Prevention Ofroot Dilatation in Marfan's Syndrome

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Prevention Ofroot Dilatation in Marfan's Syndrome

Eectrocardiogram is a more sensitive indicator than echocardiography ofhypertrophic cardiomyopathy infamilies with a mutation in the MYH7 gene 111 expression during cardiac growth. Int Rev Cytol tricular hypertrophy identified by two dimensional 1991;124:137-86. echocardiography in patients with hypertrophic car- 21 Karam R, Lever HM, Healy BP. Hypertensive hyper- diomyopathy. Am I Cardiol 1983;51:189-94. trophic cardiomyopathy or hypertrophic cardiomyopa- 24 McKenna W, Stewart JT, Nihoyannopoulous P, McGinty thy with hypertension? A study of 78 patients. JAm Coil F, Davies MJ. Hypertrophic cardiomyopathy without Cardiol 1989;13:580-4. hypertrophy; two families with myocardial disarray in 22 Gilligan D, Cleland J, Oakley C. The clinical and genetic the absence of increased myocardial mass. Br Heart J of cardiomyopathy. Current 1990;63:287-90. heterogeneity hypertrophic Br Heart J: first published as 10.1136/hrt.72.2.111 on 1 August 1994. Downloaded from Medical Literature-Cardiovascular Medicine 1990;9: 25 Panza JA, Maron BJ. Relation of electrocardiographic 155-61. abnormalities to evolving left ventricular hypertrophy in 23 Maron BJ, Wolfson JK, Ciro E, Spirito P. Relation of elec- hypertrophic cardiomyopathy during childhood. Am J trocardiographic abnormalities and patterns of left ven- Cardiol 1989;63: 1258-65. ABSTRACTS IN CARDIOLOGY Prevention of root dilatation in Marfan's syndrome Prophylactic replacement of a dilating aortic young people to long-term side effects with- root to forestall dissection is an accepted form out proven benefit is not justifiable. of management in Marfan's syndrome but The problem of dissection in Marfan's syn- there is now clear evidence, in the article by drome is not totally solved however. In some Shores et al, that the need for surgery can be patients dissection occurs before the aorta reduced by long-term fi adrenergic blockade. dilates and even in those on fi blockade aortic Such evidence was needed. The doses used to dilatation continues, albeit at a slower rate. achieve a negative inotropic effect, and theo- We are, however, closer to rational therapy. retically reduce risk, are high. To expose M J DAVIES http://heart.bmj.com/ on September 24, 2021 by guest. Protected copyright..

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Marfan Syndrome
Marfan syndrome Description Marfan syndrome is a disorder that affects the connective tissue in many parts of the body. Connective tissue provides strength and flexibility to structures such as bones, ligaments, muscles, blood vessels, and heart valves. The signs and symptoms of Marfan syndrome vary widely in severity, timing of onset, and rate of progression. Because connective tissue is found throughout the body, Marfan syndrome can affect many systems, often causing abnormalities in the heart, blood vessels, eyes, bones, and joints. The two primary features of Marfan syndrome are vision problems caused by a dislocated lens (ectopia lentis) in one or both eyes and defects in the large blood vessel that distributes blood from the heart to the rest of the body (the aorta). The aorta can weaken and stretch, which may lead to a bulge in the blood vessel wall (an aneurysm). Stretching of the aorta may cause the aortic valve to leak, which can lead to a sudden tearing of the layers in the aorta wall (aortic dissection). Aortic aneurysm and dissection can be life threatening. Many people with Marfan syndrome have additional heart problems including a leak in the valve that connects two of the four chambers of the heart (mitral valve prolapse) or the valve that regulates blood flow from the heart into the aorta (aortic valve regurgitation). Leaks in these valves can cause shortness of breath, fatigue, and an irregular heartbeat felt as skipped or extra beats (palpitations). Individuals with Marfan syndrome are usually tall and slender, have elongated fingers and toes (arachnodactyly), loose joints, and have an arm span that exceeds their body height.
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PHYSICAL ACTIVITY GUIDELINES Everyone —Including PEOPLE with MARFAN Syndrome— Benefits from Exercise
NOV 2017 PHYSICAL ACTIVITY GUIDELINES EVERyONE —iNCLuDiNg PEOPLE WiTh MARFAN SyNDROME— BENEFiTS FROM ExERCiSE. Regular exercise improves both physical and emotional well-being and can be incorporated safely into the routine of people with Marfan syndrome. Therefore, they are encouraged to adapt health measures that protect them from Marfan features that can worsen and from medical conditions that are simply part of the aging process. With an early diagnosis, treatment , and lifestyle adaptations, many people with Marfan syndrome can now expect to live a normal life span. These guidelines are intended for those with Marfan syndrome and related disorders, however, individuals may have unique disease-specific manifestations that require additional consideration and restrictions. For example, those with Loeys Dietz syndrome may have cervical instability, which impacts guidelines on certain exercise and physical activity. Please consult with your physician about your individual case. WHY DOES PHYSICAL ACTIVITY HAVE TO BE MODIFIED FOR PEOPLE WITH MARFAN SYNDROME ? Marfan syndrome is a disorder of connective tissue. Connective tissue holds all parts of the body together and helps control how the body grows. Because connective tissue is found throughout the body, Marfan syndrome features can occur in many different parts of the body, including the heart, blood vessels, bones, joints, and eyes. Sometimes, the lungs and skin are also affected. Anyone with a health concern should learn about self care for their condition. An important part of self care is physical activity. Physical activity guidelines are important because they enable people to achieve the benefits of safe levels of exercise and, at the same time, ensure that they don’t add to medical problems related to Marfan syndrome.
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Rev. 6/98 Chapter 2—Instructions for Completing a Certificate of Birth 1
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