From: Subject: The National Marfan Foundation Date: Fri, 14 Nov 2008 15:04:58 +0100 MIME-Version: 1.0 Content-Type: multipart/related; type="text/html"; boundary="----=_NextPart_000_0000_01C9466A.5C6943D0" X-MimeOLE: Produced By Microsoft MimeOLE V6.00.2900.5579 This is a multi-part message in MIME format. ------=_NextPart_000_0000_01C9466A.5C6943D0 Content-Type: text/html; charset="iso-8859-1" Content-Transfer-Encoding: quoted-printable Content-Location: http://www.marfan.org/nmf/GetSubContentRequestHandler.do?sub_menu_item_content_id=16&menu_item_id=4 The National Marfan Foundation

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 3D""=20 3D"The

Pr= inter=20 Version

The Heart and the Blood Vessels=20

Excerpt from=20 The Marfan Syndrome, by Reed E. Pyeritz, = M.D.,=20 Ph.D. and Cheryll Gasner, M.N., C./F.N.P. Fifth = Edition, July=20 1999, Revised September 2001

The heart and = blood=20 vessels (the cardiovascular system) are affected in = nearly all=20 persons, young or old, with the Marfan syndrome. The = next=20 figure schematically shows the major structures of the = central=20 cardiovascular system, and the direction of blood flow = during=20 the relaxation, or filling, phase of the cardiac cycle = (diastole) and during the contraction, or pumping, = phase=20 (systole).



Normal structure and = function of=20 the cardiovascular system in diastole (left) and in = systole=20 (right).

During diastole, both the = tricuspid and=20 mitral valves are opening allowing the right and left=20 ventricles to fill with blood. The pulmonic and aortic = valves=20 are closed. During systole, both the tricuspid and = mitral=20 valves close. As the right and left ventricles begin = to=20 squeeze the pulmonic and aortic valves open allowing = blood to=20 leave the ventricles and go forward.

The valves = of the=20 heart are fine tissue leaflets that keep the blood = flowing in=20 the proper direction. In most people with the Marfan = syndrome,=20 the valves are of abnormal composition and size. In=20 particular, the two mitral valve leaflets are usually = too=20 large for the opening they need to cover; the result = is that=20 the leaflets billow backward when the main pumping = chamber of=20 the heart (left ventricle) contracts. This abnormal = motion is=20 called mitral valve prolapse and occurs in at least = 75% of=20 people with the Marfan syndrome. However, mitral valve = prolapse is present in 5% of the general population, = and is=20 not in and of itself diagnostic of the Marfan = syndrome. In=20 many people with such prolapse, the mitral valve makes = a=20 "click" as it moves backward, a sound heard through = the=20 stethoscope. Mitral valve prolapse can only be = detected in=20 some people by an echocardiogram, a painless test = during which=20 high-frequency sound waves (ultrasound) are bounced = through=20 the chest wall and off the heart to produce a sonar = picture of=20 how the valves move. In most people with prolapse, the = mitral=20 valve does not leak. In about one-third, however, = blood leaks=20 "backward" through the valve and creates a heart = murmur of a=20 particular type, also detected by the physician using = a=20 stethoscope. For many people with a leaking mitral = valve=20 (mitral regurgitation), no symptoms develop and the = heart=20 compensates quite well; if the leak remains small, = minimal=20 therapy is necessary. A small proportion of = individuals with=20 mitral regurgitation in the Marfan syndrome do have=20 progression of the problem and develop symptoms, such = as=20 breathlessness on exercise or even at rest, a rapid or = irregular pulse (palpitations), or extreme tiredness. =



Mitral valve prolapse. = On the=20 left, the valve prolapses as the ventricle contracts = in=20 systole (note the blood exiting into the aorta). On = the right,=20 the mitral valve is so floppy that the prolapse = permits blood=20 to flow back into the left atrium during systole.=20

The heart muscle itself and the blood vessels = that=20 supply the heart muscle with blood (coronary arteries) = may=20 develop problems directly related to the Marfan = syndrome, such=20 as poor function of the heart muscle, although this = has not=20 been studied adequately. The heart muscle most = commonly=20 suffers damage as a result of valve leakage. = Additionally, a=20 person with the Marfan syndrome probably has the same = risk as=20 anyone else for developing hardening of the arteries=20 (atherosclerosis) and high blood pressure = (hypertension),=20 disorders that also lead to heart disease. =

Blood=20 vessels include arteries, veins and the small = capillaries in=20 between. Blood flows from the heart in arteries, and = returns=20 in veins. In the Marfan syndrome, it is primarily the = largest=20 artery, the aorta, that is affected. The aorta arises = directly=20 from the outlet of the left ventricle; all blood = pumped from=20 the heart must first pass into this artery. After = leaving the=20 heart, the aorta branches into large vessels that = carry=20 oxygen-rich blood to the entire body.



