is a minimally invasive surgery to repair aneurysms (abnormal bulges) in the thoracic (chest) portion of the aorta. Like in EVAR, an artificial tube (graft) is inserted through an artery in the groin, guided through the body, and placed at the site of the aneurysm. The graft creates a new lining for the artery and helps prevent a rupture or dissection. 

The Florida (FL) sleeve procedure is used to treat aneurysms (abnormal bulges) in the section of the aorta attached to the heart, the aortic root. A graft (synthetic tube) is placed over the aortic root like a sleeve and the aortic valve is not replaced. This surgery is viewed as a simpler alternative to other valve-sparing procedures such as the Yacoub procedure and the David procedure.

Bicuspid aortic valve is a birth defect that can present in LDS patients. The aortic valve is a valve separating heart from the aorta, the body's largest artery. The cusps (leaflets) on the valve open and close with each heartbeat to help blood flow from the heart to the rest of the body. Usually, the aortic valve has three cusps while a bicuspid valve only has two cusps

 

By reshaping the aortic valve leaflets, a bicuspid aortic valve can be repaired so that it can open and close more completely. Bicuspid aortic valve repair procedures include a minimally invasive “J” incision approach, aortic valve replacement, ascending aorta surgery, or balloon valvuloplasty. 

Personalized external aortic root support (PEARS) treats aneurysms (abnormal bulges) in the ascending aorta, the portion of the aorta between the heart and aortic arch where blood is travelling upwards. With PEARS, a 3D model of the patient’s aorta is used to create a tailored 3D mesh that is soft and supportive and surgically placed over the aorta. This mesh is softer and more personalized than grafts used in other procedures. Like the Florida (FL) sleeve procedure and valve-sparing aortic root repair/replacement, PEARS is valve-sparing. 

What is Orthopedic Surgery?

Orthopedic surgery is performed in individuals with LDS to repair skeletal system manifestations. 

Types of Orthopedic Surgery

Clubfoot is a birth defect and common orthopedic feature of Loeys-Dietz syndrome (LDS). One or both feet may be affected and appear smaller than normal, turned inward, and have a heel that looks pulled upwards. Clubfoot can be corrected by casting and stretching or surgery.

 

Clubfoot surgery helps to move the foot into a normal position by releasing and lengthening tight ligaments and tendons, and by working on foot bones and joints to promote proper bone growth. Small pins may be used during surgery, and post-surgical treatment can involve pin removal, casting, bracing, and follow-up appointments. 

People with LDS may have cartilage between their ribs and sternum (breastbone) that grows more than average, causing the sternum to push out (pectus carinatum) or push in (pectus excavatum). Surgery is rarely medically necessary, and is typically done for cosmetic purposes during late childhood or puberty.

 

The Modified Ravitch Procedure is a surgery that can correct pectus carinatum and excavatum by opening the chest, removing misshapen cartilage, and repositioning the sternum.

 

Another surgery, the Nuss procedure, can treat pectus excavatum by placing a curved metal bar under the sternum and turning it to push the sternum outwards, into a proper position. 

How to Prepare for Surgery

When preparing for surgery with Loeys-Dietz syndrome, it is important to consider your travel, recovery in the hospital and at home, physical and mental health, as well as how to manage daily life like work and school, children, finances, and household tasks. 

While these are general tips, each patient with LDS is unique. The surgeon and medical team will be able to best explain how to prepare and what to expect.

 

Travel
  • If surgery will take place away from home, think through travel arrangements for before and after surgery      
  • Car rides after heart surgery may be uncomfortable and it is recommended to use a chest pillow and seat belt cover
  • Patient may not be able to drive for a while, as big movements such as turning the wheel, breaking, and wearing a seat belt may be counterproductive to healing
  • Learn more about traveling with LDS
Hospital Stay
  • Bring blankets, pillows, stuffed animals, or anything that will make the patient feel comfortable
  • Plan out a schedule beforehand of who will sleep at the hospital and when
  • Figure out who can bring meals to the hospital
Recovery at Home
  • Consider any special equipment that might be necessary for post-surgery recovery (e.g., a cane, a chair for the shower, a hospital bed, wedge/chest pillow, etc)
  • Will you have any medical help available during recovery (for things like changing dressings) or will that be taken care of by yourself? 
  • Can the patient complete physical tasks, such as climbing stairs, after surgery? 
  • Where will the patient spend time after their surgery when they are home? In the TV room? Is there a comfortable chair set up? 
  • Try to minimize stress as much as possible in the home   
  • Tidy up before surgery and create a clean environment for the patient and family members

