Know the Signs, Fight for Victory

As a service to families with Marfan, VEDS, Loeys-Dietz and related conditions, we have assembled a list of academic medical centers at leading institutions that have shown interest and have experience and expertise in treating people with these conditions. The Institution Directory provides detailed information about each of the sites to help you make more informed decisions about the care you receive. You are responsible for interviewing and selecting the practitioner. The institutions listed here vary greatly in size, staff training, and services offered. We are happy to discuss options with you for your specific situation, if needed. Information on healthcare professionals is supplied solely by the medical centers themselves. All information is also self-reported and unverified. The Foundation does not endorse or recommend individual healthcare professionals or hospitals.

Some of the centers in the directory are tertiary care centers which means they provide highly specialized medical care. This can encompass an extended period of care that involves advanced and complex procedures and treatments performed by medical specialists in state-of-the-art facilities. The Foundation also encourages sites to provide quality overall care not only for the heart and blood vessels, but also for the multiple aspects of these conditions such as eyes, bone and joint, lung, gastrointestinal, and pain issues which can affect an individual’s quality of life.

Some sites consider themselves a connective tissue clinic, which can service many different conditions, including bone and skin conditions, skeletal dysplasias, etc. Others consider themselves aortic centers and concentrate on conditions that affect the aorta and heart. The list only includes sites that are affiliated with a university or medical hospital; private physician practices are not included.

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Questions to Ask When Evaluating Potential Medical Centers

  • Step 1 Why is it important to find an experienced center?

    Receiving care from a knowledgeable and experienced site is recommended for the following reasons:
    • Diagnosis – Sites that see more patients are better positioned to provide a proper diagnosis, which is critical for getting proper treatment. Genetic testing results should be reviewed by geneticists who are experienced with these conditions. Determining whether or not a mutation is a causative gene can require searching several databases because there is not one single database that covers all the mutations. Geneticists knowledgeable about Marfan, VEDS, LDS, and related conditions do this on a regular basis when reviewing reports from genetic laboratories.
    • Aortic Surgery – Surgical experience in patients with connective tissue conditions is critical for aortic root surgeries. It is especially important when a patient has to undergo a re-operation, which may present greater complications. Many surgeons are trained in traditional composite graft surgery (CVG) where mechanical valves replace the aortic valve. Fewer surgeons are trained in aortic valve-sparing surgeries that eliminate the need for life-long blood thinners. Additionally, due to the limited number of patients that undergo aortic valve-sparing procedures, many surgeons do not have a lot of experience in these surgeries. A surgeon who does 8-12 of these surgeries per year can be considered highly experienced. It should be noted that, if a center does not manage a lot of people with Marfan, VEDS, LDS, and related conditions, the surgical experience will be less.
    • Emergency Situations – Sites that have greater expertise in connective tissue conditions and aortic conditions may be better equipped to handle emergency situations. Your site should provide you with a special number to alert staff of an emergency. They should also have an emergency protocol in place. Your medical records should have a medical alert about the risks of these genetic aortic conditions. A Patient Care Coordination note or an FYI Alert signals medical professionals about the urgency to rule out emergency situations like arterial dissection, pneumothorax, or colon rupture. The note should appear in bright red and should be prominent on the upper right hand corner of the patient’s medical record home page.. Every patient should request to have this noted on their medical record. Emergency preparedness kits, emergency room information cards, and information about obtaining a medical alert bracelet are available.
    • Pregnancy – Access to a high-risk obstetrician and experienced cardiologist is needed to help ensure a safe and healthy delivery for both mother and child.

    The Foundation urges everyone living with one of these conditions to ask about specific training, volume, and special services the medical group has to offer. At times, a site might not have services all at the same physical location, but they should have collaborations with other healthcare professionals at different locations who address certain issues. The Institution Directory provides links to full applications with the names of healthcare professionals in different disciplines, if they are available.

    Many sites have websites that describe their services for people with Marfan, VEDS, LDS, and related conditions. Many healthcare professionals have a biography or curriculum vitae listing their education, training, and work experience. Websites can be helpful sources of additional information, but keep in mind that websites are a form of advertising. An impressive site may or may not reflect the level of care that is offered.

    We suggest that you look at all factors noted in the institution directory and choose a site that fits your specific needs. Don’t be afraid to ask questions. You are also welcome to submit a question to our Help & Resource Center to discuss your options.

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  • Step 2 What factors should I consider when reviewing institutions?

