Bicuspid Aortic Foundation Patients and Families Page

Coping with Aortic Disease

How does someone cope with having aortic disease? How do those who love them handle it? Some individuals may have already lost a loved one to aortic dissection or rupture. How can they face this disease again in themselves or someone else? Along with the diagnosis, there is fear, confusion, and shock.

At best, aortic disease is difficult. At worst, it is devastating. It is important to know there is hope.

At best, receiving a diagnosis of an enlarged aorta is frightening, even though it also opens the door to proactive treatment. In addition, the aortic valve and/or other heart valves may be involved. At worst, with little or no warning, lives are lost to aortic dissection and rupture. An important goal is to find aortic disease early, when it can be treated proactively, decreasing the number of life-threatening emergencies.

Finding accurate information and expertise in aortic disease treatment is the beginning of moving from fear to courage, from shock to strength. Knowing where you stand removes uncertainty, and hope and assurance come from being under expert care.

Coping with aortic disease is a balance between:
      • understanding its seriousness, while still approaching life with confidence.
      • knowing and doing what is necessary for your safety, but not letting fear dominate you.
      • seeking and participating in the best possible treatment, while continuing to pursue a    normal life.

Although at times it may seem overwhelming, when proper care and treatment are in place those with aortic disease are strongly encouraged to pursue an active, normal life.

Medical and surgical advancements have conquered many of the barriers that in the past made thoracic aortic aneurysm and dissection so formidable.

Medicine can effectively control blood pressure and increase patient safety. Great ingenuity and skill have produced surgical solutions for each part of the thoracic aorta, and aortic surgeons continue to pursue new techniques. Innovation over many years has also resulted in the current ability to repair and replace heart valves, and options in treating valve disease continue to advance. Today, this allows aortic and valve surgery to be performed with low risk in experienced centers, before there is a life-threatening emergency. The goal is that those with known aortic disease should never find themselves in the wrong place at the wrong time.

Early detection, treatment by aortic specialists, and ongoing care can make a tremendous difference when it comes to aortic disease. There are challenges, but there is also more hope and help than ever before.

The treatment of aortic disease is not a destination. It is a life long journey, a series of steps that proactively seek the best possible outcome for you.

With proactive care, the journey can be a long one, encompassing a normal, active life span. Along the way, there will be medical and surgical treatment, ongoing monitoring, and checkups. Although today aortic disease may not be cured in the traditional sense, it can be treated expertly in experienced centers with long lasting results.

There may be some important events along the way, such as surgery on the aorta or heart valves. But most of the journey consists of the daily routine of blood pressure monitoring and control while maintaining a healthy, active life style. Along with periodic aortic checkups, this is the foundation of treatment for all aortic disease, preferably both before and after surgery.

Ideally, the treatment of aortic disease is a partnership, and you are a very important partner.

Successfully living a normal, active life is the common goal of those with aortic disease and those who care for them. The best possible experience is an ongoing collaboration between those who have aortic disease and those who expertly treat it. There is an important part for the patient and family to do, along with the medical professionals who care for them. Included through out this page are some practical suggestions, drawn from the experience of those with aortic disease. The ABC's of aortic disease are a good place to begin.

Aortic ABC's

The most important goal of the Aortic ABC's is decreasing and, if possible, eliminating high-risk emergencies due to aortic disease.

Not infrequently, an emergency results in a vulnerable patient arriving at a center with little or no aortic experience. The Aortic ABC's are aimed at helping you avoid this.

The ABC's for the identification and treatment of aortic disease are
    • Accurate diagnostic testing
     • Blood pressure management
     • Comprehensive, individualized care

A -- Accurate diagnostic testing

• Accurate testing is critical in order to understand the condition of the chambers of the heart, the heart valves, and the aorta. Depending on the risk factors involved, the blood vessels of the head and neck may also be examined.

• The emphasis must be on accuracy, both in the quality of the tests and in their interpretation.

• It is important to have tests interpreted by experienced aortic treatment centers.

• These tests are extremely important both for you and your physicians. They are the basis for aortic disease diagnosis and treatment.

Echocardiography
Echocardiography is often the first test used to screen for aortic disease.
• When done through the chest wall, (transthoracic echo), it is non-invasive.
• Sound waves are used; there is no exposure to radiation of any kind, and no contrast is needed.
• This test generally:
    • shows how well the heart valves are working.
    • shows how well the chambers of the heart are functioning.
    • may show the size of the aortic root and ascending aorta.
    • does not show the entire aorta.
    • does not always detect a bicuspid aortic valve.
    • is highly dependent on how it is done and how it is interpreted.