Nutrition
  • Consider implementing better nutritional practices to improve overall health or, if necessary, to gain or lose weight 
  • After certain types of surgery, the patient may have a reduced appetite. This should go away after a couple of weeks
  • It is important to eat and drink enough protein, calories, and water
Exercise
  • Consider exercise programs to improve health, as movement helps to prevent muscle weakness. This will help strengthen the body and help with post-op recovery
  • Talk to medical professionals about what exercises are limited post-surgery
  • Recovery after open-heart surgery could take up to a year, even if the patient is able to return to regular activities
Mental Health
  • After open-heart surgery, many people experience personality and mood changes. Common expressed emotions are depression, fatigue, and anxiety which could be due to being on bypass, anesthesia, or medication such as oxycontin
  • Consider seeing a mental health professional in advance and after to discuss coping strategies
  • To find a mental health professional near you, visit a general or province-specific directory.   
Sleep
  • Sleeping can be difficult after surgery as it can be hard to find a comfortable position to sleep in and anesthesia may disturb the circadian rhythm. A lack of sleep can lead to other problems, including brain fog and irritability
  • Laying flat after open-heart surgery can be painful; the patient may have to sit upright to sleep for a few days or a week
  • Using a chest pillow to sleep may be recommended after surgery
Pain Management Strategy
  • Work with the surgical team before surgery to determine what pain management options are available and what the alternatives are if the initial strategy does not work well
  • Collarbone or sternum (chest wall) pain may be experienced after open-heart surgery, but cardiac rehab or physical therapy can help 
  • Beware of risk of addiction to certain pain medication
Constipation
  • Ensure patient has a prescription to deal with constipation (such as Colace) and that they drink plenty of liquids
Miscellaneous
  • Hormonal changes can occur after surgery. For women, their period may become irregular, heavier, lighter, or more painful 
  • Open-heart surgery can cause hair loss. Taking supplements such as biotin or zinc may be helpful, but it is important to speak to a doctor first
  • Likely no swimming for 3 months after surgery

Work
  • Arrange time off and tie up loose ends at the office
  • The average time to get back to work depends on the physical demands of the job. For example, a desk job worker is likely to return faster than a construction worker. Some patients can start back at work part-time, and slowly go back to work full-time
  • Average time back to work is 2-3 months after operation
School
  • Students can inform teachers/professors of surgery in advance so they can coordinate schoolwork for when the patient is ready to return
Child care
  • Organize child care for a patient’s children or siblings
Financial
  • Pay all bills in advance of surgery to free the mind for post-surgery recovery
  • Add trusted family member to bank accounts so they can pay bills if needed
Meals
  • Eat a substantial meal before surgery
  • Arrange for family and friends to bring food to the house after discharge from the hospital
  • Prepare food in advance to keep in freezer
Communication
  • Hold a family meeting to discuss what's coming up and how everyone can positively impact recovery. Assign roles and responsibilities 
Information Review
  • Review materials provided by surgeon before the pre-surgery meeting
  • Prepare a list of questions for the surgical team

Advice on Preparing for Surgery from People with LDS

Aalaei-Andabili, S. H., Martin, T., Hess, P., Klodell, C., Karimi, A., Arnaoutakis, G., Lee, T., & Beaver, T. (2017). Florida sleeve procedure is durable and improves aortic valve function in Marfan syndrome patients. The Annals of Thoracic Surgery104(3), 834–839. https://doi.org/10.1016/j.athoracsur.2017.01.044 


