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  • New article on HCM - Harvard Heart Letter

    Copyright 2003 Gale Group, Inc.
    IAC (SM) Newsletter Database (TM)
    Copyright 2003 Copyright by President and Fellows of Harvard
    College. All Rights Reserved

    Harvard Heart Letter

    February 1, 2003

    SECTION: No. 6, Vol. 13; Pg. 0 ; ISSN: 1051-5313

    IAC-ACC-NO: 96292374

    LENGTH: 1629 words

    HEADLINE: When big-hearted is bad.

    AUTHOR-ABSTRACT:
    THIS IS THE FULL TEXT: COPYRIGHT 2003 President and Fellows of Harvard College
    on behalf of Harvard Health Publications (Harvard)/Staywell. Inc. Subscription:
    $ 32.00 per year. Published monthly. Consumer Health Publishing Group for
    Harvard Health Publications, One Atlantic Street, Suite 604, Stamford, CT 06091.
    FAX 617-432-1506.

    BODY:

    An overgrown heart muscle can kill. In most cases, though, it's just a
    reason for extra care.

    A strong, muscular heart is a foundation of good health - except when it's
    too muscular. That's the case with hypertrophic cardiomyopathy
    (high-per-TROE-fik car-dee-oh-my-AH-puh-thee), an inherited condition in which
    part of the heart looks like it's pumped up on steroids. Instead of helping the
    heart work more efficiently, the extra muscle can interfere with its function.

    Hypertrophic cardiomyopathy, or HCM, is a confusing condition. Over the
    years it has been called at least 75 different names. And because many of the
    early reports about it were penned by researchers at high-level cardiac centers,
    which tend to receive the sickest patients, it got a reputation as a disabling
    and deadly disease.

    There's no question that HCM can restrict life, or even cut it short. The
    latest research, though, emphasizes that the risks of this condition have been
    overstated. Some people never know they have it. For many others, it's a
    controllable condition that may have little impact on life and longevity.

    As heart diseases go, HCM isn't as common as coronary artery disease or
    congestive heart failure. It affects only about 1 in every 500 U.S. adults.
    Still, that's enough people to fill a city the size of Miami or Minneapolis.

    Cause and effect

    HCM stems from a mutation in any one of at least 10 genes that code for
    proteins in heart muscle fibers. These abnormal genes make heart muscle fibers
    grow in a disorganized fashion.

    They also somehow force extra muscle growth, especially in the left
    ventricle (the muscular lower chamber that pumps blood to the body) and the
    septum (the wall separating the left and right sides of the heart). In a normal
    heart, the septum is 10-12 millimeters thick. That's just under half an inch. In
    HCM, it can measure as much as 60 millimeters, or 2.4 inches thick.

    This extra muscle poses several problems:

    * It crowds the space inside the left ventricle, taking up room normally
    used to collect oxygenated blood. The extra muscle is also stiff and has trouble
    relaxing between beats. If the left ventricle can't fill properly, it may not be
    able to deliver enough blood to meet the body's needs, especially during
    physical activity. In severe cases, fluid can back up into the lungs.

    * It can throw the mitral valve out of alignment. Instead of closing tightly
    after a contraction squeezes blood from the left atrium to the left ventricle,
    the mitral valve may flap open and block blood that's being pumped from the left
    ventricle to the aorta (and so to the body). This can cause dizziness, fainting,
    or breathlessness. It can also make exercise difficult.

    * It may demand more oxygen than the body can supply. If arteries feeding
    the left ventricle and septum can't reach all parts of the thickened muscle, or
    can't deliver enough oxygen to them, chest pain can follow. This is much like
    the pain from angina caused by cholesterol-clogged arteries.

    * It can cause disturbed and sometimes deadly heart rhythms. Disorganized
    muscle fibers and scar tissue from stretched heart muscle can interfere with the
    pattern of electrical signals that governs regular heartbeats.

    Detecting HCM

    In youngsters and young athletes, the first sign of HCM is sometimes sudden
    death caused by a lethal heart rhythm. Most people with HCM, though, find out in
    less dramatic ways. Some experience symptoms such as fainting spells,
    breathlessness, chest pain, or palpitations that are traced to a hypertrophic
    heart. Others learn they have it when they see a doctor because a close relative
    has been diagnosed with this condition. (See A family affair.)

    Checking for HCM is relatively straightforward. A heart murmur heard through
    a stethoscope can indicate that the mitral valve is opening in the strong,
    turbulent outflow from a narrowed left ventricle. An electrocardiogram can
    reveal abnormalities that signal thickened heart muscle. The ultimate test is an
    echocardiogram. Using sound waves, this test can show the size and shape of the
    heart as well as the blood-flow pattern inside it.