    • Total number of patients seen per year: Experience counts, particularly given the wide variety of health problems, complicated surgeries, etc., that face people with connective tissue conditions. Please note that patient and surgical numbers are not verified and may be estimates.
    • Total number of clinic hours per week: Usually centers of higher volume have dedicated clinic time to see patients with connective tissue or aortic issues. This could be time when families can meet others with a similar condition and discuss common issues.
    • Years servicing patients with Marfan and related conditions: This may be a good measure of a special interest and experience in treating patients with Marfan, VEDS, Loeys-Dietz, and related connective tissue conditions. However, newer centers may also offer expertise, special training, and rapidly increasing volume.
    • Clinical services offered: Be sure to review the services offered at the center. There may be particular services, such as high-risk pregnancy care, pain specialists, or the availability of a medical social worker, that are of special interest to you. In addition, check to see if the clinic provides the specialty care you need, for example, orthopedics, ophthalmology, nutritional support, etc.
    • Location: Some people choose the closest center. Others choose to travel out of area. If you do not have a site nearby, you may need to travel to receive the recommended care. After an initial consult at an institutional site, the care team can collaborate with a local cardiologist or general practitioner to provide care. This may be especially important during emergency situations.
    • Phone numbers: You may want to call the clinic to ask questions about the services offered and to schedule appointments.
    • Develop a care team with a specialized, experienced center as well as local physicians.
    • Insurance coverage:  Sometimes, because of insurance issues and difficulty traveling out of state to get medical care, it is necessary and more practical to go to a site that may be more limited in their service or experience. However, it is in a your best interest to make sure the healthcare professionals you see have an interest in learning to best care for someone with a connective tissue condition and are willing to collaborate and consult with experts when more information is needed.

  • Step 3 What should I look for when choosing a surgeon?

    Should I ask about a surgeon’s experience with my condition?

    Since aortic surgery is a key component for many of the conditions, the directory requests that sites provide five-year statistics of the surgical volume at the institution for both general aortic surgeries as well as those specifically for people with connective tissue conditions.  A high volume, however, does not always equate to good outcomes. Additionally, it is important to note that low pediatric surgical rates are due to the smaller number of surgical cases in children.

    Given that Marfan, VEDS, LDS, and related conditions are rare, even the number of surgeries at an experienced high volume center might seem low. For example, in a 2015 report from the Society of Thoracic Surgeons covering 2004 to early 2010, 13,746 patients (general population and those with connective tissue conditions) underwent aortic root replacement surgery for an enlarged aorta. The median number of operations performed at each site was only two in 2009 and only 5% of sites performed more than 16 aortic root replacements that year. Therefore, a surgeon who performs 8-12 surgeries per year on people with connective tissue conditions is performing these surgeries at a very high volume. It can be anticipated that low volume sites might only be performing one or two surgeries per year.

    Source: www.jtcvs.org/article/S0022-5223(14)00586-8/pdf

    What does the STS (Society for Thoracic Surgeons) Star rating on the institutional directory mean?

    The ratings categories are defined as:

    • Three stars: Participant performance is significantly higher than STS mean.
    • Two stars: Participant performance is not statistically different from STS mean.
    • One star: Participant performance is significantly lower than STS mean.

    For each of the two composite domains (absence of morbidity and absence of mortality), if a participant’s estimated score is lower than the overall STS average but the difference between the participant’s score and the STS average score is not statistically significant, the ratings would each be two stars. However, for the overall composite score, if the point estimate is lower than the STS average, AND the difference is statistically significant, the overall participant star rating is one star.

    Because the star rating depends upon how the database participant compares to the STS average for a given time period, and the STS average is subject to change each time the analysis is performed, there is not a prior morbidity or mortality level that a participant needs to attain in order to become a three-star institution. This also is true because the volume of cases at a given institution impacts the comparison of its performance to the STS average.