Aalaei-Andabili, S. H., Martin, T. D., Hess, P. J., Karimi, A., Bavry, A. A., Arnaoutakis, G. J., & Beaver, T. M. (2019). The Florida sleeve procedure is durable and improves aortic valve function. AORTA07(02), 049–055. https://doi.org/10.1055/s-0039-1687854 

 

Alhussaini, M., Jeng, E. I., Martin, T. D., Fillion, A., Beaver, T. M., & Arnaoutakis, G. J. (2021). Florida sleeve is a safe and effective technique for valve salvage in acute Stanford Type A aortic dissection. Journal of Cardiac Surgery37(1), 39–46. https://doi.org/10.1111/jocs.16078 

 

American Academy of Orthopaedic Surgeons. (2019, January). Clubfoot - orthoinfo - Aaos. OrthoInfo. Retrieved November 9, 2022, from https://orthoinfo.aaos.org/en/diseases--conditions/clubfoot/ 

 

Badin, D., Atwater, L. C., Dietz, H. C., & Sponseller, P. D. (2022). Talipes Equinovarus in Loeys-Dietz syndrome. Journal of Pediatric Orthopaedics42(7), 777–782. https://doi.org/10.1097/bpo.0000000000002180 

 

Blackwater, A. (2021, July 29). 11 things no one warns you about after open-heart surgery. The Mighty. Retrieved November 9, 2022, from https://themighty.com/topic/congenital-heart-defect-disease/open-heart-surgery-recovery-what-to-know-chronic-illness 

 

British Heart Foundation. (n.d.). How do replacement heart valves work? British Heart Foundation. Retrieved November 9, 2022, from https://www.bhf.org.uk/informationsupport/heart-matters-magazine/medical/replacement-heart-valves 

 

Cleveland Clinic. (2019, August 14). Aortic valve surgery. Cleveland Clinic. Retrieved November 9, 2022, from https://my.clevelandclinic.org/health/treatments/16745-aortic-valve-surgery 

 

Cleveland Clinic. (2022, March 13). Endovascular Aneurysm Repair (EVAR). Cleveland Clinic.

Retrieved November 9, 2022, from https://my.clevelandclinic.org/health/treatments/22291-endovascular-aneurysm-repair 

 

Cleveland Clinic. (2019, January 21). Pectus Carinatum. Cleveland Clinic. Retrieved November 9, 2022, from https://my.clevelandclinic.org/health/diseases/15790-pectus-carinatum 

 

Cardiovascular and Interventional Radiological Society of Europe. (2018, October 3). Thoracic endovascular aortic repair (tevar). Cardiovascular and Interventional Radiological Society of Europe. Retrieved November 9, 2022, from https://www.cirse.org/patients/ir-procedures/thoracic-endovascular-aortic-repair-tevar/ 

 

Dhannapuneni, R. (2021, January). Pectus Carinatum Repair. Alder Hey Children's NHS Foundation Trust. Retrieved November 9, 2022, from https://alderhey.nhs.uk/application/files/2115/8350/5884/Pectus_Carinatum_Leaflet_PIAG_212.pdf 

 

Freeman, K. A., Beaver, T. M., Jeng, E. I., Martin, T. D., & Arnaoutakis, G. J. (2021). Florida sleeve for type A dissection. Journal of Visualized Surgery7, 38–38. https://doi.org/10.21037/jovs-20-75 

 

Iqbal, R., Alom, S., BinSaeid, J., & Harky, A. (2020). Loeys–Dietz syndrome pathology and aspects of Cardiovascular Management: A systematic review. Vascular29(1), 3–14. https://doi.org/10.1177/1708538120934582 

 

Intermountain Healthcare. (n.d.). Aortic Root Replacement. Intermountain Healthcare. Retrieved November 9, 2022, from https://intermountainhealthcare.org/services/heart-care/treatment-and-detection-methods/aortic-root-replacement/ 

 