    A family affair

    HCM occasionally crops up in people who don't have relatives with it. Most
    of the time it's inherited. If you have HCM, each of your children has a 50/50
    chance of inheriting a faulty gene. Because of this pattern, when an individual
    is diagnosed with HCM, it's a good idea for his or her family members - brothers
    and sisters and their children - to be checked for the disease.

    Managing HCM

    This condition can make itself known at any stage of life, from infancy to
    old age. Symptoms can stay stable for years, then flare and subside. Or the
    condition can appear suddenly and quickly worsen. This variability makes HCM
    unique among cardiovascular diseases.

    It also means there's no one-size-fits-all therapy. Treatment is based on
    the presence and severity of symptoms or complications. If you have neither,
    then you need vigilance (see Living with HCM) but not treatment. Mild symptoms
    often respond to drug therapy. When they don't, or if they interfere with daily
    life, a procedure to shrink the overdeveloped heart tissue may be in order.
    Ongoing or potential rhythm problems must also be treated.

    Drug therapy. Drugs are the first line of defense against shortness of
    breath, chest pain, and other symptoms of HCM. Beta blockers reduce the heart's
    workload and help the ventricles fill with blood. So do calcium channel
    blockers. A drug called disopyramide (Norpace) can help the heart relax and
    prevent erratic heart rhythms such as atrial fibrillation. Amiodarone
    (Cordarone) is another drug used to control erratic rhythms. If HCM leads to
    atrial fibrillation, a blood thinner such as warfarin can help ward off the
    strokes that this erratic rhythm can generate.

    Surgery. If drug therapy can't relieve HCM symptoms, the next step could be
    an operation to remove some muscle from the septum. This procedure is relatively
    safe (98 of 100 people survive it) and effective (it eases symptoms in 70 of 100
    people). But because it requires open-heart surgery and the use of a heart-lung
    bypass machine, recovery can take weeks, and complications can arise.

    Alcohol ablation. A new technique offers an alternative to surgery. It uses
    an alcohol injection to thin the upper part of the septum. Alcohol ablation
    eases symptoms as much as surgery does, but with fewer complications, a shorter
    hospital stay, and a faster recovery. Although the early results look good,
    doctors don't yet know the long-term effects of this procedure. One worry is
    that the scar left behind by the alcohol injection could lead to dangerous
    rhythm problems later in life. The National Heart, Lung, and Blood Institute is
    now recruiting participants for a study on this technique.
    The HCMA does not support the protocol currently in use by the NIH for this research
    Implantable pacemaker. When HCM blocks or reroutes electrical signals to the
    ventricles, an implanted pacemaker may be needed.

    Implantable cardioverter/defibrillator (ICD). The sudden and unpredictable
    onset of fast, erratic contractions of the ventricles is a common cause of death
    among people with HCM. The only treatment is to abort these lethal arrhythmias -
    called ventricular tachycardia and ventricular fibrillation - with an electric
    shock. This can be done with an implantable cardioverter/defibrillator. An ICD
    constantly monitors the heart's rhythm and delivers a shock if it senses
    trouble.

    Living with HCM

    Because hypertrophic cardiomyopathy affects different people in different
    ways, everyone needs a different strategy for adapting to life with this
    condition. Here are a few general guides:

    * Call your doctor right away if you faint, feel dizzy, or find yourself
    unexpectedly short of breath. These could be signs of potentially dangerous
    HCM-associated trouble.

    * For most people with HCM, light to moderate exercise is perfectly fine,
    even healthy. Activity that causes or increases symptoms, though, must be
    treated with caution and advice from a doctor. Most experts recommend that
    people with HCM not participate in competitive sports, physically demanding
    activities, or activities that strain the heart, such as weight lifting.

    * Drink plenty of fluids, especially when you exercise. Dehydration worsens
    the left ventricle's filling problems.

    * Follow a basic heart-healthy eating plan. Beyond that, you don't need a
    special diet or vitamin supplements.

    * If your blood pressure is high, work with your doctor to get it under
    control. High blood pressure can further enlarge the left ventricle, the last
    thing someone with HCM needs.

    * Ask your doctor about taking antibiotics before dental work and other
    invasive procedures (Harvard Heart Letter, September 2002). A hypertrophic heart
    is susceptible to an infection of the heart valves that can originate with such
    procedures.

    * Be scrupulous about getting regular checkups. If you are doing well and
    have no symptoms, once a year should be fine. But if you have symptoms or have
    an ICD, you'll need to see your doctor more often.

    * Help the cause by taking part in a clinical trial. There's still a lot to
    be learned about HCM, from the genes that cause it to the treatments that
    control it. If you have the time or interest, ask your doctor about trials for
    which you might be eligible.