    Learn More About the Society for Thoracic Surgeons

    What should my surgeon know about open repair vs stent-grafts?
    • For someone with a complex aortic aneurysm and dissection history, it should be noted that the option of repairing a NON-life-threatening aneurysm with a stent-graft due to Marfan, Loeys-Dietz, or Familial Aortic Aneurysm is usually not recommended (see our Professional Advisory Board’s statement on stent-grafting). This is because stent-graft surgery will usually result in complicated re-operations for people with these conditions. The only time stent-grafting should be used is to bridge a life-threatening issue until open surgery can be performed in a controlled setting or when a patient is too frail to undergo an open operation. For individuals with VEDS, stent-grafts may be a better option than open surgery, however, open surgery in a controlled knowledgeable setting is sometimes required. The knowledgeable and experienced center can serve as “consultants” for the local team when the person is not nearby one of these centers.
Receiving care from a knowledgeable and experienced site is recommended for the following reasons:
  • Diagnosis – Sites that see more patients are better positioned to provide a proper diagnosis, which is critical for getting proper treatment. Genetic testing results should be reviewed by geneticists who are experienced with these conditions. Determining whether or not a mutation is a causative gene can require searching several databases because there is not one single database that covers all the mutations. Geneticists knowledgeable about Marfan, VEDS, LDS, and related conditions do this on a regular basis when reviewing reports from genetic laboratories.
  • Aortic Surgery – Surgical experience in patients with connective tissue conditions is critical for aortic root surgeries. It is especially important when a patient has to undergo a re-operation, which may present greater complications. Many surgeons are trained in traditional composite graft surgery (CVG) where mechanical valves replace the aortic valve. Fewer surgeons are trained in aortic valve-sparing surgeries that eliminate the need for life-long blood thinners. Additionally, due to the limited number of patients that undergo aortic valve-sparing procedures, many surgeons do not have a lot of experience in these surgeries. A surgeon who does 8-12 of these surgeries per year can be considered highly experienced. It should be noted that, if a center does not manage a lot of people with Marfan, VEDS, LDS, and related conditions, the surgical experience will be less.
  • Emergency Situations – Sites that have greater expertise in connective tissue conditions and aortic conditions may be better equipped to handle emergency situations. Your site should provide you with a special number to alert staff of an emergency. They should also have an emergency protocol in place. Your medical records should have a medical alert about the risks of these genetic aortic conditions. A Patient Care Coordination note or an FYI Alert signals medical professionals about the urgency to rule out emergency situations like arterial dissection, pneumothorax, or colon rupture. The note should appear in bright red and should be prominent on the upper right hand corner of the patient’s medical record home page.. Every patient should request to have this noted on their medical record. Emergency preparedness kits, emergency room information cards, and information about obtaining a medical alert bracelet are available.
  • Pregnancy – Access to a high-risk obstetrician and experienced cardiologist is needed to help ensure a safe and healthy delivery for both mother and child.

The Foundation urges everyone living with one of these conditions to ask about specific training, volume, and special services the medical group has to offer. At times, a site might not have services all at the same physical location, but they should have collaborations with other healthcare professionals at different locations who address certain issues. The Institution Directory provides links to full applications with the names of healthcare professionals in different disciplines, if they are available.

Many sites have websites that describe their services for people with Marfan, VEDS, LDS, and related conditions. Many healthcare professionals have a biography or curriculum vitae listing their education, training, and work experience. Websites can be helpful sources of additional information, but keep in mind that websites are a form of advertising. An impressive site may or may not reflect the level of care that is offered.

We suggest that you look at all factors noted in the institution directory and choose a site that fits your specific needs. Don’t be afraid to ask questions. You are also welcome to submit a question to our Help & Resource Center to discuss your options.

Ask a Question

Developing a Care Team

Everyone should aim to establish a care team.  A care team should consist of day-to-day general care that is handled by a primary care physician who is knowledgeable in genetic aortic conditions, a cardiologist, or a physician who is willing to learn about your specific condition.  You should speak to your physician about developing these goals. The care team should include several specialists depending on your condition and how your diagnosis affects you.

In selecting practitioners for the care team, it is important to define and explain the role of the primary care physician. They should be willing to:

  • Form a partnership with you to achieve health management goals.
  • Form a partnership with a specialist team at a high volume center. You should visit an expert at least once so that they have your history and medical records and can be called upon in an emergency.
  • Develop a care plan that includes medications, lifestyle changes, exercise modification, nutrition, and surveillance and imaging reminders.
  • Provide criteria and contacts in case of sudden healthcare developments and be sure that there is always designated back-up.
  • Provide care and management to high risk patients with a chronic and progressive condition.
  • Provide seamless cross coordination with specialists, especially in an emergency situation.
  • Be the main point of contact for the patient, family, and other healthcare professionals throughout the care process.
  • Review healthcare management information with ongoing updates and mentoring opportunities published in the medical literature, available from independent resources, or from The Marfan Foundation.
  • Add an emergency alert on the electronic medical record for arterial dissection, pneumothorax, carotid cavernous sinus fistula, and colon rupture, as well as emergency imaging considerations. Instructions are provided by The Marfan Foundation here.

When choosing a primary care physician, it is important to keep in mind and emphasize with the physician that, while they may not have experience caring for someone with a genetic aortic or vascular condition, they may have managed complications like arterial dissections, aneurysms, and lung collapses in individuals in the general public. Primary care physicians must be willing to collaborate within the care team, learn the risks associated with your condition, and become familiar with managing these complications. You can also check with them to ask for their referrals to trusted colleagues.