Johns Hopkins Medicine. (2019, November 19). Endovascular repair of an abdominal aortic aneurysm. Johns Hopkins Medicine. Retrieved November 9, 2022, from https://www.hopkinsmedicine.org/health/conditions-and-diseases/abdominal-aortic-aneurysm/endovascular-repair-of-an-abdominal-aortic-aneurysm 

 

Johns Hopkins Medicine. (2020, July 20). Loeys-Dietz syndrome. Johns Hopkins Medicine. Retrieved November 9, 2022, from https://www.hopkinsmedicine.org/health/conditions-and-diseases/loeysdietz-syndrome 

 

Loeys, B. L., & Dietz, H. C. (2018, March 1). Loeys-Dietz Syndrome. GeneReviews® [Internet]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1133/

 

Loeys-Dietz Syndrome Foundation. (2019). Preparing For Cardiothoracic Surgery. Loeys-Dietz Syndrome Foundation. Loeys-Dietz Syndrome Foundation. Retrieved November 9, 2022, from https://static1.squarespace.com/static/5be355670dbda39d591a525e/t/5d0fd53ea71ad0000109c690/1561318721293/CVS+Resource_Adult_May+2019.pdf. 

Loeys-Dietz Syndrome Foundation Canada. (2022, October 27). Treatment options loeys-dietz syndrome - LDSFC. Loeys-Dietz Syndrome Foundation Canada. Retrieved November 9, 2022, from https://loeysdietzcanada.org/treatment/ 

 

Massachusetts General Hospital. (n.d.). Clubfoot. Massachusetts General Hospital. Retrieved November 9, 2022, from https://www.massgeneral.org/orthopaedics/children/conditions-and-treatments/clubfoot 

 

Mayo Foundation for Medical Education and Research. (2021, July 30). Aortic root surgery. Mayo Clinic. Retrieved November 9, 2022, from https://www.mayoclinic.org/tests-procedures/aortic-root-surgery/about/pac-20383396 

 

Mayo Foundation for Medical Education and Research. (2022, April 21). Congenital heart disease in adults. Mayo Clinic. Retrieved November 9, 2022, from https://www.mayoclinic.org/diseases-conditions/bicuspid-aortic-valve/cdc-20385577 

 

Simion, F. (n.d.). Endovascular Aneurysm Repair (EVAR). Circulation Foundation. Retrieved November 9, 2022, from https://www.circulationfoundation.org.uk/help-advice/abdominal-aortic-aneurysm/endovascular-aneurysm-repair-evar 

 

The State of Queensland (Metro North Health). (2021, August 20). Pears marks sweet milestone. Metro North Health. Retrieved November 9, 2022, from https://metronorth.health.qld.gov.au/news/pears-marks-sweet-milestone 

 

UCSF Department of Surgery. (n.d.). Endovascular Aneurysm Repair. UCSF Department of Surgery. Retrieved November 9, 2022, from https://surgery.ucsf.edu/conditions--procedures/endovascular-aneurysm-repair.aspx 

 

UCSF Department of Surgery. (n.d.). Pectus Carinatum. Department of Surgery. Retrieved November 9, 2022, from https://surgery.ucsf.edu/conditions--procedures/pectus-carinatum.aspx 

 

U.S. National Library of Medicine. (2020, April 1). Loeys-Dietz Syndrome: Medlineplus Genetics. MedlinePlus. Retrieved November 9, 2022, from https://medlineplus.gov/genetics/condition/loeys-dietz-syndrome/ 

 

VASCERN ERN. (2022). Before and After Aortic Surgery: Q&A for Marfan PatientsYouTube. Retrieved November 9, 2022, from https://www.youtube.com/watch?v=7ObVyvPbikY&t=986s. 

 

Wheatley, G. (2017, April 9). Pears for your aorta: Not just another fruity idea. BadAorta. Retrieved November 9, 2022, from https://badaorta.com/pears-for-your-aorta-not-just-another-fruity-idea/ 

 

Yale Medicine. (2022, September 24). Minimally invasive surgery. Yale Medicine. Retrieved November 9, 2022, from https://www.yalemedicine.org/conditions/minimally-invasive-surgery 

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