    For more information

    Hypertrophic Cardiomyopathy by Barry Maron, MD, with Lisa Salberg. Futura
    Publishing Co., Armonk, NY, 2001.

    The Hypertrophic Cardiomyopathy Association, 973-983-7429 or
    www.hcma-heart.com or www.4hcm.org
    Or visit www.health.harvard.edu/heart

    IAC-CREATE-DATE: January 9, 2003

    LOAD-DATE: January 11, 2003
    Knowledge is power ... Stay informed!
    YOU can make a difference - all you have to do is try!

    Dx age 12 current age 46 and counting!
    lost: 5 family members to HCM (SCD, Stroke, CHF)
    Others diagnosed living with HCM (or gene +) include - daughter, niece, nephew, cousin, sister and many many friends!
    Therapy - ICD (implanted 97, 01, 04 and 11, medication
    Currently not obstructed
    Complications - unnecessary pacemaker and stroke (unrelated to each other)

  • #2
    Lisa:

    I received my copy of the Harvard Heart Letter yesterday. I was very happy to see them mention the HCMA, Dr. Maron and You!! Keep up the good PR.

    Tricia

    Comment


    • #3
      I am glad you saw that... I was unaware they were running it... and VERY happy to see it!
      Nothing like Harvard giving you some props!
      Lisa
      Knowledge is power ... Stay informed!
      YOU can make a difference - all you have to do is try!

      Dx age 12 current age 46 and counting!
      lost: 5 family members to HCM (SCD, Stroke, CHF)
      Others diagnosed living with HCM (or gene +) include - daughter, niece, nephew, cousin, sister and many many friends!
      Therapy - ICD (implanted 97, 01, 04 and 11, medication
      Currently not obstructed
      Complications - unnecessary pacemaker and stroke (unrelated to each other)

      Comment


      • #4
        Harvard Health Letter

        Lisa, hi! New to your site ... old to HCM.

        Why the statement that HCMA does not support NIH alcohol ablation protocol? What's up?

        Bob Sexton

        Comment


        • #5
          The manner in which the NIH has designed the study is not one which I can place my support behind. I will only support protocols in which I feel I would enter myself or suggest a family member participate in.
          I have been in contact with many participants in this study who have had significant symptoms post procedure that have been dismissed as non issues- -this is the same practice the investigators used in the early days of DDD pacing - which later turned put to be highly problematic.
          There are other centers preforming the procedure and providing CARE to the patients afterward.
          HCM patients are entitled to CARE and treatment not simply to researched upon.
          I highly recommend centers that provide CARE over those who are research based.

          I hope this clears up your questions.

          Lisa
          Knowledge is power ... Stay informed!
          YOU can make a difference - all you have to do is try!

          Dx age 12 current age 46 and counting!
          lost: 5 family members to HCM (SCD, Stroke, CHF)
          Others diagnosed living with HCM (or gene +) include - daughter, niece, nephew, cousin, sister and many many friends!
          Therapy - ICD (implanted 97, 01, 04 and 11, medication
          Currently not obstructed
          Complications - unnecessary pacemaker and stroke (unrelated to each other)

          Comment


          • #6
            Harvard Heart Letter

            Lisa, what highly problematic with DDD protocol?

            Comment


            • #7
              Bob, Unfortunately, as participants at the NIH, we found out later rather than sooner, that they are not able to perform all treatment options at that facility. The DDD pacing was the protocol in effect at that time and that is what we were offered, and adequate info about other options were not forthcoming. Even more unfortunate was the fact that we were not well informed enough to press for this info. Long story short, we discovered after a cardiac arrest event(he recovered thankfully) that this treatment plan was not of benefit in our situation. Not one, but several HCM experts from several different facilities agreed on the necessary treatment - myectomy/valve repair. Who can say, but I must continue to wonder if earlier repair would have been more beneficial. All options must be offered and explained, even if not available at that facility. This is not only morally right, legally required. The patient has the right to choose to go somewhere else if the care is not available there. I would caution anyone who chooses to consult at a research facility, to go with a good understanding of treatment options ahead of time. How else will you know if you have been offered all options and info? Now somehow, that doesn't seem right, as consumers, we expect the experts to give us that info, that's why we went there, right? But we must also know that nothing is perfect, and we must be constantly pro-active. Watch out for yourself! Enter into any treatment plan only after all questions have been addressed satisfactorily. So much good has come from the NIH over the years. I wish I had a more positive outcome to offer, so I will just say that the nurses and ancillary staff were always wonderful to us, and we have many fond memories of them. I hope they can understand how much they meant to us. Linda

              Comment

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