The entire aorta and = its major=20 branches. The aorta is roughly divisible into four = segments:=20 ascending (including the root); arch; descending = thoracic;=20 and, abdominal.

The aortic valve lies = between the=20 left ventricle and the aorta and prevents blood, once = pumped=20 out to the aorta, from leaking back to the heart. The = wall of=20 the aorta consists of three parts: A thin inner layer, = one=20 cell thick (intima), a thick, elastic middle layer = (media),=20 and a tough, outer layer (adventitia). In the Marfan = syndrome,=20 the media is weaker than normal.



A cross-section of the = ascending=20 aorta, showing the three layers of the aortic wall. = The=20 elasticity of the aorta is due to the media. =

Year=20 after year, as the aortic wall faces the constantly = changing=20 stress generated by the beating heart, the wall, = particularly=20 the first 2-4 inches above the aortic valve gradually=20 stretches. The diameter of this region (sinuses of = valsalva)is=20 usually about 1 to 1 1/2 inches (25-37mm)in an adult. = As the=20 diameter of the sinuses widens (dilates), an anuerysm = may form=20 when the enlargement becomes substantial (an arbitrary = concept=20 at best, but about 2 inches, or 50 mm, in practice). =



The ascending aorta in=20 cross-section along the long axis. The head is above = the top=20 of the figure. In an adult, the diameter of the = sinuses of=20 Valsalva is 1 to 1 1/2 inches. The arrows show the = normal=20 direction of blood flow during systole.

As = the=20 aorta widens, two things can happen. First, the aortic = valve=20 leaflets can be stretched and fail to close the aorta=20 completely. This means that between heartbeats, blood = leaks=20 back into the heart (aortic regurgitation), causing = the left=20 ventricle to work harder to pump blood. Without = treatment, the=20 heart can become seriously overworked and reach a = situation=20 called heart failure. At this stage, the individual = may have=20 the same symptoms as someone with severe mitral = regurgitation=20 and, in addition, may faint or develop chest pain. =



Enlargement = (dilatation) of the=20 aortic root. In the main figure, the heart is in = diastole and=20 the aortic valve is closed, preventing blood that has = just=20 been pumped into the aorta from leaking backward into = the left=20 ventricle. The aorta is moderately dilated. The large = arrow=20 shows normal diastolic flow of blood from the atrium = into the=20 ventricle. In the inset, the aortic root is = considerably=20 dilated and the aortic valve can no longer prevent = backward=20 leakage of blood (aortic regurgitation). =

Second,=20 as the aorta widens, a tear can develop in the inner = wall and=20 rapidly extend into the weakened middle layer. Once = the tear=20 develops, the force of pumping blood can cause the = middle=20 layer to separate, forming an abnormal second channel = through=20 which blood can flow. This process is called = dissection and=20 may be associated with severe pain in the front of the = chest,=20 in the neck, in the back, or in a combination of = sites.=20 Eventually, at the leading edge of the abnormal = channel, a=20 second tear usually occurs, either inward, allowing = blood to=20 reenter the aorta, or outward, producing a rupture = with=20 bleeding into the body.



Aortic dissection. In = the Marfan=20 syndrome, most tears in the aortic wall occur just = above the=20 aortic valve.

The first portion of the = aorta=20 enlarges to some degree in nearly everyone with the = Marfan=20 syndrome. The echocardiogram is the simplest method = for=20 measuring the aorta in this region. Enlargement = usually begins=20 quite early in life, and is detectable in most = children and=20 many infants with Marfan syndrome by echocardiography. = In=20 years past, most affected persons experiencedx one of = the=20 serious aortic complications. As will be discussed = later,=20 treatment methods are available which can prevent = these=20 problems before major complications occur.


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