Spiral CT with Intravenous Contrast
• The majority of centers now use spiral CT with intravenous (IV) contrast.
• This test uses x-rays to produce an accurate view of the entire aorta.
• It is quick and generally available at most medical centers.
• CT scans without IV contrast cannot detect aortic dissection.
• The intravenous contrast needed may affect the kidneys.
• This test is not technician dependent, but should be interpreted by an aortic specialist.

MRI and MRA with Intravenous Contrast
• This test, using a magnetic field, also gives an accurate view of the entire aorta.
• There is no x-ray exposure, and there is much less affect on the kidneys from the intravenous contrast that is used.
• MRI technology has continued to progress. However, only the more advanced centers can produce high quality images of the aorta as well as the heart and heart valves. This has improved the ability to identify bicuspid aortic valves.
• MRI tests take longer than CT scans and are not as practical in an emergency situation.

How can you be sure you have an accurate measurement of your aorta?
• Experience has shown that when tests are interpreted by an aortic center, there is increased accuracy in diagnosis.
• If you cannot travel for a consultation in person, you can send test results to an experienced aortic center for analysis.

B -- Blood pressure management

Keeping blood pressure lower than normal is part of the life long treatment for those with fragile aortic tissue. The traditional use of beta blockers was the first recommended treatment for those with aortic disease. However, the addition of ACE inhibitors and ARBs, followed by calcium channel blockers, and sometimes diuretics, have shown markedly improved results.

Heavy weight lifiting causes blood pressure to rise too high, placing stress on the aortic wall that may result in aortic dissection. Individuals should discuss lifting and other exercise guidelines with their physicians.

If you have aortic disease, you can:

• Obtain a home blood pressure monitor. Fully automatic devices with an arm cuff and digital read out are easy to use.
• Take your blood pressure daily, and keep a log of your blood pressure and pulse.
• Work with your doctor to find the medication combination that is best for you.

Please visit our Health Records Page, where you can print a form for recording your blood pressure.

C -- Comprehensive, individualized care

One size does not fit all when it comes to aortic disease. It is important to be treated as the unique person you are.

In determining the best treatment plan for an individual, a comprehensive, detailed approach is needed. The conditions that cause aortic disease are often complex, and there are important decisions to make about what is best for each individual. You need to understand and be comfortable with the treatment planned for you.

Factors such as hypertension, general overall health, strength and activity level, age, family history, the normal size of the aorta, the size and location of the aortic aneurysm, the functioning of the heart valves, and whether or not there are aortic symptoms are all examples of things that are important in looking at the big picture for each person.

Questions often arise about exercise, sports participation, and heavy weight lifting (generally heavy weight lifiting is prohibited for those with aortic disease). These should be discussed with each individual as part of their assessment.

Understanding the underlying cause of aortic disease is important in planning an individual's care. In patients with bicuspid aortic valve disease, there are decisions to be made regarding the repair or replacement of the aortic valve, as well as the surgical approach for the aortic root and ascending aorta. However, considerations can be quite different in someone with an atherosclerotic ascending aortic aneurysm if the aortic valve is not affected and the aortic root is normal.

It is important to know exactly what applies to you as an individual. Experienced aortic treatment centers are positioned to provide this kind of comprehensive assessment and individualized care based on underlying disease factors.

Contacting an Aortic Treatment Center

Seeking help from an experienced aortic center becomes necessary for various reasons.
For some people, an aortic aneurysm in the chest was found by accident, while looking for something else. Others receive a diagnosis of bicuspid aortic valve disease or one of the other connective tissue syndromes. Still others may have an unspecified familial aortic disease. Regardless of how it happens, when aortic disease is found early, there is an opportunity to seek expertise to treat it before there is a life-threatening emergency.

Generally you should be able to arrange a consultation at an aortic treatment center by simply contacting them.

Whether you already know you have an aneurysm or a chronic aortic dissection, need screening as part of a family affected by aortic disease, or for any reason are seeking an initial evaluation, when you contact an aortic disease center you should expect a prompt, compassionate response from someone who will guide you through the evaluation process there.

Preparing for an Aortic Consultation

Plan to take someone with you to your appointments if possible.
• A lot of information will be covered and having someone else to listen along with you will help you.
• It is extremely difficult to learn that you have an aneurysm, and it is important to have support.

Create a medical history.
Any diseases and surgeries that you have had are an important part of who you are medically. Even if the story is a simple one, it is helpful to write it down in a brief, factual way, including dates whenever possible.

Capturing key points about your parents and other family members may also be important. In asking about expanded family, such as grandparents, aunts, and uncles, a pattern of sudden heart related death is sometimes uncovered. Unless an autopsy clearly ruled out aortic aneurysm rupture or dissection, it is possible that was the cause of death, not a heart attack. Familial aortic disease is sometimes discovered in this way.

Although aortic disease health care professionals will ask you questions and record your medical history, you may also wish to share a copy of your medical history with them. This is especially true if you have several complex medical conditions.

