From: Subject: What Are the Signs and Symptoms of Marfan Syndrome? Date: Wed, 26 Nov 2008 15:05:27 +0100 MIME-Version: 1.0 Content-Type: multipart/related; type="text/html"; boundary="----=_NextPart_000_0000_01C94FD8.6AA29560" X-MimeOLE: Produced By Microsoft MimeOLE V6.00.2900.5579 This is a multi-part message in MIME format. ------=_NextPart_000_0000_01C94FD8.6AA29560 Content-Type: text/html; charset="iso-8859-1" Content-Transfer-Encoding: quoted-printable Content-Location: http://www.nhlbi.nih.gov/health/dci/Diseases/mar/mar_signsandsymptoms.html What Are the Signs and Symptoms of Marfan = Syndrome?
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What Are the Signs and Symptoms of Marfan = Syndrome?

The signs and symptoms of Marfan syndrome vary from = one=20 person to another, even within the same family. Some = people=20 have mild signs and symptoms, while others may have = severe=20 problems and discomfort. Signs and symptoms occur in = many=20 parts of the body, including:

  • The bones and ligaments=20
  • The heart and blood vessels=20
  • The eyes=20
  • The lungs=20
  • The skin

Appearance and Body Build

Some of the major signs of Marfan syndrome are the = common=20 physical features seen in people with the condition. = People=20 with Marfan syndrome often have:

  • A tall, slender body build. They may be very = tall or=20 taller than other family members who do not have the = condition. However, it should be noted that short, = heavy=20 people also can have Marfan syndrome.=20
  • Long arms, legs, fingers, and toes. A person=92s = arm span=20 (the distance from the fingertips of one hand to the = fingertips of the other with the arms stretched out = from the=20 sides) may be greater than his or her height.=20
  • A long and narrow face.=20
  • A highly arched roof of the mouth with crowded = teeth.=20
  • A receding lower jaw, causing an overbite.=20
  • A protruding or sunken chest.=20
  • A curved spine.=20
  • Flat feet that are rotated inward (some people, = however,=20 have exaggerated arches).

Bones, Cartilage, and Ligaments

The bones of the limbs, hands, and feet often grow = too long=20 in people with Marfan syndrome. This typically leads = to a=20 tall, thin body with disproportionately long arms, = fingers,=20 legs, and toes. People with Marfan syndrome have = loose,=20 relaxed ligaments and are usually loose jointed.

Chest abnormalities may occur due to an overgrowth = of the=20 ribs. There are two types of chest abnormalities:

  • Pigeon breast, also called pectus carinatum. The = chest=20 protrudes outward like a bird=92s chest. This can = affect heart=20 and lung function.=20
  • Funnel chest, also called pectus excavatum. The = chest is=20 sunken or indented, reducing the space between the=20 breastbone and the backbone. As a result, the heart = and=20 lungs are displaced. Heart and lung function may be=20 affected, leading to breathing and endurance = problems.=20

Curvature of the spine may occur. It usually = develops=20 during childhood, often gets worse during the teenage = growth=20 spurt, and may require surgical treatment. The three = main=20 types of abnormal spine curvature are:

  • Scoliosis=97a side-to-side curvature=20
  • Lordosis=97an inward curvature of the spine in = the lower=20 back, just above the buttocks=20
  • Kyphosis=97an outward curvature of the spine in = the upper=20 back (hunchback)

Curvature of the spine can cause back pain, posture = problems, and deformity. Scoliosis can sometimes = reduce lung=20 function.

Another problem that can occur is dural ectasia=20 (ek-TA-ze-a), which is the stretching of the membrane=20 surrounding the brain and spinal cord. Dural ectasia = can wear=20 down the bones of the spine over time. Symptoms may=20 include:

  • Pain in the lowest part of the back=20
  • Pain in the abdomen=20
  • Pain, numbness, or weakness of the legs =

Dural ectasia is a hallmark of Marfan syndrome that = is very=20 rare in the general population.

Heart and Blood Vessels

People who have Marfan syndrome often have problems = with=20 the heart and blood vessels. The valves of the heart = may not=20 work properly and may permit some blood flow to be = reversed,=20 causing the heart to do extra work. The aorta=97the = large artery=20 that carries blood away from the heart to the = body=97may stretch=20 and enlarge. These problems can cause signs and = symptoms, such=20 as:

  • A heart=20 murmur. This can be the result of valves not = functioning=20 properly or other heart problems.=20
  • Sudden chest pain. This can be a symptom of = aortic=20 dissection, a life-threatening complication that can = occur=20 due to weakened aorta walls. The pain may occur in = the front=20 or in the back, usually in the midline of the body. = The=20 location of the pain may change as the tear in the = aorta=20 grows. In some cases, a dissection does not cause = dramatic=20 pain.=20
  • Abnormal heart rhythms called arrhythmias (a-RITH-me-as). A = person with=20 Marfan syndrome may develop an arrhythmia as a = result of=20 problems with the heart valves or due to car= diomyopathy=20 or c= ongestive=20 heart failure.=20
  • Shortness of breath and fatigue (tiredness). = These can=20 be symptoms of heart failure due to an overworked = heart.=20

Two of the heart and blood vessel complications = that can=20 occur in people with Marfan syndrome are mitral valve prolapse (MVP) and = enlargement of=20 the aorta.

Mitral Valve Prolapse

MVP is a problem with the heart=92s mitral valve. = The mitral=20 valve controls the flow of blood through two of the = chambers=20 in the heart, the left atrium and the left ventricle. = The=20 flaps of the valve are designed to allow blood to flow = in one=20 direction=97from the left atrium into the left = ventricle=97and to=20 prevent blood from flowing the other way.