Prepare a list of questions ahead of time.
Often we later find ourselves saying, "I forgot to ask about that". Making a list ahead of time will help make sure that your important questions are covered.

Write down the questions that are really bothering you. Getting answers to your questions will clear up any confusion and allow you to focus on the things that are most important.

You will find that with time, there will always be more questions. Experienced aortic centers will be prepared to anticipate many of your questions and answer new ones as they come up.

To help you get started, here are samples of questions, in no particular order, that are typically important for those being treated for aortic disease.

1. Heart valves (There are four heart valves, but two of them, the aortic and mitral, are most commonly affected.)
    a. How well are my heart valves working?
    b. When should they be checked again?
2. Other heart conditions
    a. Should I be screened for coronary artery disease?
    b. How do I know if anything else is wrong with my heart?
3. Aorta
    a. How big should my aorta be normally?
    b. Do I have an enlargement or aneurysm of my aorta? If so, where?
    c. If I have an aortic aneurysm, how dangerous is this?
    d. If I have an aortic aneurysm, what caused it?
4. Tests
    a. Do I need any other tests?
    b. If so, what tests and what are they for?
    c. How safe are these tests for me, if I have an aortic aneurysm?
    d. Are there tests that I should avoid if I have an aneurysm?
5. Questions for the Surgeon (The answers apply to surgery done by this surgeon, at this center.)
    a. Do I need surgery? If so, how soon?
    b. What kind of surgery do I need? (valve, aorta, or both)
    c. What is the risk of death?
    d. What is the risk of permanent injury?
    e. What is the risk to me if I do not have surgery?
    f. How rare is this kind of surgery?
    g. How many of these surgeries have you done in the last year?
6. Infection
    a. Am I at greater risk if I get an infection?
    b. When do I need to take antibiotics?
7. Blood Pressure
    a. What should my blood pressure be?
    b. What medication do I need, and how should I take it?
    c. What if my blood pressure goes too low?
8. Exercise and lifestyle
    a. What exercises can I safely do?
    b. What exercises are dangerous for me?
    c. Would taking Coumadin affect my lifestyle?
    d. What about my diet?
    e. Should I stop smoking?
    f. I don't smoke, but someone else in my home does. Is this a problem?
9. My Family
    a. If I have aortic disease, could others in my family have it?
    b. I would like to have a baby. Is this dangerous for me?
    c. If I have children, are they at risk of inheriting this?

An Aortic Consultation

Aortic disease and those who have it are complex. A thorough aortic evaluation takes time and will result in a comprehensive understanding for both the doctor(s) and the individual. Listed below, in no particular order, are things that you should know following your aortic consultation. Several tests and office visits may be needed, but at the conclusion of an aortic disease evaluation, you should understand:

Your Aorta:
• The normal size of your aorta
• The size and location of any aortic enlargement

Your Heart:
• How well your heart valves are working
• If there are any other heart conditions that need treatment

Your Medication:
• What new medicine you have been given, and how to take it
• Any changes to your existing medicine

Your Tests:
• What is the assessment of any tests taken
• If additional tests are needed soon, what the tests are, and what they measure

If You Need Surgery:
• What surgery will include, and how soon it is needed
• What the risks of surgery are in that center, with that surgeon (% of deaths, % of injuries)
• What the risk of waiting and not having surgery is

Your Diet and Lifestyle:
• What changes you must make in your exercise routine, your diet, and your life style

Your Next Steps:
• When you should have your next appointment, and with which doctor(s)
• When you should have your next follow up tests, and what those tests are for
• Whether or not there is an aortic liaison, local support group, or other resources to help you
• What to do if you experience chest and back pain

Important Things to Know

It is helpful to know some important information about yourself or your loved one. Following is an example of some things that you should know. Some of the information below may not apply to those who have already had valve repair or replacement. A list something like this is also convenient for sharing with health care professionals.

     • List of medications you take (how much, how often)
     •Average non-exercise blood pressure and pulse (for example, blood pressure 105/60, pulse 55)
     •Date of your last echocardiogram
     •Aortic Valve
           •Is it bicuspid or trileaflet?
           •Is there stenosis? (Mild, moderate, or severe?)
           •Is there regurgitation? (Mild, moderate, or severe?)
     •Mitral Valve
           •Is there regurgitation? (Mild, moderate, or severe?)
     •Date of your last aortic CT or MRI scan
     •Normal size of your aorta
     •Size (in centimeters) and location (ascending, arch, or descending) of your aortic aneurysm (or enlargement).

Contact Us

Bicuspid Aortic Foundation
30100 Town Center Drive, Suite O-299
Laguna Niguel, CA 92677

Telephone
Toll free in the US (888) 310-HOPE (4673)
or
(949) 495-0027

Fax: (949) 606-0413

Email: contactus@bicuspidfoundation.com

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