In MVP, the mitral valve does not work correctly. = The valve=20 flaps are large and floppy. They may overlap or not = close=20 completely. One or both flaps may flutter or swing = back into=20 the atrium.

The abnormal mitral valve can allow blood to flow = backward=20 through the valve in the wrong direction. This is = called=20 mitral regurgitation. When this happens, the heart has = to work=20 harder to make up for the backward flow of blood. Over = time,=20 the heart can become overworked, leading to heart = failure.

3D"Figure

Figure A shows the normal mitral = valve=20 separating the left atrium from the left ventricle. = Figure B=20 shows the heart with mitral valve prolapse. Figure C = shows the=20 detail of mitral valve prolapse. Figure D shows a = mitral valve=20 that allows blood to flow backward into the left = atrium.

MVP occurs in about 3 out of every 4 people with = Marfan=20 syndrome. It also occurs in people who do not have = Marfan=20 syndrome. In many people with MVP, little or no blood = leaks=20 back through the valve, there are no symptoms, and no=20 treatment is needed. In some people, blood does leak = back=20 through the valve, and these people may have symptoms = and=20 require treatment.

Enlargement of the Aorta (Aortic Dilation)

The aorta is the large artery that carries = oxygen-rich=20 blood away from your heart out to the rest of the = body. When=20 weak connective tissue causes the walls of the aorta = to=20 weaken, the aorta widens and stretches. Most often, = the=20 enlargement begins where the aorta connects with the = left=20 ventricle of the heart, just above the aortic valve. = This part=20 of the aorta is called the aortic root.

Enlargement of the aorta can lead to several = serious=20 complications:

  • Aortic root dilation. The aorta can widen and = bulge=20 where it attaches to the heart. This happens due to=20 continued weakening of the wall of the aorta. Aortic = root=20 dilation may not cause any symptoms until it becomes = quite=20 large. If symptoms occur, they may include pain in = the=20 chest, upper back, neck, and jaw; coughing and = hoarseness;=20 and trouble swallowing. As part of this dilation, = one=20 section may enlarge and bulge out. This is called an = aortic=20 aneurysm (AN-u-rism). The aneurysm can rupture, = causing=20 massive internal bleeding, which is a = life-threatening=20 medical emergency.=20
  • Aortic regurgitation. The aortic valve sits = between the=20 left ventricle and the aorta and allows blood to = flow one=20 way, from the ventricle to the aorta. In Marfan = syndrome, as=20 the aorta widens, the aortic valve also is = stretched, and=20 the valve flaps cannot close properly. This allows = blood to=20 leak the wrong way, from the aorta back into the = left=20 ventricle (regurgitation). As a result, the heart = has to=20 work harder to pump blood out to the body. The heart = can=20 become overworked, leading to heart failure. People = with=20 aortic regurgitation may have shortness of breath = and=20 exercise intolerance.=20
  • Aortic dissection. This is a tear that occurs in = the=20 weakened layers of the inner wall of the aorta. The = tear=20 allows blood to get under the inner layer of the = wall. As a=20 result, the aorta tears further. An aortic = dissection is a=20 life-threatening emergency. Signs and symptoms of an = aortic=20 dissection include abrupt onset of chest pain, which = may=20 range from mild to severe. There may be pain in the = back or=20 the abdomen, or both. The person may sweat, vomit, = faint,=20 and have a weak pulse.

Eyes and Vision

Nearsightedness (myopia) is the most common eye = problem in=20 people with Marfan syndrome. It can range from mild to = severe.=20 People with Marfan syndrome often have astigmatism=20 (a-STIG-ma-tizm), which causes visual distortion and = blurred=20 vision.

Dislocation of the lens of the eye (ectopia lentis) = is=20 another common complication of Marfan syndrome. It is=20 considered a hallmark of Marfan syndrome because it = occurs in=20 very few other conditions. It may affect one or both = eyes, and=20 the lens may be higher or lower than normal or shifted = off to=20 the side. Vision in the affected eye(s) may be = severely=20 affected.

Other, less common eye problems in Marfan syndrome = include=20 detached retina, cataracts, and glaucoma. A detached = retina=20 occurs when there are holes or tears in the inner = lining of=20 the eye. A cataract is a clouding of the lens. = Glaucoma occurs=20 as a result of high pressure in the eye. People with = Marfan=20 syndrome tend to get cataracts and glaucoma at a = younger age=20 than people who do not have Marfan syndrome.

Lungs

In Marfan syndrome, the air sacs in the lungs may = be larger=20 than normal. This can cause breathing problems. People = with=20 Marfan syndrome are at an increased risk of developing = emphysema or chronic=20 obstructive pulmonary disease (COPD), a serious = lung=20 disease.

Sudden collapse of the lung, called spontaneous=20 pneumothorax (noo-mo-THOR-aks) can occur in people = with Marfan=20 syndrome. About 1 in every 20 people with Marfan = syndrome=20 develops this problem. Collapsed lung can happen after = only a=20 slight blow to the chest, or even while at rest = without a=20 known cause. Collapsed lung usually causes sudden = shortness of=20 breath and requires immediate medical attention.

Sleep=20 apnea is a problem that is often associated in the = general=20 population with being overweight or obese. But thin = people=20 with Marfan syndrome can also suffer from it. This may = be due=20 to looseness of the connective tissue in the = airways.

Skin

Stretch marks on the skin occur in about 2 out of = every 3=20 people with Marfan syndrome. The stretch marks are = usually on=20 the lower back, buttocks, shoulders, breasts, thighs, = and=20 abdomen. They differ from stretch marks in the general = population because, in people with Marfan syndrome, = they occur=20 without excessive weight gain or loss. The marks = usually=20 appear around the time of puberty, but may occur in = childhood.=20 They do not require=20 = treatment.


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